A seven-month-old male with an eye turn since birth had an esotropia (ET) at near of 60 prism-diopters (PD), alternate fixation with a preference for the right eye (R/E), -4 abduction of both eyes (B/E), a cycloplegic refraction of +1.50 diopters (D) B/E and an otherwise unremarkable ophthalmic examination. Uncomplicated 6mm medial rectus muscle recession B/E and 6 mm lateral rectus resection of the left eye (L/E) were performed; intraoperative forced duction test did not reveal significant restriction. One week postoperatively the patient was orthotropic with a narrow palpebral fissure L/E (described as 1 mm narrower than that of R/E).
The patient was next seen at 16 years of age because of exotropia (XT) L/E that reportedly occurred gradually one year after his strabismus surgery. Vision was 20/20 R/E, 20/30 L/E. Fixating R/E, there was a 40PD XT with a variable left hypertropia (LHT) up to 25PD. The variable LHT was related to an upshoot during abduction L/E. Motility was significant for the following: -2 abduction and -1 adduction R/E; -1 adduction and -3 abduction L/E; severe palpebral fissure narrowing and marked globe retraction during attempted abduction in either eye that appeared to be due to horizontal rectus muscle co-contraction . There was no significant refractive error. Forced duction test performed in the office under topical anesthesia revealed no resistance to abduction and slight resistance to adduction in either eye. The patient was undecided about further strabismus surgery.


A 64-year-old male presented to us six months after an
uneventful phacoemulsification with IOL implantation, with
history of four episodes of redness and pain starting two
months after surgery. The signs and symptoms apparently
responded to topical prednisolone acetate eye drops (eight
times a day and tapered gradually), but recurred following
cessation of the treatment. According to the records, his
vitreous was never involved. Two anterior chamber taps
done previously were negative on microbiological workup.
The last aqueous tap was negative even on polymerase
chain reaction (PCR) examination for Propionibacterium acnes
genome and eubacterial genome. At presentation, his vision
was 20/60 in the affected eye. There were keratic precipitates,
aqueous flare and cells. The IOL was in situ and there was
no definitive plaque made out at the posterior capsule. At
12 O′clock meridian, some creamy material was suspected
behind the anterior capsule. Under direct vision using the
operating microscope, with maximum pupillary dilatation, a
27-gauge needle connected to a 2-ml syringe was introduced
from the infero-temporal quadrant of the limbus under topical
anesthesia. With the bevel of the needle facing forwards, the
anterior capsule was lifted gently and the creamy material was
scraped. The loosened material was gently aspirated and was
subjected to smear (KOH, Calcoflour white and Gram′s stain)
and culture (blood agar, chocolate agar, Brucella blood agar,
brain heart infusion broth and Robertson′s cooked meat broth).
The smear showed plenty of gram-positive pleomorphic bacilli
[], which grew Propionibacterium acnes on Brucella blood
agar, confirmed with PCR technique. Once the organism was
identified, vancomycin (1 mg) was injected into the capsular
bag, repeated again after an interval of five days. The infection
was totally controlled and he could be weaned off all the
medications (moxifloxacin and prednisolone drops initially
instilled eight times a day and tapered within one month).
At the last examination one year after the intracapsular bag
injection, his vision was 20/20 and the eye was quiet.


A 65-year-old woman presented to us five months after
epiretinal membrane surgery, with a complaint of decreased
vision in her left eye. Her past history suggests that she
underwent uneventful cataract surgery with intraocular lens
implantation in the left eye following which her visual acuity
(VA) was 20/400. She was diagnosed to have vitreomacular
traction with neurosensory detachment which was confirmed
on optical coherence tomography (OCT). Based on the
diagnosis, she underwent pars plana vitrectomy, epiretinal
membrane peeling and fluid gas exchange in the left eye one
month later. Four weeks following surgery, her VA was 20/200
in the left eye.
On examination, the best corrected visual acuity (BCVA) was
20/20 in the right eye and 20/160 in the left eye. Anterior segment
examination was within normal limits. Intraocular pressure
was 12 and 14 mm Hg in the right and left eye respectively.
Fundus examination of the right eye was unremarkable. The left
eye showed serous elevation of the macular area with cystoid
changes and a thin foveal roof. Residual epiretinal membrane
was also seen [Figure ]. Optical coherence tomography of
the left eye confirmed the findings []. There was no
evidence of a macular hole. Retinal vasculatures were within
normal limits. Fundus fluorescein angiography (FA) showed
neither leakage nor any evidence of choroidal pathology. She
was advised ICG-assisted ILM peeling with silicon oil injection
in left eye. The reason for ILM peeling was serous detachment
persisting for five months following surgery, raising the
possibility of a missed small macular hole.
Intraoperatively, 0.5% ICG was injected in the vitreous
cavity using a double cannula and after a 30-sec gap, the ICG
was aspirated. Though an obvious retinal hole or tear was not
seen in the macula and other retinal area, we noted subretinal
presence of ICG during aspiration. Internal limiting membrane
peeling was done up to the arcades. The subretinal presence
of ICG was noted prominently after removal of the ILM. An
attempt was not made to remove the ICG as there was no gross
evidence of hole and ICG binds to RPE immediately. Fluid air
exchange was performed and the macula was flat following
which silicone oil was injected. On postoperative Day 1, BCVA
was counting fingers (CF) at 1/2 meter in the left eye and fundus
examination showed subretinal ICG in the superior half with
a flat macula [Figure ]. At one week, her BCVA was CF at ½
meter with an extensive area of RPE alteration in the macula
[Figure ]. Optical coherence tomography of left eye showed
foveal thinning (83 micron) with inferior shallow neurosensory
elevation []. After three months, her BCVA was counting
fingers at 1.5 meters in the left eye. Fundus examination showed
RPE atrophy with pigment stippling in the superior half of the
macula within the arcades [Figure ]. She underwent silicone
oil removal in the left eye. Four months after silicone oil removal,
her BCVA was CF 2.5 meters in the left eye which was maintained
till 12 months of follow-up. Optical coherence tomography at
this time showed foveal thickness of 80 microns.


A 70-year-old male patient presented to us with pain and
redness in right eye for one month. He had undergone
cataract operation elsewhere following which he had iris
prolapse. Repeat surgery was done for iris prolapse, after
which he developed macular scarring and secondary
glaucoma. Medications had failed to control his intraocular
pressure.
Visual acuity of the right eye was finger-counting two meters.
Intraocular pressure (IOP) of the right eye was 40 mm Hg on
maximal medical therapy (dorzolamide-timolol combination
in twice daily dosage, bimatoprost at night, brimonidine eye
drops in thrice daily dosage and oral acetazolamide in four
times daily dosage).
The examination of the right eye revealed a clear cornea
with a 12 o′clock cataract wound. The pupil was irregular with
6 mm dilatation and an intraocular lens (IOL) in the sulcus.
The macula showed retinal pigment epithelial changes with
macular scarring. The disc revealed a cup to disc ratio of 0.6:1
in the right eye.
Examination of left eye revealed a visual acuity of 20/20
and IOP of 14 mm of Hg. The eye had undergone an IOL
implantation a year back with cornea being clear, IOL in
position and the fundus revealing no abnormality.
Patient refused trabeculectomy for the right eye. We
performed transconjunctival diode laser cyclophotocoagulation
(Nidek DC 3300) under local anesthesia with the consent
of the patient. The patient received 16 applications in the
inferior 180 degrees using power of 1700 mW for 2 seconds
per application. Postoperatively, IOP ranged between 7 and
10 mm Hg on no antiglaucoma medication.
The cornea was clear on the first postoperative day but
became hazy inferiorly from the third postoperative day and
covered inferior three-fourths of the cornea by the first week
[]. The patient was started on hyperosmotic saline drops
along with frequent topical steroids and atropine eye drops.
The corneal edema cleared gradually over six weeks and the
patient regained preoperative visual acuity. The IOP was
maintained at 8 to10 mm of Hg without any antiglaucoma
medications. The specular microscopy of the right eye was
done at six weeks when the cornea was clear enough to allow
it. It revealed a gross polymorphism and pleomorphism with
an endothelial cell count of 880 cells per mm. We suspected
poor endothelium health to be the cause and to corroborate our
suspicion, we did the specular microscopy of the left eye too.
It revealed some polymegathism and pleomorphism with an
endothelial cell count of 1600 cells per mm.


A 64-year-old female presented with gradual loss of vision in the right eye (OD) of one year duration. About six months ago, she had undergone intracapsular cataract extraction, anterior vitrectomy, and scleral fixation of intraocular lens (IOL) in the left eye (OS). At that time, she was suspected of iridocorneal endothelial syndrome (ICES).
The preoperative best corrected visual acuities (BCVA) were 20/400 OD, 20/30 OS. Intraocular pressures (IOP) in each eye were 15 mmHg by applanation. The Slit lamp biomicroscopy of both eyes (OU) showed clear cornea, deep and clear AC, and the angle was wide open. Both eyes had splitting of the anterior layers of the iris with fibrillar degeneration extending from 3 to 6 o' clock OD, and from 4 to 6 o' clock OS. Additionally, there was diffuse atrophy of iris from 11 to 1 o' clock OS (). The underlying iris pigment epithelium appeared intact with no transillumination defect. The clinical appearance was consistent with iridoschisis OU. The right eye showed nuclear and cortical cataract associated with posterior subcapsular cataract, and the left eye was pseudophakia. Specular microscopy of the OD revealed regular, hexagonal shape of endothelial cells without any abnormalities like bullae or guttae, and the endothelial cell count was 2865 cells/mm2. The rest of the ocular examination was normal.
Pupil dilation is performed using 3 drops of tropicamide 0.5% and phenylephrine hydrochloride 0.5% at 5-minute intervals 1 hour prior to surgery. Intraoperative pupillary diameter was about 5~6 mm. Topical anesthesia was applied using Alcaine® and 3% Lidocaine, and a paracentesis was done at 10 o' clock. A 2.75 mm main clear corneal incision was performed using the keratome blade. A dispersive ophthalmic viscosurgical device (OVD), sodium hyaluronate 3%-sodium chondroitin sulfate 4% (Viscoat®, Alcon) was injected through the clear corneal wound to stabilize the anterior chamber and protect the iris stroma by displacing aqueous from the AC and pushing the iris strands from the pupillary aperture. An anterior continuous curvilinear capsulorhexis not exceeding over the pupillary margin was performed using the 26 G needle and capsular forceps. After hydrodissection and hydrodelineation, careful phacoemulsification was performed (Allergan Sovereign® Phaco System, SOV680330) for nucleus removal with special care not to touch the iris tissue. Aspiration was used to clean residual cortical fibers from the capsular bag. When a loss of the protective effect of viscoelastic material was observed during surgery, Viscoat® was reinjected additionally to inhibit iris fibril movement with the BSS stream.
The maximum limit of the vacuum was 300 mmHg and the aspiration rate, 20 cc/mmHg. The height of the irrigation bottle is maintained less than 60 cm to minimize the turbulence within the chamber. Total US time was 48 seconds with a mean final rate of 10% of potency.
The capsular bag was inflated with viscoelastics, and a single-piece acrylic intraocular lens (Acry-Sof® SA60AT, Alcon) was inserted. The IOL was safely placed in the capsular bag and centered, followed by aspiration of the residual viscoelastics. If there were some movement of iris fibrils, additional viscoelastics was inserted.
Finally, BSS Carbacol (carbachol 0.01%, Miostat®) was used to constrict the pupil.
Postoperatively, the inflammatory response in the AC was moderate, some fibrillar materials were seen. The cornea showed some Descemet folds, and the pupil was round and undamaged ().
One week after surgery, the uncorrected visual acuity was 20/100 OD, and improved to 20/30 on the two weeks postoperative day. There were traces of cells in the AC, but no fibrillar materials were seen ().
The patient's last visit was 1 month after surgery when UCVA was 20/40, and BCVA 20/20. The cornea, AC and iris were clear and intact without any inflammatory sequalae.


A 60-year-old woman admitted for elective excision of a right thyroid papillary carcinoma. She had complained of hoarseness and aspiration while swallowing for several months. Rigid endoscopy confirmed paramedian vocal cord fixation with large glottic chink. Preoperative computed tomography (CT) detected a solid thyroid mass invading the perithyroid tissue with suspicion of right tracheoesophageal groove invasion. Total thyroidectomy with central neck dissection was carried out via transcervical incision. Intraoperatively, the tumor was found to invade the right recurrent laryngeal nerve and along the right tracheoesophageal groove (). The right recurrent laryngeal nerve was sacrificed and the tumor invading the tracheoesophageal groove was shaved off the trachea and esophagus. After tracheal shaving, a small perforation (0.5×0.5 cm) was noticed in the posterior membranous portion of first tracheal ring (). The pleura of the lung and mediastinum were intact. The perforation was repaired primarily with No. 4-0 Vicryl suture and reinforced with Surgicel® and tissue glue. The trachea did not leak by underwater testing during positive anesthetic ventilation and the operation was completed.
One day after surgery, the patient developed dyspnea, cervical emphysema, and ecchymosis over the neck after an episode of vigorous coughing. With the impression of a postoperative hematoma an emergency neck exploration was performed under general anesthesia. A small hematoma was found beneath the platysma and strap muscles, No obvious bleeding point was evident and the reconstructed trachea portion was relatively intact. Postoperatively, while still in the recovery room, the patient showed signs of acute respiratory failure. A chest radiograph revealed bilateral pneumothorax, pneumomediastinum, and massive subcutaneous emphysema (). Her dyspnea and cyanosis were relieved after an emergency chest tube was inserted. Over the next week, the pneumomediastinum and pneumothorax resolved gradually. The patient discharged without any problems.


A 30-yr-old Korean man visited to hospital for the evaluation of a growing palpable abdominal mass, which he had felt growing over 6 months. There was no family or personal history of tuberous sclerosis. Physical examination revealed a non-tender, palpable mass in the periumbilical area. The chest radiogram was normal. Colonoscopic examination revealed non-specific abnormal findings. A computed tomography (CT) scan of the abdomen showed an intraabdominal mass with a size of about 7.5 cm that was supposed to originate from the falciform ligament. The mass was well enhanced and showed wall calcification (), and laboratory data showed no abnormalities. Surgical excision was performed. The resected specimen had a weight of 199.1 g and dimensions of 8.0×7.0×5.5 cm. The resected specimen showed a uniloculated cystic mass with a solid portion. The solid portion was yellow-brown in color and revealed foci of hemorrhage and necrotic changes (). Histological examination showed sheets of spindle-to-epithelioid cells with clear-to-eosinophilic cytoplasm (). Immunohistochemically, tumor cells were positive for HMB-45 () and smooth muscle actin (SMA) (). They were also positive for the S-100, a marker of neurogenic and melanocytic tumors, and were negative for the CD117 (c-kit), which typically shows positivity in classic gastrointestinal stromal tumors (GISTs). The tumor cells were also negative for Factor VIII, CD34, CD31, CK, and desmin. However, the tumor showed high cellularity and necrotic foci. These features suggest that the tumor had aggressive potential and a high probability of local recurrence or metastasis. The Ki-67 proliferative index was approximatively 15 percent of cells. At 6 days after surgical excision, the patient was discharged. Because the biological behavior of this tumor is still unclear, with descriptions in the literature of PEComas with metastases, we had planned follow-up investigation in the form of CT scans of the abdomen and the thorax. Until now, there was no tumor recurrence.


A 43-year-old man visited the emergency room with epigastric pain. According to the patient, he had no significant medical or surgical history, except for hospitalization 20 years ago due to similar abdominal pain that resolved with conservative treatment. The patient's vital signs were stable and mild tenderness was noted on the epigastric area. The bowel gas pattern was non-specific on the initial plain radiographs. The abdominal CT scan was performed with using a 16 row detector CT (Sensation16, Siemens, Erlangen, Germany) at the portal phase after administering intravenous iodinated contrast agent (Ultravist 300, Bayer Schering Pharma, Berlin, Germany). We obtained the axial and coronal reformatted images that were 5 mm thick. A well-demarcated lesion approximately 15×10×6 cm in size with homogeneous fluid attenuation was noted in the pelvic cavity. The lesion was located within the rectovesical pouch, and it mimicked peritoneal inclusion cysts or loculated ascites. The abdominal pain subsided with conservative therapy and the patient was discharged.
Twenty days after the first visit to our hospital, he revisited the emergency room with recurred epigastric pain. He also complained of nausea and vomiting. The blood urea nitrogen and creatinine (42.4 and 2.09 mg/dL, respectively) were slightly more increased than before (14.2 and 1.06 mg/dL, respectively), but the other laboratory results were normal. On physical examination, there was tenderness and rebound tenderness on the epigastric area, with hyperperistaltic bowel sounds. The plain radiograph suggested a markedly distended inverted U-shaped small bowel loop with an air-fluid level (). A second abdominal CT scan was performed after administering an intravenous contrast agent. A fluid density mass abutting to the small bowel loops was again noted in the pelvic cavity (). A thin fatty layer between the mass and the small bowel suggested that the mass probably originated from the mesentery rather than from the bowel loop. Whirling of the mesenteric vessels and small bowels around the superior mesenteric artery was disclosed on the axial and coronal reformation images (). The superior part of the cystic mass was insinuated into the whirl. A dilated small bowel was seen tapering with a beaked appearance at the center of the whirling and this continued to the collapsed small bowel that abutted the cystic mass in the pelvic cavity. A markedly distended small bowel loop proximal to the collapsed segment was found, and this corresponded with the distended bowel loop seen on the plain radiographs. All the findings were suggestive of volvulus of the small bowel with closed loop obstruction, and we presumed that the cystic mesenteric mass was the cause of the volvulus.
Diagnostic laparotomy was performed and a large cystic mass arising from the small bowel mesentery at 60 cm distance from the Treitz ligament was found (). The mass consisted of multiple well-defined locules filled with clear fluid. There was no communication between the cystic mass and the lumen of the small bowel. We observed torsion of this cystic mass that caused volvulus of the connecting mesentery and 55 cm of the small bowel, and this eventually resulted in closed loop obstruction of the small bowel. The volvulus of the small bowel and mesentery was reduced by rotating the mass. There was stricture and luminal narrowing at the twisted small bowel, so segmental resection and end-to-end anastomosis were performed. The mass was pathologically confirmed as being a lymphangioma ().


A 22-year-old, right-handed, unmarried woman was admitted to the psychiatric unit due to her selective retrograde amnesia that occurred after a severe stressful event. She could remember nothing of the autobiographical events for the preceding four years, that is, her university life period. She was aware only of a hallucinatory vision of a parking lot in which she was kidnapped and violated by a masked robber. However, she could recount in detail the events of her high school life and also the events of her life before that. There were no neurological signs, no suspicion of head trauma and no epileptic discharge on the electroencephalography. Her brain MRI was normal.
To examine the underlying brain functional disturbances associated with psychogenic amnesia, we performed a fMRI study for a face recognition task. The patient viewed three kinds of face photographs: recognizable faces of familiar high school friends, unrecognizable faces of familiar university friends, and unfamiliar control faces. The patient was instructed to try to recognize the faces that were shown to her. All faces were presented to the patient through a mirror located at the top of the head coil that received the face photographs from outside of the magnetic room during the fMRI procedure.
The T1-weighted images (time to repeat/echo time/flip angle = 500 ms/50 ms/90°) were obtained for anatomical localization by using a 1.5 T MRI scanner (GE Medical System, Horizon Milwaukee, WI, USA). The BOLD-contrast fMRI images were acquired from 11 slices that covered the limbic areas with using gradient-echo EPI (time to repeat/echo time/flip angle = 3000 ms/50 ms/90°). Post-processing of images was performed with MATLAB (MathWorks, Natick, MA, USA) and SPM99 (Statistical Paramatric Mapping software; Wellcom Department of Cognitive Neurology, London, UK). The SPM99 software was used for image realignment, normalization, smoothing and to create the statistical maps of the significant relative changes in the regional blood oxygenation level-dependent (BOLD) response.
The blocked design fMRI paradigm, which was preceded by three dummy scans to allow the magnetic resonance signal to reach a steady state, was comprised of seven repetitions with nine seconds of rest (fixation cross), and six repetitions of a nine seconds activation state (three kinds of photograph that were randomly comprised of three seconds of recognizable faces of high school friends, three seconds of unrecognizable faces of university friends, and three seconds of unfamiliar control faces). A correlation coefficient algorithm was used to correlate the time course data sets with the periodic boxcar function. Three different subsets of the activation periods were independently compared with those of the rest periods. The BOLD signals were estimated for the comparison of whole brain signals across all the experimental conditions. Specific effects were tested for by applying appropriate linear contrasts to the parameter estimates for each condition, and this resulted in a Z-score for each voxel. The voxels were identified as being significant only if they passed a height threshold of Z = 3.5 and they belonged to a cluster of at least 30 activated voxels. The data were analyzed for the main effects of each stimulation: the recognizable faces of high school friends, the unrecognizable faces of university friends and the unfamiliar control faces.
For the recognizable condition, different patterns of brain activation were found as compared with the unrecognizable condition. The functional maps of the representative brain slices demonstrated differences for activation in the limbic areas: the amygdala, hippocampus, parahippocampal gyrus and insula. Especially, the activation of the amygdala and hippocampus was significantly greater during the stimulation with recognizable faces of high school friends (). However, the activation patterns stimulated with the unrecognizable faces of university friends and the unfamiliar control faces were similar to each other. They showed significantly less activation in the limbic area than the recognizable faces ().


A 56-year-old woman presented with multiple, soft, palpable masses in her right breast. Three months earlier, heavy falling boxes injured her breast; soon after she noted gradually increasing multiple masses. Medical history was negative for any hematologic disease or bleeding tendency. Mammogram showed multiple, hyperdense masses with well-defined margins in the right subareolar and upper outer quadrant areas (). Ultrasonogram (US) of the right breast showed multiple, round-shaped cysts with floating echogenic debris (); there were no suspicious echogenic lesions projecting from the wall. Fine needle aspiration biopsy was performed; aspirate was blood-stained, but there was no remaining residual mass and cytologic evaluation was negative for malignancy. Our impression was post-traumatic hemorrhagic cysts, and we recommended a three month follow-up US. The patient returned two months later for recurrence of the masses. The second US revealed recurrent cysts at the same site. Surgical excision and follow-up was recommended, however, the patient refused. After an additional three months, symptoms worsened and the patient complained of growing masses and circumferential, painful, nodular lesions. The third US revealed marked enlargement of the cystic masses with irregular, thickened, echogenic solid lesions along the cyst walls (). Blood flow signals of the papillary solid portion were seen on color Doppler sonography (). A biopsy of the solid portion was performed using a 14-gauge automated gun; the mass was diagnosed as papillary carcinoma. The patient was treated with modified radical mastectomy and axillary dissection. The gross sample was ill-defined with dark reddish colored cystic masses with polypoid projections from the wall. The confirmed final diagnosis was intracystic papillary carcinoma with invasion. Axillary dissection found metastasis at two lymph nodes among twenty resected nodes.


A 39-yr-old man visited the clinic due to trigger finger disease in every digit of both hands which had been aggravated during the past 3 months. A year ago, the patient was treated three times with local steroid injections in both trigger thumbs. Since childhood, the patient has suffered from tingling sensation, coldness and bluish discoloration of his fingers during winter and cold weather. Therefore, the patient had to constantly wear gloves.
The patient's symptom was aggrevated after changing his occupation to a laborer 3 months ago. He had worked for 6 hr a day and 6 days a week by using vibrating tools with forceful hand grip such as grinders.
His mother and maternal aunt also had a history of operative treatments due to multiple trigger fingers. Examination showed obvious nodules on the A1 pulleys in both thumbs, middle fingers and ring fingers for which the patient always felt uncomfortable. There were no nodules in the index and little fingers even though an intermittent triggering occurred particularly in the morning and after working. He could not perform a forceful grip when flexing the fingers due to pain and tendon entrapment at the A1 pulley (). In addition, he could not extend his fingers freely (). The Allen test was normal. Initial plain radiographs showed dense bone island in the middle phalanx shaft of the right little finger and in the metacarpal head of the left thumb. Laboratory test for thyroid functioning was normal. The patient refused to do the provocative test for Raynaud's disease.
To relieve the symptoms, A1 pulley was released in all ten fingers: longitudinal incision for the fourth finger of the right hand and transverse incisions for the other 9 fingers. Intraoperatively, a partial tear of the flexor pollicis longus tendon and hypertrophied A1 pulleys in both thumbs were observed. There were no nodules in the flexor tendons and no tears in the other flexor tendons. All fingers except both thumbs did not have any nodules in the flexor tendons or tears of the flexor tendons. The partial tear of both flexor pollicis longus tendons in both thumbs might be due to previous steroid injections. After operation, the patient had a recovery period for a month without working. Three months after the operation, the patient could return to his previous job without discomfort and showed normal range of motion in all fingers. The previous symptoms such as impairment of a forceful grip and locking in flexion disappeared completely ().


A 46-year-old man was referred for ophthalmologic examination due to gradually decreasing visual acuity. He had suffered from disturbances of ocular movements, ptosis and weakness of the extremities for two years. Through polymerase chain reaction (PCR), the patient was found to have amplification of CTG repeats on chromosome 9, which is compatible with myotonic dystrophy type 1.
On ophthalmologic examination, the patient's best corrected visual acuity in both eyes was 0.7. Intraocular pressure was 13 mmHg in the right eye and 15 mmHg in the left eye. The levator function was 0 mm in the right eye and 1 mm in the left eye. In the primary position, exotropia of 10 prism diopters was found. Limitation of ocular movement was observed in every gaze direction (). On slit lamp examination, a bilateral iridescent cataract was noted. Fundus examination showed geographic depigmentation of the retinal pigment epithelium along the vascular arcade and pigment clumps in the peripheral area () in both eyes. Fluorescein angiography showed blocked fluorescence by pigment clumps and window defects (). The b-wave amplitude was decreased on electroretinography. Eye movement recordings showed slowed saccades and impaired smooth pursuit.
Fourteen months later, the patient's best-corrected visual acuity had decreased to 0.3 in both eyes. Phacoemulsification and intraocular lens implantation were sequentially performed in both eyes and his best-corrected visual acuity improved to 0.7. Fundus examination showed no significant interval changes.


A 53-year-old woman presented with metamorphopsia in her right eye. She was diagnosed with subfoveal CNV secondary to age-related macular degeneration (AMD) and the best-corrected visual acuity of the right eye was 20/50. PDT with Verteporfin was performed according to the standard protocol of the Treatment of Age-Related Macular Degeneration with Photodynamic Therapy Investigation and repeated after 5 months. The visual acuity of the involved eye did not change.
Fifteen months after the second PDT, fluorescein angiography of the affected eye revealed persistent subfoveal CNV and the best corrected visual acuity remained 20/50 (, ). Combined PDT according to the same standard protocol and 1.25 mg intravitreal bevacizumab injection was performed on the same day. One day after the treatment, the patient's vision had decreased to counting fingers. Fundus examination and optical coherence tomography (OCT) showed serous retinal detachment involving the macula ().
On post-treatmentday two, the patient was initiated on an oral steroid (Triamcinolone® 24 mg #1) to complete a two week course. One week into steroid therapy, the visual acuity remained the same, but OCT findings of the right eye showed decreased serous retinal detachment (). The vision slightly improved to 20/200 upon completion of the two week steroid course and fluorescein angiography showed decreased leakage of fluorescein dye ().


A 45-yr-old female patient presented with a history of headache and dizziness, both of which progressed slowly. A neurological examination showed nothing remarkable. Preoperative magnetic resonance imaging (MRI) revealed a left frontal lesion that was slightly hypointense on T1-weighted images and hyperintense on T2-weighted images. The lesion extended to the cingulate gyrus with peritumoral edema and strong contrast enhancement (). Gross total resection of the tumor revealed a dark-brown, friable mass, consisting of a soft, aspiratable portion and a fibrous hypervascular nonaspiratable portion. Microscopically, the ganglioglioma contained atypical glial cells and scattered ganglion cells. The tumor cells infiltrated the gray matter and subarachnoid space (). The tumor cells in the gray matter consisted of neoplastic ganglion cells and glial cells. Those in the subarachnoid space were mainly glial cells and showed increased cellularity, nuclear atypism, and occasional mitosis (). The abnormally clustered ganglion cells lacked orderly distribution and polarity, and most had large nuclei with prominent nucleoli (). The neoplastic ganglion cells were immunoreactive to synaptophysin and neuron specific enolase (NSE) (). The atypical glial components were positive for glial fibrillary acidic protein (GFAP) (). A diagnosis of anaplastic ganglioglioma was made. The patient recovered postoperatively without any neurologic deficits. MRI taken 12 days postoperatively showed multiple irregularly shaped enhancing lesions along the resection margin of the left frontal lobe and cingulate gyrus (). Two weeks after surgery, she received one cycle of intravenous nimustine HCl (ACNU, 60 mg) and cisplatin (CDDP, 60 mg). Subsequently, radiation therapy was administered in 30 doses for a total dose of 6,000 cGy over five weeks, and additional three cycles of chemotherapy with the same regimen were given. During chemotherapy, she was managed for pancytopenia. Five months later, follow-up MRI revealed that the remaining mass in the cingulate gyrus and resection margin had disappeared (). She has shown intact neurological status and no other complications until now, and the last follow-up MRI at 35 months postoperatively demonstrated no evidence of recurrence ().


A 32-yr-old, gravida 2, para 1 woman at 18 weeks of gestation was referred to our hospital by a university medical center because of severe dyspnea accompanying polyhydroamnios. While receiving antenatal care at a local hospital, polyhydroamios was noted at 17 weeks of gestation, and then the patient was transferred to the university medical center. At the university medical center, the patient was treated with amnioreduction. While waiting for the result, the patient complained of severe dyspnea at 18+4 weeks of pregnancy and was transferred to our hospital via emergency department. The patient had no present and past medical history. In addition, the family history was not contributory. This twin conception was spontaneous without any medication or ovulation induction and there was no known significant obstetric or gynecological history of the mother.
A detailed two-dimension high-resolusion ultrasound was done right after admission and showed monochrorionic monoamniotic placenta. Twin had no significant size difference, and the estimated size of the 1st fetus was 18+5 weeks while the 2nd fetus was 17+6 weeks. However, the 2nd fetus was stuck due to the scanty amount of amniotic fluid, and the amniotic fluid index (AFI) of the 1st fetus was 320 mm showing polyhydroamnios. For the relief of patient's symptoms, 1,400 cc of amnioreduction was conducted at the day of admission. And the day after the procedure, the patient was discharged with AFI of 220 mm. Five days after discharge, the patient's dyspnea recurred and the sonographic finding showed AFI increased to 260 mm. The growth of the 1st fetus was normal measuring 19+2 weeks size. But the 2nd fetus was only 17+6 weeks size, presenting the findings of stuck twin. The second amnioreduction treatment was performed and 800 cc of amniotic fluid was removed. However, further reduction couldn't be performed due to intractable pain. Regarding the speed of increasing amniotic fluid, amnioreduction was expected to be performed every week. Complications of amnioreduction and poor outcome of early severe TTTS was explained to the patient and her family. After obtaining informed consent, we decided to perform selective abortion using radiofrequency (RF).
The method of selective abortion using RF ablation was as follows. Just before the procedure, pethidine (Demerol®) 50 mg was injected intramuscularly for emotional stability and pain killing, and prophylactic IV antibiotics was given. Betadine sterilization was done and 2% lidocaine was injected for local anesthesia. Under local anesthesia, 1 mm skin incision was done at the point where the electrode will be inserted. Under the guidance of real-time ultrasonography and color doppler, 17 gauge radio-frequency electrode was carefully inserted to the initiating portion of umbilical cord of the 2nd fetus and radio-frequency ablation was done. Intensity of radiofrequency was estimated by hypoechogenic lesion representing air bubbles which was formed as temperature of tissue rises by radiofrequency (). The RF intensity was increased up to 50 W. A few minutes after the procedure, cardiac arrest of the 2nd fetus was observed. Doppler flow at the cord of 2nd fetus was checked before and after the procedure (). The cord occlusion was successful and there was no blood flow to the 2nd fetus. Mild uterine contraction was noted after the procedure so that tocolytics (ritodrine [Yutopar®], 30 cc/hr) was infused. After the procedure, the patient's symptoms were improved and there was no other complications such as spontaneous premature rupture of membranes or vaginal bleeding. The patient was discharged 4 days after the procedure.
At the follow up ultrasonography performed after the procedure at 20+5 weeks of pregnancy, AFI was increased to 270 mm. The patient complained of dyspnea and 1,900 cc of amnioreduction was done. The patient regularly visited outpatient department and ultrasonography was done to check normal growth and development. and amniotic fluid of the surviving 1st fetus. Growth of the 1st fetus was normal but right mild ventriculomegaly of l.02 cm was noted at 24+6 weeks of pregnancy (). However, it was normalized to 0.9 cm at 28 weeks of pregnancy. Mild oligohydramnios developed after amnioreduction and continued until 24+6 weeks of pregnancy and AFI was measured as 80 mm at 28 weeks of pregnancy. This mild oligohydroamnios status continued until delivery.
At 35 weeks of gestation, the patient was admitted to our hospital with chief complaints of spontaneous premature rupture of membranes and delivered healthy male baby weighing 2.33 kg. Apgar score at the first 1 min was 9 point and 10 point at 5 min. Baby was sent to pediatric department due to prematurity and low birth weight but no other abnormality was found. Selectively aborted 2nd fetus was delivered 5 min after the delivery of first fetus along with placenta. It showed iatrogenic gastroschisis possibly due to radio-frequency ablation to the initiating portion of the umbilical cord of (). Placenta was monochorionic diamniotic, fused type and weighted 430 g. Placenta of selectively aborted fetus was completely infarcted ().
Two days after delivery, the mother was discharged without specific complication and brain sonography of the baby showed normal finding. During the neonatal period baby showed normal growth rate and pattern.


A 60-yr-old woman presented with incidentally detected prevesical mass. Her history was remarkable in that she had a total hysterectomy by lower median abdominal incision for endometriosis 11 yr ago. The family history was unremarkable. Physical examination showed no palpable abdominal mass. Routine blood tests, blood chemistry and urine test revealed no abnormal findings. In the previous ultrasonographic examination, heterogeneous hyperechoic pelvic mass was incidentally detected, and she was transferred to our hospital on the impression of urachal tumor ().
Magnetic resonance imaging of the abdomen and pelvis showed a mass located anterior to the bladder in the prevesical space of Retzius and helped to establish the relation of the tumor to the bladder in the sagittal plane ().
Complete excision with bladder sparing was successful by lower median abdominal incision. At surgery, two tumor masses were founded in the prevesical space of retzius and had not infiltrated the wall of the bladder and urachus.
Grossly, well-circumscribed yellowish tumors were 4.5×2.5×2 cm, 1.5×1×1 cm in size, respectively. Microscopically, the lesion was characterized infiltrating nest of tumor cells. The tumor consisted of relatively large cells resembling those of the endometrial stroma, which encircled small vessels (). Tumor cell nuclei was round to ovoid and a small amount of cytoplasm was present. Mitotic activity was less than 10 mitotic figures per 10 high power field (HPF) (). Immunohistochemically, the tumor cells were positive for CD10 but negative for cytokeratin. Most of the cells were also negative for estrogen receptor, and positive for progesterone receptor (data not shown). Based on these findings, a low-grade ESS was diagnosed. Her postoperative recovery was uneventful. She was free of symptoms at the last follow-up, 15 months after the abdominal surgery, with no evidence of recurrence.


A 61-yr-old woman was admitted to the emergency department with symptoms of sudden abdominal pain, nausea, vomiting and painful swelling in a previously diagnosed umbilical hernia. She had had severe abdominal pain and the sensation of a mass for the previous 2 hr. Because of her low socioeconomic status, she had no history of routine healthcare, and she had a history of uncontrolled hypertension.
On examination, she had a 15×10 cm, irreducible, purple-colored umbilical hernia, and there was extreme tenderness and hyperemia on hernia. Because of the incarcerated umbilical hernia and possible strangulation of the hernia content, the patient underwent an urgent operation without any diagnostic evaluation.
During the operation, a partial omental infarct and an intraabdominal tumor were found in the hernia sac (). A partial omentectomy was performed, and abdominal incision was extended from pubis to the epigastrium. A large, multilocular, left ovarian cyst, which extended from the transverse colon to rectovesical space, was observed (). After dissecting the tumor from the adjacent tissues, tumor excision, total abdominal hysterectomy and bilateral salphingo-oophorectomy were performed with no complications. During the operation, a frozen section was unavailable. The abdominal wall was closed with primary continuous sutures.
The resected tumor measured as 25×23×16 cm and weighed 3,680 g. On microscopic examination, the tumor was uniform and composed of pale oval cells with prominent grooves (coffee bean appearance). The diagnosis of a granulosa cell tumor was supported by the immunohistochemical expression of inhibin in the neoplastic cells ().
Because she had high blood pressure, the patient was followed-up in the intensive care unit. The hypertension was controlled, and the patient recovered uneventfully. She was discharged 6 days after her admission. About 2 weeks after discharge, she underwent second operation (ie, a staging operation) that used the same incision. An omentectomy, a paraaortic and pelvic lymphadenectomy, peritoneal washing, cytology analysis, and peritoneal biopsies were done at that time, and no metastases were found. After the second operation, combination chemotherapy with bleomycin (10 mg/m2), cisplatin (100 mg/m2) and etoposide (100 mg/m2) was begun.


The patient was a 29 year old, with a previous bad obstetric history and an abnormal karyotype (46xx, t[3:9] [p21.3;p24], t [7:10] [p22;q22.1]), which necessitated the use of donor oocytes for the present conception. The reason for the use of frozen oocytes sourced from egg-sharing and egg-donation patients was the non-availability of compatible fresh donors and insistence of the couple to continue the treatment cycle. The couple was then counseled about the use of frozen–thawed oocytes and the expected low success rate for this cycle after obtaining informed consent.
The sourced oocytes were cryopreserved using the slow freezing method. Freezing was initiated 1–2 h post-retrieval, after denuding the cumulus with hyaluronidase and confirming metaphase-II maturity status. Cook's oocyte freezing media (Cook Medical, Queensland, Australia) was used after warming the required quantity to room temperature, the active cryoprotectants being propanediol in increasing concentrations from 0.75 to 1.5 mol/l (solutions 1 and 2 – for 7min and 30 s, respectively) and then transferred to solution 3 containing 1.5 mol/l propanediol and 0.2 mol/l sucrose [] for 5 min. The oocytes were loaded into pre-labeled straws (Cryo-Bio Systems, Rocket Medical PLC, Washington, England), plugged and placed into the cryochamber (Planer Kryo 360, Planer Products Ltd, Burnsville, USA). The initial chamber temperature at +20°C was slowly cooled to −8°C at the rate of −2°C/min. Seeding was manually performed at −8°C. After a hold time of 10min at −8°C, the straws were cooled slowly to −30°C at a rate of −0.3°C/min and then rapidly to −150°C at a rate of −50°C/min. After 10 min of stabilization, the straws were transferred to a liquid nitrogen tank and stored until thawing.
The oocytes were subjected to cryopreservation for 6 months. Because a suitable fresh donor was unavailable for this particular patient, we were able to use the cryopreserved oocytes for this couple. The endometrium was prepared with incremental doses of estrogen (Estradiol Valerate 2 mg; German Remedies, Mumbai, India) commencing from Day 2 or Day 3 of the cycle to a maximum of 8mg/day with initiation of micronised progesterone 400mg thrice a day, from the day before thawing. The optimum endometrium for transfer was considered to be 10mm, which was observed in mid-cycle. We decided to thaw at least 10 oocytes in order to enhance the chances of achieving enough good-grade embryos for transfer, success having evaded us earlier. For the thawing process, the straws containing the oocytes were removed from the liquid nitrogen, air dried for 30s, and then immersed in a water bath at 30°C for 40s. The oocytes were first passed through solutions 1–2 containing cryobuffer supplemented with decreasing concentration of propanediol (1.0–0.5mol/l) and increasing concentration of sucrose (0.2–0.3mol/l) for 5 min at room temperature, respectively. The oocytes were then transferred to solution 3 (0.3 mol/l sucrose) for 10 min at room temperature followed by 37°C on a warmer plate.
A survival check carried out under the inverted microscope revealed eight oocytes that survived the thawing process []. Oocytes with intact zona, clear perivitelline space, and visible polar body were considered as normal and were further cultured in the Cleavage media (Quinn's, SAGE In vitro fertilization, Trumbull CT, USA) at 37°C and 5% CO2 for 2 h before injection. Subsequently, the oocytes were injected with frozen–thawed and prepared sample of husband's sperms. Intracytoplasmic sperm injection [Figures and ][–] was the preferred method for fertilization, owing to an expected zona hardening following oocyte cryopreservation. The injected oocytes were further cultured in cleavage media in a Trigas incubator until Pronucleus check 16–18h later. Five embryos (two and three cells, grade II–III) [Figures and ] were observed and transferred on Day 2 using a Labotect catheter (Labotect GmbH, Gottinggen, Germany). The assessment of embryo grading was performed as described by Veeck L.[] Why five and not the recommended three embryos? Well, considering our previous experience of inadequate embryos for transfer following thawing and negative result for pregnancy, we decided to transfer at least five of this crop, which, as we mentioned, did not have the conventional cleavage or grade as expected (i.e., four cells, grade I–II). Once the serum β-HCG was positive, an ultrasound on the 38th day revealed a single intrauterine gestational sac. Since then she progressed uneventfully, with early fetal screening tests being normal until the 5th month, when she was diagnosed with mild pregnancy-induced hypertension and was treated with anti-hypertensives. During her level-2 scan at 22 weeks, where the fetal screening was normal, a shortened cervix of 1.5 cm was imaged, suggestive of cervical incompetence. Since then, she was admitted with us for careful surveillance of both the mother and the baby. She did moderate physical activities and her blood pressure was well controlled with anti-hypertensives. An elective cesarean was performed at 35–36 weeks on 27/8/2008 owing to severe oligohydraminos. She delivered a healthy male baby weighing 2.54 kg []. Both the mother and the baby were discharged uneventfully. A general follow-up at 3 months showed normal development for age [].


A 41-year-old woman presented with a three-year history of a painless mass in the region of the right parotid gland. A physical examination revealed a non-tender, firm and slightly mobile mass without clear borders that was palpable in the right parotid region. The overlying skin was indurated and warm. A clinical examination of the neck revealed no lymphadenopathy. Facial nerve function of the patient was normal on both sides.
CT of the maxillofacial region was performed using a 16-slice multidetector CT (MDCT) scanner (Sensation 16, Siemens Medical Systems, Erlangen, Germany) with unenhanced and enhanced scans. CT scanning was performed using parameters of 120 kV, 200 mAs and 5-mm collimation. An automatic injector was used to inject 90 mL of Iopamiro into the antecubital vein at a rate of 3 mL/s. An unenhanced CT image demonstrated the presence of a 5-cm round mass that was heterogeneous with central lower density and showed a poorly defined margin without calcification or invasion of the ambient structures in the superficial lobe of the right parotid gland (). After intravenous injection of contrast material, the mass demonstrated heterogeneous attenuation with peripheral enhancement and central irregular non-enhancement. At the periparotid areas, multiple, smooth margins and well-defined nodules with similar CT features were demonstrated in the subcutaneous fat of the right cheek and parapharyngeal spaces (). No evidence of an abnormality was found by the use of chest radiograph and abdominal ultrasonography.
Surgical excision of the total right parotid gland and the nodules at the periparotid areas were performed. All facial nerve branches were preserved. The gross appearance of the resected specimen for the large mass showed a tan-colored, lobulated and solid tumor that measured 5.6 × 5.5 × 3 cm (). A histopathological examination of the mass showed many thin-walled branching vessels surrounded by proliferative, relatively monomorphic oval-to-spindle shaped myoid cells in the peripheral area, but hyaline degeneration in the central area had no vessels (). There were not malignant features such as nuclear anaplasia, increased mitoses and infiltrative growth. Immunohistochemical staining was positive for smooth muscle actin (SMA) (), but staining was negative for CD34 (cluster of differentiation 34) and desmin. Based on these findings, this tumor was diagnosed as a benign myopericytoma that arose from the parotid gland. A histopathological examination of the periparotid nodules showed increased folliculus lymphaticus and proliferative lymphocytes that did not contain tumor cells, which indicated the presence of reactive lymphadenopathies.
The patient was alive without any discomfort as examined during follow-up at nine months after surgical excision of the mass. A follow-up CT examination performed at nine months showed no evidence of any residual or recurrent tumor in the right parotid gland region.


A 43-year-old woman positive for hepatitis B surface antigen was diagnosed as having hepatocellular carcinoma (HCC) with two intrahepatic tumor nodules, one nodule 2 cm in diameter in the left lateral segment of the liver and the other nodule 3.5 cm in diameter in the dome. The patient underwent one session of transarterial chemoembolization (TACE), followed by percutaneous ethanol injection targeting the area of the residual viable tumor in the liver dome. However, two months later, a viable portion was found to be present at the margin of the nodule in the hepatic dome and the patient was referred to our center. At that time, the laboratory test results included an aspartate transaminase (AST) level of 86 IU/L (reference, 0-40 IU/L), alanine transferase (ALT) level of 88 IU/L (reference, 0-40 IU/L), total bilirubin level of 1.0 mg/dL (reference, 0.2-1.2 mg/dL) and alpha-fetoprotein level of 1,460 ng/mL (reference, 0-20 ng/mL). RFA was performed percutaneously under ultrasonographic guidance, with the use of local anesthesia and conscious sedation. A single, 17-G, internally cooled electrode (ValleyLab, Burlington, MA) was used and radiofrequency current was emitted for 12 minutes with a 200 W generator set to deliver maximum power with the automatic impedance control method. Two ablations were performed with the use of the same method. A follow-up CT scan obtained immediately after RFA showed complete ablation of the HCC without direct evidence of diaphragmatic injury except for a small amount of reactive pleural effusion (). The patient reported pain at the right upper quadrant of the abdomen and right shoulder after RFA and the pain gradually improved over five days with the administration of analgesics. The patient developed a 38.0℃ fever on the next day after RFA, which subsided after one day. Two months after RFA, the patient revisited our hospital with a complaint of a productive cough with large amounts of green-yellow sputum (up to 200 mL per day), accompanied with a 38.4℃ fever with chills and right upper quadrant abdominal pain. Decreases in breath sounds on the right lower lung field and slight tenderness to percussion of the right upper quadrant of the abdomen were noted on a physical examination. Icteric sclera or hepatosplenomegaly was not noted. Plain chest radiograph demonstrated the presence of an ill-defined consolidation in the right lower lobe with right-sided pleural effusion. An abdominal CT scan showed the presence of a liver abscess at the RFA site and pneumonia, with a small abscess in the right lower lung, suggestive of a focal diaphragmatic defect with communication between the lung and liver abscesses (). Ultrasonography-guided percutaneous drainage of the liver abscess with a 10.2 Fr pigtail catheter was performed and 10 ml of thick dark-yellowish pus mixed with necrotic debris was drained. Contrast material injection via the needle demonstrated the presence of opacified cavities of the lung and the bronchial tree communicating with the biliary tree, which was consistent with a BBF (). The patient coughed vigorously when the above contrast study was performed. No organism other than Bacillus spp, which is a common contaminant, was isolated from a culture of the drainage material. Following catheter insertion and treatment with antibiotics, symptoms including fever and bilioptysis were markedly improved. Bile mixed with pus (20 to 100 ml daily) was drained initially, but the rate of drainage gradually decreased to less than 10 ml per day over a month. Follow-up abdominal CT scans obtained five weeks after catheter insertion showed the presence of a small residual lesion with low attenuation in the hepatic dome around the catheter, and repeated cholangiography confirmed no leakage of contrast material through the fistula. The pigtail catheter was therefore removed and antibiotic treatment was discontinued. However, fever and productive cough with sputum containing bile resumed the day after removal of the pigtail catheter. A new cavitary lesion with low attenuation and parenchymal consolidation in the right basal lung were observed on CT images, suggesting reopening of the BBF. A pigtail catheter was reinserted into the remnant abscess in the liver through the previous drainage tract. Percutaneous tubography showed filling of the contrast material in the right lower lobe of the lung, with contrast material leakage into the right subphrenic space, indicating the presence of a BBF although there was no definite communication between the biliary and bronchial systems (). Thereafter, the amount of percutaneous drainage decreased gradually and the lesions in the liver and lung shrank significantly, as shown on follow-up CT scans three weeks after reinsertion of the pigtail catheter (). As there was no drainage for a week and the patient did not complain of any fistula-related symptoms, the pigtail catheter was removed. Four weeks after removal, there was no evidence of a BBF or other related lesions on CT images. Two months later, the patient underwent a repeat session of RFA for a recurrent HCC in segment 7 without any treatment-related complications, including a BBF.


A 66-year-old male presented with a history of sudden ocular pain and redness in OD for 10 days. The patient had a history of a hypermature cataract in the right eye 7 years previously. He denied recent trauma in OD. No inflammatory antecedents were reported. Best corrected visual acuity in OD was hand motion close to the face, while OS was 0.8. Slit lamp examination of OD showed conjunctival congestion, moderate corneal edema and a deep anterior chamber with lens particles at the inferior angle (). There was a chamber reaction with flare (2+) and cells (2+). Numerous large white flecks were observed circulating in the aqueous. Lens cortex material was noted to protrude through the dehiscence site of the anterior capsule. The pupil was mid-dilated and adhered to the disrupted anterior lens capsule. The lens showed the presence of a hypermature cataract.
The intraocular pressure (IOP) measured with Goldmann applanation tonometer was 55 mmHg in OD and 17 mmHg in OS.
Gonioscopy with Goldmann 2-mirror goniolens demonstrated the presence of wide-open angles and lens particles that were thickly attached to the inferior trabecular meshwork (). There was no sign of angle recession, and no corneal or scleral perforation was noted in OD. Following dilation of the pupils, slit lamp examination of the anterior segment showed fragmented lens particles protruding through the ruptured anterior capsule margin. Evaluation of the anterior capsule of the lens showed that there was a pre-existing anterior capsular dehiscence, extending along the pupillary margin. This was associated with an intracapsular distension, and protrusion of the anterior sub-capsular lens particles through the dehiscence into the anterior chamber. Phacodonesis was not observed. The fundus was invisible due to the hypermature cataract. The examination of the left eye was within normal limits. Ultrasound B-scan of the right eye was within normal limits.
A diagnosis of right eye lens-particle glaucoma was made. Medical therapy for the elevated IOP included 250 mL of 20% mannitol (1 g/kg body weight) intravenously over 45 minutes in the acute phase, followed by Acetazolamide 250 mg tablet four times daily, Dorzolamide 2%/timolol 0.5% eye drops twice daily and brimonidine 0.15% eye drops twice daily for maintenance of IOP control. Cataract surgery was recommended for the treatment of lens-particle glaucoma. An anterior chamber paracentesis was performed, and a 0.2-mL sample of the white material in the aqueous was obtained.
The patient underwent an extracapsular cataract extraction. A posterior lens capsule rupture which might have been produced during surgery was confirmed intraoperatively. Anterior vitrectomy was performed with an automated vitrector (Alcon. Accurus® 400VS) for the vitreous prolapse and for the removal of the sticky cortical matter in the anterior vitreous and anterior chamber angle. Histological examination of the anterior chamber aspirate revealed lens cortical fibers, numerous hemosiderin laden macrophages and neutrophils (). No bacterial analysis, florescence stain or culture with the aqueous humor sample was done.
Postoperatively, the patient was treated with topical moxifloxacin 0.5%, prednisolone 1% eye drops in a tapering dose regimen and atropine 1% eye drops twice daily for 2 weeks. At the 1-month postoperative follow-up, the right eye IOP was 16 mmHg without any intraocular antihypertensive medication.


A 20-year-old female was presented with a mild left flank pain of one-week duration, with no associated history of hematuria or any other systemic symptoms. Physical examination revealed a large nontender lump involving left lumbar and left hypochondriac region.
Computed tomography of the abdomen and pelvis showed a large heterogeneously enhancing mass of 14.3 × 9.4 × 8.5 cm over the mid-region and lower pole of the left kidney with areas of necrosis within it. An iso-to-hypodense heterogeneously enhancing thrombus was noted in the left renal vein and adjacent portion of the inferior vena cava [Figures and ].
Renal cell carcinoma was suspected pre-operatively. Intraoperatively, a tumour mass was seen replacing the whole kidney. Left radical nephrectomy was performed and left renal vein ligated flush with the IVC after milking the thrombus into the left renal vein. Macroscopic examination of the specimen revealed a tumor of size 12.8 × 11 × 4.5 cm, replacing the entire renal parenchyma, involving pelvi-calyceal system and medulla with thin rim of cortex seen all around. Tumor thrombus was seen within the lumen of left renal vein [].
Microscopically, the tumor was composed of spindle cells arranged in the intersecting fascicles, alternating with hypocellular areas, suggestive of monophasic SS [ and ]. On immunohistochemistry, tumor cells expressed bcl-2, calponin, and EMA. Both Mic-2 and CK were focally positive. Molecular analysis revealed a translocation between the SYT gene on chromosome 18 and SSX on chromosome X, which is consistent with the diagnosis of SS.


A 7-month-old girl presented with a prolonged period of chronic diarrhea. On admission she showed profound abnormalities in both cell-mediated and humoral immunities and severe combined immunodeficiency was diagnosed. Laboratory values were as follows: immunoglobulin (IgG), 173 mg/dL (normal range, 500-1300 mg/dL); IgM, 31 mg/dl (normal range, 50-199 mg/dL); IgA, 29 mg/dL (normal range, 23-190 ml/dL); T2/ T4/ T8 /B cell /Natural Killer (NK) cell, 4% / 4% / 0% / 2% / 90%.
At the age of 10 months she was readmitted due to left axillary and cervical lymphadenopathy; several courses of antibiotic treatment had failed to change the course of her illness. There was no family history of tuberculosis, but she had been vaccinated with BCG at the age of one month. A specimen for culture was obtained from the axillary lymph node, but bacteriological culture was negative. Numerous acid-fast bacilli were demonstrated on smears, and cultures revealed growth of Mycobacterium bovis, BCG strain. She was treated with isoniazid (15 mg/kg per day), rifampicin (15 mg/kg per day), and pyrazinamide (20 mg/kg per day). In spite of antituberculous therapy, her lymphadenopathy worsened.
At the age of 16 months, she was readmitted with persistent lymphadenopathy in the left axilla and cervical area, and reddish swelling of the right third finger. A chest radiograph revealed no abnormality of lung parenchyma or hilar lymphadenopathy, but a round osteolytic lesion was incidentally detected in the right distal humerus. A plain radiograph of both lower extremities revealed multiple, round, osteolytic lesions in the femora, tibiae, and fibulas (). Both tibiae showed extensive periosteal thickening along the entire diaphyses. Ultrasound examination of the abdomen showed enlarged lymph nodes containing an eccentric hypoechoic portion at the porta hepatis and hypoechoic nodules in the spleen (). Contrast-enhanced CT scanning demonstrated the presence of multiple nodules in the spleen, and portocaval lymphadenopathy with a central low-attenuated area ().


The patient was a 62-yr-old man who had undergone curative subtotal gastrectomy for gastric cancer 14 yr ago. He presented himself with signs of cachexia and complained of heartburn and epigastric discomfort for approximately 1 month before admission. The endoscopic examination revealed a recurred gastric adenocarcinoma. A total gastrectomy and Roux-en-Y esophagojejunostomy were performed with dissection of the multiple adhesions around the small sac and between the transeverse colon and liver.
Four days after the operation, the patient developed fever. He also showed consolidation in the left lower chest and increasing pleural effusion on chest radiography. Thoracostomy was performed. Because Klebsiella pneumoniae and Candida albicans grew in the cultures of sputum and pleural effusion, antibiotics treatment including intravenous cefepime and clindamycin was initiated. Although the regimen was changed to meropenem, the empyema on the left side did not improve, and decortication of the left lung was performed 30 days after the operation. After decortication, no more microorganisms were isolated from pleural effusion. However, methicilin-resistant coagulase-negative staphylococci was recovered from the exudates of the tube insertion site. Despite the vancomycin treatment, the wound did not improve, and marginal resection of the tube insertion site was performed 15 days after decortication. After marginal resection, vancomycin-resistant enterococci (VRE) was recovered in the culture of exudates from the wound site. The abdominal computed tomography showed multiple abscesses in the left upper abdomen at that time. Two days after the marginal resection, the patient underwent re-exploration of the left thoracic cavity due to a hemothorax from intercostal arterial bleeding at the resection site. The laboratory findings showed white blood cell count of 19,100/µL with a neutrophil predominance (85%), increased ESR (72 mm/hr) and CRP (28.34 mg/dL). In the culture of blood and exudates from the pleural cavity obtained during exploration, both VRE and V. cholerae were recovered. The patient was treated with linezolid, imipenem and levofloxacin. VRE and V. cholerae were also isolated in the patient's stool. No more pathogens were isolated on follow-up cultures of pleural effusion, rectal swab and stool, and the laboratory findings were normalized.
V. cholerae isolated from the pleural effusion and stool formed white beta-hemolytic colonies on sheep blood agar and yellow colonies on thiosulfate-citrate-bile sucrose agar. It was identified as V. cholerae by the MicroScan Gram-negative Combo panel (Dade International Inc., Califonia, U.S.A.) and API 20E (bioMerieux, France). It was susceptible to amikacin, ceftazidime, ceftriaxone, cephalothin, ciprofloxacin, gentamicin, imipenem, piperacillin, tobramycin, trimethoprim-sulfamethoxazole and cefepime. The PCR for the cholera toxin gene was performed with primers, 5'-ACAGAGTGAGTACTTTGACC-3'and 5'-ATACCATCCATATATTTGGGAG-3', which did not yield a PCR product at 307 bp. By agglutination test for serogrouping, the isolate was finally confirmed as a non-cholera toxin-producing, non-O1, non-O139 V. cholerae.


A 36-yr-old woman visited in our infertility clinic, complaining of primary infertility for 1 yr. Her menstrual cycle was irregular, about 60-120 days, body mass index was 27.5 (body weight: 73 kg, height: 163 cm). She had a history of explo-laparotomy due to endometriosis. Neither she nor any other members of her family had diabetes or hypertension. Abdomino-pelvic examination showed the unremarkable findings and transvaginal ultrasonogram showed small uterine myoma, 24-21 mm, normal contour of endometrium, 10 mm, and well defined normal both ovaries. Full infertility work up was performed, results of semen analysis was normal, hormonal study (LH, FSH, Estradiol, Prolactin, TSH, Testosterone, DHEA-S) showed normal findings except high FSH level of 9.66 mIU/mL. The office endometrial aspiration biopsy showed secretory phase and finding of luteal phase defect. Hysterosalpingogram revealed one point filling defect in the uterine cavity and mild dilatation of ampullary portion of Fallopian tubes, but good intraperitoneal spillage (). On laparoscopic examination, peritubal adhesion was detected and adhesiolysis was performed. On hysteroscopic examination, there was a polypoid mass on fundal area and hysteroscopic biopsy and curettage was done. The pathologic evaluation of endometrial tissue revealed well differentiated endometrial adenocarcinoma (Grade 1, ) with positive estrogen and progesterone receptors. After curettage, we confirmed that there was no detectable endometrial mass or myometrial invasion or cervical involvement by MRI imaging (). Final diagnosis was primary infertility accompanying endometrial carcinoma, FIGO grade I without myometrial invasion.
After counseling, the patient wished to retain her ability to conceive, so high dose progestin therapy was recommended. Oral megestrol acetate was prescribed 600 mg daily for 8 weeks. On follow-up hysteroscopic examination, she still got a small protruding mass on fundal area. We performed hysteroscopic biopsy and endometrial curettage. The pathologic evaluation of endometrium revealed complex hyperplasia without cellular atypia (). She continued to receive megestrol acetate but complained intolerable weight gain from 73 to 88 kg. We decided to reduce the dose to 400 mg, daily for 4 weeks, then 320 mg, daily for 12 weeks. After megestrol medication of total 71,680 mg during 24 weeks, we found smooth atrophic endometrium by hysteroscopic examination, and this was confirmed as atrophic endometrium through the histological examination of tissue obtained by curettage. Then we decided to perform IVF program in order to optimize the patient's potential for fertility and achieve a viable pregnancy without further delay.
After pituitary down-regulation by the GnRHa (Buserelin acetate, Superfact®, Hoechst), follicle-stimulating hormone preparation (Follimon®, LGIC, Korea) was administrated, 375 IU daily for 12 days. The follicular growth was monitored from gonadotropin day 5 by 3 days interval and human chorionic gonadotropin (IVF-C®, LGIC, Korea) 10,000 IU was administrated when the at least 3 or more follicles reached 18 mm in diameter. And 35 hr later, oocytes aspiration was performed under the guidance of transvaginal ultrasonography. Two mature oocytes were retrieved and inseminated with her husband's prepared spermatozoa. Fifty seven hours after oocyte retrieval, the two high grade 4-cell embryos were transferred. Measurement of β-hCG revealed 3,449 mIU/mL on 19 days after embryo transfer, and on 23 days after embryo transfer, intrauterine single gestational sac with yolk sac and right tubal pregnancy were identified by transvaginal ultrasonogram. Right salpingectomy was performed under the epidural anesthesia, on 27 days after embryo transfer, we could detect the fetal pole with heart beat in the intrauterine gestational sac after that procedure. Then she received routine obstetrical care and delivered by Cesarean section at 38 weeks in gestational periods. The baby was female, 2,590 g, and the Apgar score was 8 at 1 min, 9 at 5 min. Placenta was grossly and microscopically normal, there was no evidence of residual tumor in the uterus and adnexa or abdominal organ.
Twelve weeks after delivery, endometrial thickness measured by transvaginal ultrasonography was 4 mm and endometrial biopsy revealed no evidence of recurrent disease. Two years after delivery, she was healthy without any evidence of recurrent disease.


A 51-year-old man with known mild, bilateral nonproliferative diabetic retinopathy was referred for evaluation of CRAO that had developed in his left eye 72 hours prior to presentation. His past medical history included systemic arterial hypertension and diabetes mellitus for the past 15 years.
On examination, his best-corrected visual acuity was 0.6 in the right eye and hand motion in the left eye. Intraocular pressure (IOP) was 14 mmHg bilaterally by Goldmann applanation tonometry. Anterior segment evaluation showed no NVI in the left eye, and both eyes had open angles. Fundus examination showed midperipheral retinal hemorrhages in both eyes. In the left eye, the retina was pale with attenuated arteries and a cherry red spot at the posterior pole. An intravenous fluorescein angiogram showed a delayed arm-to-retina time of 25 seconds and incomplete retinal venous filling throughout the test (>6 minutes). Choroidal perfusion was normal. Noninvasive carotid examination delineated 70% stenosis in the left proximal internal carotid artery and plaques in both internal carotid arteries.
Five weeks later, on routine follow-up examination, he returned complaining of ocular pain in his left eye for the previous three days. The visual acuity in the left eye had decreased to no light perception, and the IOP was 45 mmHg. Neovascularization of the iris and angle was noted in the left eye. The retinal whitening and cherry red spot were still present.
Despite maximal tolerable medical therapy to control intraocular pressure, the patient experienced marked discomfort. Treatment options were explained in detail, and after the patient gave consent, an intravitreal injection of 0.75 mg (0.03 ml) of bevacizumab was performed in a sterile fashion without anterior chamber paracentesis. One week later, there was a complete regression of the NVI, and the IOP had decreased to 25 mmHg with two topical antiglaucoma medications (dorzolamide/timolol fixed combination and brimonidine). PRP consisting of a total of 1200 burns was administered in three sessions starting two weeks after the injection. Over the next three months, recurrent NVI developed, and the patient received another IVB injection and additional PRP. Over the course of treatment, the patient received 2000 burns in all. Two months after the additional combination therapy, there was no recurrence of NVI, and the IOP was 21 mmHg with the same medications.


A 59-year-old man with a ten-year history of diabetes mellitus was referred for evaluation of decreased vision in the previous two weeks and ocular pain for three days. He was normally followed for mild diabetic retinopathy in both eyes for the past two years.
On examination, his best-corrected visual acuity was no light perception in the right eye and 0.8 in the left eye. IOPs were 40 mmHg in the right eye and 18 mmHg in the left eye by Goldmann applanation tonometry. Anterior segment evaluation revealed neovascularization of the iris and angle in the right eye. Fundus examination showed CRAO characterized by retinal whitening with attenuated arteries and a cherry red spot at the posterior pole in the right eye. Both eyes had midperipheral retinal hemorrhages. Fluorescein angiography showed normal choroidal filling. Retinal arterial filling began at 30 seconds after injection, and the retinal vein was not completely filled even late in the angiogram. A noninvasive carotid examination revealed 70% stenosis of the right internal carotid artery and 50% stenosis of the left internal carotid artery.
After obtaining informed consent, bevacizumab (0.75 mg/0.03 ml) was injected. One week after the injection, the NVI had regressed, and the IOP was 18 mmHg/AT with two topical antiglaucoma medications (dorzolamide/timolol fixed combination and brimonidine). PRP was started two weeks after the injection. The patient received 1800 burns overfour sessions. Four months later, there was no recurrent neovascularization, and the IOP was 15 mmHg with one topical antiglaucoma medication (dorzolamide/timolol fixed combination).


A 75-year-old woman presented with a ten-day history of decreased visual acuity in her right eye. Her past medical history included diabetes mellitus for the last five years.
Her visual acuity was hand motion in the right eye and 0.9 in the left eye. IOPs were 14 mmHg in the right eye and 12 mmHg in the left eye by Goldmann applanation tonometry. There was no anterior chamber reaction or NVI in the right eye. The right fundus showed a CRAO characterized by retinal opacification of the posterior pole with a cherry red spot. No clinical evidence of diabetic retinopathy was present in either eye. Fluorescein angiography showed sluggish retinal arterial flow and delayed retinal venous filling. Noninvasive carotid examination showed no evidence of obstruction.
Two weeks later, the patient returned for a follow-up examination. Her visual acuity in the right eye remained hand motion. However, the right eye now exhibited NVI and had an IOP of 40 mmHg. The retinal opacification was still present. After obtaining informed consent, bevacizumab (0.75 mg/0.03 ml) was injected. One week after the injection, the NVI had regressed, and the IOP had decreased to 22 mmHg with two topical antiglaucoma medications (dorzolamide/timolol fixed combination and brimonidine). PRP was performed in the right eye two weeks after the injection. The patient received 1600 burns over four sessions. Four months after combination therapy, the visual acuity remained hand motion with no recurrent neovascularization. The IOP was 17 mmHg with one topical antiglaucoma medication (dorzolamide/timolol fixed combination).


A 59-year-old woman was admitted to the emergency room of a local hospital with the sudden onset of severe chest pain radiating to her back. She had a history of hypertension and a cerebrovascular accident. At the time of presentation, she had a drowsy mental status. The cardiac enzymes were elevated on baseline laboratory testing. A chest film showed mediastinal widening (). The initial electrocardiogram (ECG) showed a normal sinus rhythm without significant ST-T changes (). An emergent chest CT scan was performed under the suspicion of an acute aortic dissection, which showed an acute Stanford type A aortic dissection (). The patient was then transferred to our institution for repair of the aortic dissection. She was hemodynamically unstable.
Emergent ascending aortic replacement surgery using a graft was performed with the patient under general anesthesia using a median sternotomy. Upon opening the pericardium, a hemopericardium was noted. The aortic root was incised, and an intimal tear was identified just above the sinus of Valsalva. The dissection had not extended to the orifice of the left coronary artery. We carried out an ascending aortic replacement with a 28-mm Hemashield® graft. Systemic circulation was then restarted. ST segment elevation was observed on the ECG monitor immediately postoperatively. Sedation and mechanical ventilation were maintained, and she was transferred from the operating room to the intensive care unit (ICU).
In the ICU her blood pressure and cardiac output remained low in spite of sufficient inotropic support. An ECG showed ST segment elevation in leads II, III, and aVF, and in the precordial leads, suggesting broad myocardial ischemia (). In addition, transthoracic echocardiography showed regional wall motion abnormalities in the territories of the left anterior descending artery (LAD) and left circumflex artery (LCx), and moderately decreased LV systolic function. These results suggest coronary artery malperfusion, probably caused by progression of the aortic dissection into the left coronary artery. Accordingly, emergent coronary angiography was performed 90 minutes postoperatively.
Coronary angiography revealed no significant luminal narrowing in the right coronary artery, but almost complete collapse of the lumen of the LAD (). After forceful contrast injection into the LAD, the collapsed lumen was reopened, but it subsequently recollapsed without forceful contrast injection. A similar finding was observed in the LCx (). We reasoned that this life-threatening coronary artery occlusion was caused by pulsatile compression of the false lumen of the left coronary artery dissection. A zotarolimus-eluting stent was deployed in the mid-portion of the LAD and a bare metal stent (Tsunami®, 3.5×30 mm) was deployed in the proximal portion of the LAD. Three bare metal stents (Driver®, 3.0×30 mm; Tsunami®, 3.0×30 mm; and Driver®, 3.0×18 mm) were successfully deployed from the distal portion to the proximal portion of the LCx. However, coronary blood flow was not improved. Thus, direct stenting at the left main stem, and a bare metal stent (Driver®, 3.5×18 mm) was successfully deployed. The final angiogram of the left coronary artery showed Thrombolysis In Myocardial Infarction (TIMI) flow grade 3 in the LAD and LCx.
Follow-up coronary angiography was performed 45 days after percutaneous coronary intervention (PCI) because the patient remained unstable. Dyspnea was New York Heart Association (NYHA) class III/IV and follow-up two-dimensional echocardiogram (2D-UCG) revealed a severe decrease in LV systolic function. Follow-up coronary angiogram showed significant in-stent restenosis at the proximal portion of LAD (). An intravascular ultrasound (IVUS) study showed the remaining coronary artery dissection in the diagonal branch of the LAD (). Repeat balloon angioplasty using a 4.0×20 mm balloon (Apollo®) was performed at the site of in-stent restenosis. The final angiogram of the LCA showed TIMI flow grade 3 in the LAD and LCx. Her clinical course following the repeat PCI was stable and uneventful. The patient was discharged from hospital 51 days after the surgical repair. A follow-up coronary angiogram was performed 8 months after the PCI and showed no significant in-stent restenosis ().


A 41-year-old male with a history of cigarette smoking sought evaluation at the emergency room due to pain in the anterior chest and epigastrium of 35 hours duration. At the time of onset of the chest pain, he was isolated on an island and unable to access transportation for transfer to the hospital. On registration in the emergency department, the patient's vital signs were as follows: temperature, 37.1℃; heart rate, 116 beats per minute; blood pressure, 130/90 mmHg; and pulse oximetry, 98% on room air. The electrocardiogram (ECG) showed marked ST segment elevation in the precordial leads (). The results of laboratory tests were notable for a white blood cell count of 1,7540/mm3 and elevated cardiac biomarkers. The C-reactive protein was elevated to 151.54 mg/dL. An echocardiographic examination revealed akinesia of the left ventricular septum and severe left ventricular dysfunction. He subsequently underwent coronary angioplasty and stenting of the left anterior descending artery using two sirolimus-eluting stents ().
One day after the PCI, the patient complained of severe pleuritic chest pain radiating to both shoulders and the lower jaw. On physical examination, pericardial friction rubs and a crunching sound were heard over the precordium. The erythrocyte sedimentation rate was elevated to 120 mm/h. A repeat echocardiogram was performed, and the results were unchanged. Computed tomography (CT) showed small gas bubbles around the aortic wall and mild pericardial thickening with subtle air densities suggesting acute mediastinitis (). Based on these results, the patient was diagnosed with PCIS and acute mediastinitis, and was given oral ibuprofen and broad-spectrum intravenous antibiotics (ceftriaxone, aminoglycoside, and lingcosimide). The patient began to feel some subjective improvement within 24 hours and the pericardial friction rub was no longer heard on physical examination. The antibiotics were terminated on the 6th day. A chest CT was repeated; the previously noted soft tissue density lesion surrounding the ascending aortic wall and the subtle streaky fatty infiltrations with small gas bubbles had resolved. However, multiple low-density nodules were noted in the apex of the left ventricle, suggesting acute thrombi (). Thus, intravenous heparin with oral warfarin treatment was started. The patient was discharged from the hospital on the day 17 with persistent apical mural thrombi.


A 69-year-old male patient with chest pain and dyspnea (New York Heart Association class III) was admitted to the hospital. The patient blood pressure was 150/90 mmHg and the heart rate was 65 beats per minute. Electrocardiography revealed atrial fibrillation and negative T waves in the precordial leads. Transthoracic echocardiography revealed asymmetric septal hypertrophy (maximal thickness, 36 mm) and apical hypertrophy (maximal thickness, 18 mm) with a normal ejection fraction (70%). This was compatible with mixed type hypertrophic cardiomyopathy. Severe dynamic LVOT obstruction (LVOT peak velocity=4.6 m/s, peak pressure gradient=85 mmHg) was also noted (). Moderate eccentric mitral regurgitation was induced by systolic anterior motion of the anterior mitral valve leaflet and prolapse of the posterior leaflet of the medial segment. The effective regurgitant orifice area measured 0.34 cm2, and there was no evidence of chordal rupture. The patient was treated with oral beta blockers and was discharged in stable condition.
The patient presented to the emergency department 1 year later due to sudden dyspnea (New York Heart Association class IV). Blood pressure was 88/50 mmHg, and electrocardiography demonstrated atrial fibrillation with a rapid ventricular response (145 beats per minute). Chest radiographs revealed cardiomegaly and pulmonary edema of both lung fields. Transesophageal echocardiography revealed flail motion of the medial segments of both mitral leaflets (P3 and A3 segments) with chordal rupture (). Severe mitral regurgitation occurred secondary to the rupture, and the effective regurgitation orifice area increased to 0.98 cm2 (). Systolic anterior motion of the mitral leaflet and dynamic LVOT obstruction completely resolved (). However, aggravated symptoms did not respond to medical management. Concomitant mitral valve replacement, septal myectomy, and the Maze procedure were successfully performed. Spontaneous chordal rupture of the middle segment of the anterior mitral leaflet (A2) and the posteromedial commissure were confirmed during surgery (). Histopathology revealed myxomatous degeneration of the mitral valve without evidence of infective endocarditis. Normal sinus rhythm was maintained postoperatively. Follow-up echocardiography demonstrated a functional prosthetic mitral valve without dynamic LVOT obstruction. The patient was discharged in stable condition with remarkable clinical improvement.


In March 2004, a 50-yr-old woman was admitted to our hospital complaining of epigastric pain and severe anterior chest pain of 1 week duration. She was without any significant prior medical history. An electrocardiogram and an echocardiogram showed no significant abnormalities of the heart. We concluded that the chest pain was atypical and that it did not originate from the heart. Gastroendoscopic examination revealed a geographic mucosal irregularity at the antrum (), and we performed a biopsy at the site. The pathology examination showed undifferentiated anaplastic neoplasm. Endoscopic ultrasonography showed the mucosal thickening (), and the computerized tomography scan of the abdomen showed no evidence of distant metastasis. We performed follow-up gastroendoscopic examination after 2 weeks. The lesion was then more aggravated; her pain was persistent and did not respond to H2-blocker or proton pump inhibitors. She underwent total gastrectomy and the dissection of 16 regional lymph nodes.
The gross finding of the resected stomach tissue revealed an 8×4.5 cm well-demarcated ulceroinfiltrative lesion at the pyloric antrum along the lesser curvature (), which infiltrated into the submucosal layer. There were many enlarged lymph nodes. The microscopic examination of the resected stomach tissue revealed a diffuse and solid proliferation of large atypical cells with pleomorphic nuclei. Some giant tumor cells were confined in the mucosa-submucosa area, and they showed multiple nuclear lobulations ().
An immunohistochemical study was performed on the formalin-fixed, paraffin-embedded surgical specimens by the streptavidin-biotin-peroxidase complex method (LSAB kit; DAKO, Japan). The tumor cells were positive for CD30 (), vimentin and CD3, but they were negative for cytokeratin, carcinoembryonic antigen (CEA), epithelial membrane antigen (EMA), CD45, CD20, CD45RO and anaplastic lymphoma kinase (ALK) (). Eight of the sixteen lymph nodes contained metastasized tumor.
The patient had no B symptoms, and there was no bone marrow involvement. She had no palpable peripheral lymph nodes and there were no abnormal findings on chest radiography. The initial laboratory test results were as follows: white blood cell count, 4,300/µL; hemoglobin level, 10.3 g/dL; platelet count, 196,000/µL; lactate dehydrogenase level, 716 IU/L (240-460 IU/L). The serum calcium level, aspartate aminotransferase level and alanine aminotransferase level were normal. Her ECOG performance status was 1.
We diagnosed her as having CD30-positive primary gastric ALCL IIE, and we started chemotherapy with cyclophosphamide, adriamycin, vincristine and prednisolone. After two cycles of chemotherapy, prolonged leukopenia was noted. We then performed a bone marrow examination. The bone marrow revealed a hypocellular marrow (the cellularity was 10%) without evidence of tumor involvements. We decided to stop any further chemotherapy and the patient was closely followed up. At 7 months after diagnosis, the patient remains alive and without tumor recurrence.


A 13-month-old boy, who had suffered from fever, generalized rash, and multiple joints swelling for four months, visited pediatric rheumatology clinic. At admission, fever, which had shown an intermittent high pattern, nearly subsided, but salmon pink-colored rheumatoid rash was diffusely present on his abdomen. His hands, lower legs and feet were bilaterally swelled with the involvement of metacarpal and proximal interphalangeal joints of second fingers, knee joints, and fifth toes (). Physical examination revealed cervical lymphadenopathy and hepatosplenomegaly. Under the diagnosis of S-JRA, he had been treated with aspirin (100 mg/kg for nine days) and ibuprofen at local hospital until one week before admission.
Laboratory results of rheumatoid factor, anti-streptolysin O, lupus anticoagulant, and antinuclear antibody were all negative. Complement and immunoglobulin data were: C3, 199 mg/dL; C4, 33.5 mg/dL; IgG, 766 mg/dL; IgA, 112 mg/dL; and IgM, 81.5 mg/dL. There was no evidence of viral infection or hepatitis. Other laboratory findings at admission are presented in .
Bone marrow (BM) examination, which was performed as a further work-up of hematologic abnormalities showed normocellular marrow with a cellularity of 90%. Granulocytic and megakaryocytic lineages were normal in maturation, but erythroid lineage was hypoplastic. Benign-looking macrophages were remarkably increased, and some of them showed hemophagocytic features. Their presence was confirmed with CD68 immunostain ().
He was diagnosed as having MAS. Immunosuppressive therapy with methylprednisolone (2 mg/kg/day for three days, and switched to oral prednisolone) and cyclosporin (2.5 mg/kg/day, under continuous medication) was started from the third and the ninth hospital days, respectively. Clinical and laboratory findings improved with decreased hepatomegaly and joints swelling at the time of discharge (-). He has remained stable without relapse of MAS during the follow-up period of six months.


A 73-year-old woman attended our hospital emergency department after a sudden onset of severe abdominal pain and fever. A physical examination revealed a high fever (39℃), a chilling sensation, abdominal tenderness, and muscular defense. The patient had none of the following symptoms: diarrhea, body-weight loss, history of malnutrition, or food intolerance. We could not detect any palpable cervical lymph node.
The laboratory tests on admission showed mild leukocytosis (white blood cell count of 13,050/mm2) and azotemia (serum blood urea nitrogen/creatinine of 18/1.2 mg/dL). Peripheral blood morphology indicated normocytic normochromic anemia (hemoglobin of 9.9 g/dL) with anisopoikilocytosis, and the patterns of serum electrophoresis were nonspecific. A chest radiography revealed no definite free air under the diaphragm. Abdominal computed tomographic imaging showed an 8.5 cm length of wall thickening in the distal small bowel, with central necrosis and pseudoaneurysmal dilatation. Within the pelvic area, multiple homogeneous enhancing lymphadenopathies were present on the mesentery near the mass (). In light of these factors, we planned a laparoscopic exploration for a suspected malignant gastrointestinal stromal tumor (GIST).
The laparoscopic findings showed localized peritonitis arising from the perforation of the terminal ileum and that the lesion was adherent to the bladder and the sigmoid colon. We performed a segmental resection of the perforated ileum and side-to-side anastomosis through a minilaparotomy after a meticulous dissection. The central wall of the resected ileum was diffusely thickened and it showed a relatively well-defined encircling the mural mass measuring 10.0 cm in length. The mass was gray-white to tan and indicated hemorrhage and necrosis ().
In the pathology report, immunohistochemical staining was positive for CD5, CD3, CD56, CD8, and Ki-67, and negative for CD20, CD15, and the Epstein-Barr virus (). The T-cell nature of the tumor was reflected in its reactivity for CD3 and CD8, and its negativity for the B-cell marker CD20. The patient expressed the HLA-DQ6 and HLA-DQ9 alleles. On the basis of the immunohistochemical and microscopic findings, we made a diagnosis of ETTL.


A 73-yr-old woman presented with multiple trauma and chest pain after a traffic accident. Traumatic aortic injury was suggested by a chest radiography, which showed mediastinal widening and left pleural effusion. A spiral computed tomography (CT) scan revealed an extraluminal hematoma in the distal aortic arch with a contained aortic rupture (). Further examination showed no other aortic lesions. However, the patient had concomitant comminuted fractures in the right tibia, left femur, and the radius bilaterally, which required surgery. Surgery for the TAR was therefore delayed for about 1 month to allow orthopedic postoperative recovery. To allow a minimally invasive surgery, a hybrid aortic stent-grafting procedure was performed, consisting of a left subclavian artery (LSCA) debranching procedure combined with proximal descending thoracic aortic stenting. Median sternotomy for the debranching procedure was avoided by transposing the LSCA to the left common carotid artery (LCCA) through a 5 cm left supraclavicular incision. The LSCA and LCCA were dissected and freed from their surrounding attachments after moderate systemic heparinization with an intravenous (IV) of 5,000 IU. The proximal LSCA was clamped and transected, and the proximal stump was closed with a continuous running suture of 4-0 polypropylene. The LCCA was partially clamped and opened longitudinally, and the transected distal portion of the LSCA was attached in an end-to-side manner. The wound was closed in layers. The patient later recovered full neurologic function without deficits. On postoperative day 5, the interventional radiologist inserted a thoracic aortic stent percutaneously using the Seldinger technique. The common femoral artery was punctured under local anesthesia. A 5F-calibrated angiographic catheter was advanced into the aortic arch via the contralateral common femoral artery to determine the morphology and extent of the aortic rupture (). The delivery system was advanced under fluoroscopy after IV injection of 5,000 IU heparin until the tip reached the origin of the LCCA. Finally a SEAL® endoprosthesis (S & G Biotech, Korea) was inserted into the aortic arch to complete the procedure. A series of aortograms were performed during deployment to ensure accurate proximal and distal positioning. The proximal bare stent was placed precisely in the middle of the ascending aorta, allowing free blood flow to both arch vessels. Intraoperative angiography was used for post-procedural assessment of stent-graft positioning and endoleak (). An in-hospital thoracic CT scan on postoperative day 12 () showed successful exclusion of the rupture site, with no any signs of endoleak or graft-related complications. At the first visit to the out-patient department, the patient was doing well and showed good healing of the supraclavicular incisional wound ().


A 5-yr-old male was admitted with well-demarcated necrotic skin lesions and severe swelling on right ear lobe developed after mosquito bites (). In addition to cutaneous lesion, he also had a high temperature. He had suffered several similar symptoms since last summer, which complicated as deep scars on skin. Laboratory tests showed white blood cell (WBC) 7,280/µL (neutrophil 39%, lymphocyte 56%), total eosinophil count 156/µL, IgE by PRIST above 1,000 kIU/L. IgG, A, M levels were normal. Specific IgE for Aedes communis by radioallergosorbent test (RAST) was negative. To perform a skin patch test, we prepared salivary gland extracts from 2 mosquito species, including Aedes togoi (Ae. togoi) and Culex Pipiens (Cx. pipiens). These species were chosen because Ae. Togoi is prevalent throughout eastern Asia and Cx. pipiens is found worldwide (), especially in Chungnam area of Korea. The protein concentration of the salivary gland extracts were as following; Ae. togoi, 0.079 mg/dL: Cx. pipiens, 0.523 mg/dL. The result of the skin patch tests showed a positive response to Cx. pipiens after 72 hr (). Lymphocyte subset analysis demonstrated increased NK cells (CD16+CD56, 43%) and decreased CD3 and CD4 cells. IgM for anti-nuclear antigen (EBNA), IgM for viral capsid antigen (VCA) and IgM for anti-early antigen (EA) DR to EBV were all negative. However, the levels of anti-VCA IgG (>200 U/mL), anti-EA DR IgG (>150 U/mL) and anti-EBNA IgG (62 U/mL) were increased: these findings are consistent with chronic EBV infection. Type A EB virus was demonstrated in blood mononuclear cells by DNA polymerase chain reaction (PCR) method (). EBER (EBV encoded RNA) in situ hybridization was negative in necrotic tissues. Immunostaining with NK-cell marker (CD56) revealed many immunoreactive cells with the perivascular inflammatory infiltrates in necrotic tissue (). The patient had a bone marrow biopsy performed, and was diagnosed with the hemophagocytic syndrome 2 yr and 6 months after the first symptom appeared. During the immunosuppressive therapy, the patient had four additional episodes of mosquito bites, each of which gave rise to intense local skin reactions as well as a high fever.