A 29-year-old woman was referred to our institution for a refractive consultation. Uncorrected distance visual acuity was counting fingers in both eyes. Corrected distance visual acuity was 20/20 in both eyes, with a manifest refraction of −7.00 to 1.25×10 in the right eye and −7.00 to 0.50×180 in the left eye. Keratometric readings (Galilei™ dual scheimpflug analyzer; Ziemer Ophthalmology, Biel, Switzerland) were 41.58/40.77 diopters and 42.30/40.48 diopters in the right and left eyes, respectively. Central corneal thickness was 563 μm and 568 μm for the right and left eyes, respectively. Ophthalmic examination showed clear corneas with mild vascularization in the corneal periphery, especially in the superior part of the cornea; no other anterior or posterior segment abnormality was detected. LASIK using the Wavelight® FS200 femtosecond laser and the Allegretto® Wave Eye-Q 400 Hz excimer laser (Wavelight GmbH, Erlangen, Germany) was performed; flap tunnels were created with the same settings in both eyes and orientated superiorly extending from the flap hinge to the limbus. The surgical procedure was conducted uneventfully in both eyes as described in our previous report.
At the end of the procedure, a drop of ofloxacin 0.3% (Exocin®; Allergan, Irvine, CA, USA) was instilled. The patient was examined 60 minutes postoperatively on a slit-lamp (Haag Streit 900; Haag Streit AG, Bern, Switzerland) for flap alignment and adherence. Postoperative treatment consisted of nepafenac 0.1% drops (Nevanac®; Alcon Laboratories) four times daily, dexamethasone 1% (Maxidex®; Alcon Laboratories) four times daily, ofloxacin 0.3% drops four times daily, and sodium hyaluronate drops hourly. The patient was advised to wear a plastic shield during sleep.
On the first postoperative day, uncorrected distance visual acuity was 20/25 in both eyes. However, slit-lamp examination revealed a dense, white, granular reaction combined with some blood droplets (stage I DLK) in the flap “chimney” tunnel of the left eye (); the flap was well positioned and the interface was clear. The remainder of the slit-lamp examination was within normal limits for both eyes. An intensive course (every second hour) of Maxidex together with ofloxacin 0.3% was prescribed for both eyes.
On the second postoperative day, uncorrected distance visual acuity was 20/20 in the right and 20/25 in the left eye; there was an improvement in DLK of the left eye. During the following days, DLK responded rapidly to the treatment and by the fifth day it was completely resolved (); uncorrected distance visual acuity was 20/20 for both eyes. Corticosteroids were tapered over the next 5 weeks without evidence of DLK recurrence.


A 48-year-old black male with chronic alcoholism presented with a soft, bulky mass
lesion in the posterior region of the neck. According to the patient, the mass had
grown slowly and progressively over the course of 5 years, with later development of
similar nodules in the upper trunk.
The patient sought care at the dermatology service with a chief complaint of recent
weight loss, muscle weakness, ataxia, confusion, and loss of orientation to place
and time.
Physical examination revealed a bulky tumor in the posterior neck, with no defined
borders on palpation. Other nodular lesions were present bilaterally in the deltoid,
trapezius, and quadratus regions and in the posterior triangle of the
sternocleidomastoid ( and ).
Histopathological examination of a needle aspiration specimen confirmed the diagnosis
of lipoma.
A systemic workup revealed no metabolic changes, only anemia and mild elevation of
liver enzyme levels. Abdominal ultrasound was within normal limits. A CT scan of the
chest revealed an extensive mass isodense to fat in the trapezius and
sternocleidomastoid regions, with no infiltration of adjacent structures (). Surgical treatment was deferred in
view of the patient's clinical condition.


A 54-year-old male patient presented with GI bleeding of unknown origin that had started 2 weeks previously. He had approximately 500 to 1,000 mL/day, fresh red or dark red blood in the stool. He had no nausea and vomiting, with negative findings on upper gastric endoscopy and colonoscopy. He experienced bleeding recurrence 2 hours before saw a doctor, and was emergently admitted to the hospital. He had approximately 500 to 1,000 mL/day of fresh red/dark red blood in the stool. Blood transfusion and somatostatin were administered without considerable improvement. A stool test showed three to five red blood cells per high-magnification field. His hemoglobin level was 93 g/L. Computed tomography (CT) and CT angiography, including CT ateriography and CT venography, failed to yield a definitive diagnosis. He was prepared for capsule endoscopy by fasting for an additional 12 hours. He was asked to swallow the capsule endoscope with a small cup of cool water, and real-time monitoring of the procedure was set up with a laptop computer beside his bed. The capsule endoscope used was of the model OMOM (Chongqing Jinshan Science and Technology Co., Ltd., Chongqing, China) with the following features:size, 13×27.9 mm; weight, approximately 6 g; one side view; view angle, 140±10°; sample rate, 2 frames per second ±10%; and performance time, approximately 7 to 9 hours. At the start of capsule endoscopy, we found significant food retention in the patient's stomach (). The examination quality did not improve even after the patient was instructed to drink some water. To avoid possibly worsening of bleeding, we did not administer any prokinetic drugs. Instead, the patient was asked to lie on the bed on his right side, as is required in normal procedures, followed by raising his hip gradually and lowering his head (). Meanwhile, the real-time monitoring system of the capsule endoscope was adjusted until the pylorus was positioned at the center of the screen. We found that it took 15 minutes of body position adjustment to make the pylorus appear at the center of viewer () and another 5 minutes for the capsule endoscope to enter the duodenum (), then jejunum. After 2 hours, the capsule endoscope entered the cecum () and ulcer lesions were seen at the terminal ileum () with fresh blood in the cecum (), and there was no fresh blood in another part of colon (). The whole process was recorded on Supplementary Video 1 (available online at ).


A 71-year-old female presented with abdominal pain, for which esophagogastroduodenoscopy (EGD) was performed. Subsequently, a smooth, protuberant mass of descending duodenum was found (). By endoscopic ultrasound (EUS), performed at Shengjing Hospital, the lesion was homogeneous and hypoechoic, and measured approximately 36 mm × 35 mm (). No internal vessels or blood flow was detectable. Due to its size and indistinct margins, the site of origin was also difficult to ascertain. EUS-FNA was thus elected.
A linear echo endoscope (EG3630, Pentax, Tokyo, Japan) and a 22-gauge needle (Echo Tip Ultra, Cook Endoscopy, Winston-Salem, North Carolina, USA) were used to access the mass. After lesion the needle was inserted (under EUS guidance) into the targeted tissue (), the stylet was completely withdrawn by carefully retracting the plastic hub housed in the needle handle. A syringe (prepped in advance) was attached by way of Luer lock to the fitting on the needle handle, and its stopcock was turned to the “open” position (aligned with the syringe),10 cc of negative pressure was allowed in the syringe for aspiration of cells. Guided by EUS, the needle handle was moved back and forth gently in small increments. The tissue had been penetrated several times, when a hyperechoic line was noted in the needle track (). Thereafter, a 10 mm × 7 mm cystic area materialized within the mass, and hyperechoic flow was noted (). In a matter of seconds, the cystic zone grew to roughly 25 mm × 20 mm, and the entire mass expanded to 62 mm × 40 mm (). Because hemorrhage within the mass was likely, the procedure was halted, and the patient was hospitalized.
Two hours afterwards the patient complained of severe abdominal pain, later developing diffuse abdominal rigidity and guarding. Non-coagulated blood was withdrawn by abdominocentesis. By contrast-enhanced computed tomography (CT), the mass of descending duodenum was now measured 81 mm × 62 mm. Its appearance bordered on high-density, with a low-density mass inside (). Findings were suggestive of rupture, accompanied by hematocele, accumulated exudate, and ascites.
Four hours following EUS-FNA, emergency surgery was performed, and the clinical impressions were confirmed. The abdominal cavity contained approximately 1000 ml of blood. Consequently, the tumor was completely resected.
Immunohistochemical staining of the lesion was positive for CD-117, CD-34, and DOG-1 markers, as well as for smooth muscle actin. Positivity of Ki67 was <5%, and staining for Desmin was negative. A diagnosis of high-risk duodenal stromal tumor was ultimately rendered.


A 45-year-old female patient came to the Department of Oral Medicine and Radiology with a chief complaint of gingival enlargement in the anterior region of upper jaw. History revealed that the growth had started 1 month back and rapidly enlarged to the present size. Her past medical history revealed that the patient was under treatment for hypothyroidism with levothyroxine sodium (100 mcg/day) for the past 5 years. No other medical disorders were observed apart from hypothyroidism.
On extraoral examination, the patient's face was asymmetrical due to an ovoid swelling present on the left side of her face. The swelling was approximately measuring 5 × 5 cm in size, extending from the philtrum of the upper lip to the zygomatic arch anteroposteriorly. Superiorly the swelling was seen extending almost to the floor of the orbit. Inferiorly the swelling extended upto the line joining the corner of the mouth to the tragus of the ear [].
On palpation, the swelling was firm in consistency without any tenderness. Patient's left submandibular lymph nodes were also palpable, nontender and hard in consistency measuring 2 × 2 cm in size.
On intraoral examination, a massive, lobulated, firm, nontender and erythematous growth involving the gingiva was seen extending from the left maxillary lateral incisor to the left maxillary third molar []. The growth was extensive and lobulated, covering the teeth on the left side of the maxilla. Patient's peripheral blood picture was completely normal except for anemia (hemoglobin 7.8 g/dl).
Orthopantomograph (OPG) revealed the presence of root stumps in relation to 21, 22 and 25 teeth (Federation Dentaire Internationale World Dental Federation notation) in the lesiona area. However, gross resorption of bone was not evident at the area of lesion []. PNS view (paranasal sinuses radiograph) revealed opacification of the left maxillary sinus, indicating the spread of the lesion to the maxillary sinus. A provisional diagnosis of metastatic tumor of unknown origin was made based on the clinical presentation of rapid growth with enlarged and fixed submandibular lymph nodes.
An incisional biopsy was done and the tissue was sent for routine histopathological evaluation. H and E-stained sections showed hyperplastic surface epithelium with extensive aggregates of small round cells with eosinophilic cytoplasm in the underlying connective tissue [Figures and ]. Tumor cells showed atypical features in the form of cellular and nuclear pleomorphism, hyperchromatism and numerous abnormal mitotic figures. An initial diagnosis of a small round cell tumor was made based on the routine H and E-stained sections.
On IHC, the lesion stained positive with cluster of differentiation (CD) 45 marker indicating the lesion to be of hematopoietic origin ruling out other small round cell tumors from further consideration. Subsequently, negative staining for CD20 and CD3 markers ruled out B-cell and T-cell lymphomas. Finally, the lesion stained positive with CD68 and CD117 markers establishing the myeloid origin of tumor cells. This favors the diagnosis of AML with minimal differentiation. Because CD68 [] and CD117 [] are also positive in MS, we used MPO to differentiate the lesion from myeloid leukemia. MPO [] was strongly positive in the present case confirming the lesion as MS.
On final diagnosis, the patient was immediately referred to the regional oncology center for further investigations related to the assessment of metastasis and treatment. However, the patient died 2 months after the initial diagnosis of MS on chemotherapy.


A 65-year-old female patient reported with the chief complaint of mid facial swelling since 4 months. On extraoral examination, diffuse swelling was seen on left side of face pushing the floor of the orbit with deviation of the nasal septum. On palpation, the lesion was tender and soft in consistency. Intraorally, the swelling was seen obliterating the buccal vestibule in the upper left molar region with an intact overlying mucosa. Computed tomography (CT) scan revealed a destructive hypodense solid mass, occupying the whole of maxillary sinus destroying the lateral wall of nose, floor of orbit and alveolar process.
Scanner view showed tumor epithelial cells in a papillary configuration around a fibrocellular connective tissue core []. Epithelial cells showing two distinct patterns were seen The tumor cells immediately surrounding the connective tissue core showed eosinophilic cytoplasm with vesicular nuclei []. Cellular atypia in the form of nuclear hyperchromatism, pleomorphism with increased and abnormal mitotic figures was frequently seen [] Cells farther away from the core were predominantly clear in appearance [] with minimal atypical features [] Immunohistochemical analysis showed strong cytokeratin (CK) positivity [] with negative vimentin [], confirming the epithelial origin of tumor cells Negative mucicarmine staining ruled out the glandular origin of the lesion.
Clear cell odontogenic tumors are of three types: clear cell odontogenic carcinoma (CCOC), clear cell ameloblastoma and clear cell calcifying epithelial odontogenic tumor (CCEOT).
CCOC shows three architectural patterns: biphasic, monophasic and ameloblastomatous,[] commonest being the biphasic pattern with nests of large clear cells with small islands of hyperchromatic polygonal cells with eosinophilic cytoplasm.[]
Clear cell ameloblastoma shows typical tumor islands similar to follicular ameloblastoma, with peripheral ameloblast-like cells and the clear cells replacing the central stellate reticulum-like cells.[]
CCEOT is characterized by polyhedral epithelial cells alternating with large epithelial cells with a clear foamy cytoplasm and amorphous eosinophilic amyloid in the connective tissue.[]
Malignant clear cell salivary gland tumors are clear cell acinic cell carcinoma (CCACC), clear cell mucoepidermoid carcinoma (CCMEC) and clear cell epithelial myoepithelial carcinoma (CCEMC).
CCACC has typical acinar cells admixed with clear cells that are found in small clusters lining the microcystic spaces. The tumor is positive for amylase, cytokeratin (CK), S-100 protein and vimentin.[]
CCMEC is biphasic with a squamous cell and a mucous cell component and few cells with features of both types termed as intermediate cells. Clear cells may contain Periodic acid-Schiff (PAS)-positive diastase sensitive droplets of glycogen. Special staining for mucicarmine or alcian blue can identify the mucous cell population.[]
CCEMC has a distinct biphasic pathognomonic appearance. The islands of tumor cells are composed of small ducts lined by cuboidal epithelium that are surrounded by clear cells that interface with a thickened hyaline-like basement membrane.[]
Carcinomas from kidney, liver, large bowel, prostrate and thyroid are known to have the potential for clear cell differentiation and tend to metastasize to the maxillofacial area.
Renal cell carcinoma (RCC) is the most common tumor that metastasizes to the jaws, especially the mandible and it is the main diagnostic possibility when considering a distant primary neoplasm with clear cell differentiation.
RCC is characterized by tumor cells that are round or polygonal in shape with abundant clear/granular cytoplasm, which contains glycogen or lipids along with prominent sinusoidal vascular component and hemorrhagic areas.[]
Papillary squamous cell carcinoma of maxillary antrum-clear cell variant.
This tumor is infiltrative and is managed by radical surgical resection followed by radiotherapy. It has a better prognosis than the conventional squamous cell carcinoma of the similar clinical stage, provided the treatment protocol is followed.


A 40-year-old man was admitted for poisoning. His son was incidentally noted to have SHFM. He was born of nonconsanguineous marriage. Similar deformities were present in his mother and brother []. This was present over four generations of the mother's family (seven more members) suggesting AD pattern of inheritance. None of the observed members had any features of ectodermal dyplasia. Despite the deformities, both the brothers were employed, while the mother could knit clothes in her free time.
A 12-year-old girl who had come to visit a patient was found to have SHFM without any skin or teeth anomalies. Similar deformities were present in her sister [], but not in the parents; suggestive of AR pattern.
A 19-year-old boy was on follow-up for diabetes. He had developmental delay with deaf mutism. He had SHFM of the left upper limb []. Skin and teeth were normal. He was born of consanguineous marriage and his father said that the boy's elder sibling died after birth and it had similar shaped hands and feet. The parents were normal suggesting AR inheritance.


A 38-years-old lady was referred for corneal transplantation in left eye for corneal decompensation secondary to ICE syndrome. Best corrected visual acuity (BCVA) in right eye was 20/20, N/6 and in left eye was 20/400, N/60. Slit lamp examination of left eye showed diffuse corneal edema. Peripheral anterior synechiae (PAS) were intermittently present all around 360°. Details of iris, lens, and fundus were hazily seen due to corneal edema. Right eye examination was within normal limits []. Intraocular Pressure and not pressures. (IOP) readings by applanation tonometry were 15 mmHg in right eye and 11 mmHg in left eye. Ultrasound pachymetry readings were 489 μ in right eye and 641 μ in left eye. Ultrasonic central anterior chamber depth (ACD) was 3.68 mm in right eye and 3.75 mm in left eye. Fundus examination including optic disc assessment was within normal limits in right eye. Central endothelial cell density as measured by non-contact specular microscope (Topcon SP 2000P) in right eye was 2316 cells/mm2. Specular image acquisition was not possible in left eye due to marked corneal edema. Patient was diagnosed ICE syndrome with endothelial decompensation in left eye, and simultaneous DSEK and CLE with PCIOL implantation under peribulbar anesthesia was planned for her left eye.
Donor dissection was performed before the patient was called in operation room. 49-years-old, phakic, clinically ‘very good’ donor corneal tissue was used for surgery. Donor tissue was manually dissected into anterior 2/3rd and posterior 1/3rd of stroma using artificial anterior chamber (Madhu Instruments, New Delhi, India) and blunt lamellar dissectors (Ankur Metal Works, Kolkotta, India). Edematous corneal epithelium was removed to improve the visibility. A 5.5 mm superotemporal scleral tunnel was used for the surgery. The internal wound of this tunnel was made ahead of PAS. Anterior chamber (AC) was continuously formed by anterior chamber maintainer, attached to balanced salt solution (BSS). While attaching irrigation in the form of AC maintainer to the eye, it was ensured to avoid touching PAS. After performing circular curvilinear capsulorhexis (CCC) and hydro dissection, nucleus was delivered from sclero-corneal tunnel with the hydrostatic pressure of BSS. A single piece acrylic PCIOL was implanted in the capsular bag after cortical wash. Intracameral pilocarpine was used to keep the intraocular lens in position during graft insertion. In view of simultaneous EK, the planned postoperative refraction was −1.5 diopters. Descemet's membrane (DM) was scored and separated in a circular pattern (descemetorhexis) in peripheral cornea central to PAS with the help of a reverse Sinskey hook and removed from anterior chamber. Trypan blue was not used, and descemetorhexis was done against red glow of clear crystalline lens. Pre-dissected donor tissue was trephinated using 8.0 mm disposable corneal trephine (Madhu Instruments, New Delhi, India). The diameter of the trephine was selected after measuring the PAS free central cornea with calipers and subtracting 1 mm from it. The idea was to avoid graft-PAS touch. A sheet's glide was placed in the scleral wound such that the tip of the glide covers the pupil. The part of the glide outside the wound was covered with cohesive viscoelastic substance (Healon, Abbott Medical optics). Posterior stromal donor tissue with healthy endothelium was also covered with same viscoelastic substance on endothelial side and separated from anterior layer. The graft was placed on the sheet's glide with endothelium facing down and slipped into AC with the help of Sinskey hook. The graft was secured in position by filling anterior chamber (AC) with air and centered by stroking on the surface of host cornea. It was ensured that the edge of the graft doesn’t touch PAS anywhere. The air was replaced with balanced salt solution 1 hour after the surgery. (Video; Supplementary File).
Postoperatively, the patient was prescribed topical gatifloxacin 0.3% 4 times per day for 2 weeks and topical prednisolone acetate 1% 6 times per day, which was tapered in 3 months. After 3 months, patient was advised topical fluorometholone 0.1% once per day indefinitely. There was no IOP spike postoperatively at any time till last follow-up (1 year).
Corneal edema cleared completely in 5 weeks with a best corrected visual acuity (BCVA) of 20/30 (−2.5DSph/ −3.0D cyl @ 120 degrees). This improved further to 20/20 at last follow-up visit of patient (1 year post-op). There was no interface haze. Central endothelial cell count at 6 months was 1315 cells/mm2 with coefficient of variation in cell size of 47. It was stable (1290 cells/mm2) till last follow-up (1 year). Postoperative central ultrasonic pachymetry was 590 microns in left eye. Iris atrophic patches were visible through clear cornea []. Gonioscopic examination of left eye showed patches of peripheral anterior synechiae in all quadrants, which were documented with anterior segment OCT []. IOP (18 mmHg), optic disc assessment (0.5: 1 Cup: Disc ratio with healthy neuroretinal rim) and visual field evaluation (Humphrey's field analyzer) were within normal limits. Since there was no evidence of glaucoma in either eye, no antiglaucoma medications were prescribed.


The patient is a 63-year old female who visited our emergency department with altered mental status. Two weeks ago, she admitted local medical center for left knee pain and underwent arthroscopic surgery of cartilage resection. After discharge, she continuously suffered from nausea and vomited more than 20 times a day, leading to dehydration and weight loss. Four days after vomiting, she complained of lethargy and somnolence. She had no history of hypertension, hematologic disorders, or other systemic disease such as diabetes mellitus.
On admission, her vital signs were stable. Blood pressure was 130/84 mmHg and body temperature was 36.8°C. On neurological examination, mental status was drowsy. She did not show sign of meningeal irritation. Brainstem sign was intact and deep tendon reflexes were normoactive bilaterally. In our emergency room, she developed one episode of generalized tonic clonic seizure. She was injected with lorazepam 4 mg and recurrent seizure did not develop. She showed continuously drowsy mentality and complained of severe headache.
In laboratory test, she showed hyponatremia, which is 124 mmol/L. Cerebrospinal fluid study revealed normal, which was performed to exclude central nervous system infection. Electroencephalography showed 5–6 Hz diffuse background slowing and there was no epileptiform discharges. Brain magnetic resonance imaging (MRI) showed bilaterally diffuse abnormal signal intensities in the parieto-occipital lobe and superior frontal sulcus, which involved predominantly the deep white matter (). All these changes showed hyperintense on T2-weighted and FLAIR images. There was no enhancement in meninges and brain parenchyme. Her clinical symptoms and brain MRI findings suggested the possibility of PRES.
During admission, her blood pressure was normal. She was treated with saline infusion for correction of hyponatremia. After treatment, her consciousness gradually improved. After three days, her sodium level was normal and she did not complain of lethargy or somnolence. She discharged from hospital without neurological deficit. After one month, follow-up brain MRI revealed that abnormal signals involving both parieto-occipital cortex and superior frontal sulcus were much improved ().


A 75-year-old man was diagnosed with gastric cancer in June 2012 at Showa University Fujigaoka Hospital. Fifty years previously, he had undergone a gastric bypass operation with a loop gastrojejunostomy to treat stenosis caused by a duodenal ulcer.
His medical history included chronic renal failure (managed with hemodialysis), right idiopathic pleural effusion, third degree AV block (managed by a pacemaker), and myelodysplastic syndrome. As of 2006, chronic gastric hemorrhage had been observed at the GE anastomotic site, and endoscopy was performed. In June 2012, an upper gastrointestinal endoscopy revealed a slightly irregular mucosa surrounded by a smooth elevated area at the stomal site. The irregular mucosa was biopsied, and a papillary and tubular adenocarcinoma were diagnosed. Endoscopic ultrasonography revealed that the tumor extended to the muscularis propria. CT showed an enlarged lymph node on the lesser curve of the stomach, which is suggestive of metastasis. However, the gastric wall did not appear thickened, which can occur secondary to neoplasia, and there was no indication of distant metastasis. The cancer was diagnosed as T2 N1 M0 stage IIB according to the TNM classification. Since the patient already had poor systemic function, surgery carried a very high risk of morbidity and mortality. Similarly, cancer chemotherapy could not be initiated. The surgeon repeatedly discussed the treatment options with the patient who ultimately elected surgical excision to avoid potential obstruction and hemorrhage at the stomal site. In October 2012, a distal gastrectomy, a small intestinal partial resection (afferent and efferent loops) and a Roux-Y reconstruction were performed.
During the laparotomy, hepatic sclerosis and ascites were observed. The significant collateral vasculature and the strong adhesion between the stomach and the liver and between the jejunum and the transverse colon from the previous GE made surgery difficult. Numerous firm and small lymph nodes were identified in the lesser omentum and suspected to be metastatic. A small intestinal partial resection, including the BEE site, was performed followed by a distal gastrectomy. Metastasis was observed in the common hepatic arterial lymph nodes similar to the metastasis observed in the para-aortic nodes, and a curative resection was deemed impossible. The surgery lasted 6 h, and there was a 1,492-ml intraoperative hemorrhage. Immediately after transferring the patient to the intensive care unit, he suddenly stopped breathing, and resuscitation was initiated. It progressed into multiple organ failure and the patient died in December 2012.
The surgical specimen is illustrated in figure . A 5 × 4 cm type-5 lesion was identified in the posterior wall at the stomal site on gross pathologic examination. HE staining (fig. ) showed that the tumor histologically comprised two elements: a tubular adenocarcinoma on the gastric side and NEC-like cells in a typical mass lesion comprising infiltrating nests of small uniform tumor cells on the jejunal side, which made up 40% of the tumor. The gastric mucosa adjacent to the tumor did not show evidence of gastritis. Immunohistochemistry (fig. ) showed that the NEC-like cells were positive for neural cell adhesion molecules and partially positive for synaptophysin and somatostain receptor type 2, but negative for chromogranin A. The Ki-67 labeling index was >20%. The final pathological diagnosis was MANEC, according to the 2010 WHO classification system for digestive neuroendocrine tumors.


The first clinically confirmed family member was patient No. 1's mother. In 1972, the Caucasian female, who was 24 years old at the time, underwent her first procedure, which was Miles procedure for rectal cancer located at 13 cm from the anal verge. On pathology specimen, several polyps with high-grade dysplasia were found in the rectal ampulla. At the age of 33, a correction of sigmoidostomy was performed due to a prolapse of the stoma, and multiple polyps were found in the specimen. Clinical diagnosis of FAP was established. At the age of 42, total colectomy with Kock's reservoir was performed (around 50 polyps with low- to high-grade dysplasia were recorded on pathology report). Further follow-up was carried out according to FAP protocols, and at the age of 55, polyps were detected in the gastric antrum and duodenum and a biopsy revealed adenoma. At the age of 57, 4 large periampullar polyps were removed, one of which comprised adenocarcinoma in situ. At the age of 63 and 65, another 2 polyps, gastric and duodenal, were removed. At the age of 45, adenoma of the parathyroid had been removed.


A 50-year-old man presented with an 8-year history of severe itching, hypohidrosis, total loss of body hair, nail changes, acneiform lesions on the trunk, and scaly plaques on the scalp and palmoplantar regions ().
The patient's condition had been misdiagnosed as acne conglobate, and he had been treated with oral isotretinoin as well as oral and topical antibiotics for several years without any response. Soon after the loss of scalp hair and body hair, skin biopsy specimens were taken, and MF associated with follicular mucinosis was diagnosed (). Immunohistochemical examination showed that these atypical cells were CD4+ T-cells. He had been treated with oral methotrexate and cyclophosphamide-vincristine-adriamycin-prednisolone chemotherapy without any improvement of the skin lesions.
On dermatologic evaluation, the most relevant features were the pterygium formation observed in all the nails; palmoplantar involvement with atrophic erythematous scaly patches; purplish-brown linear macules on the axillary region; lichenoid, infiltrated, and purple papules on the trunk; and total loss of body hair. These findings prompted us to propose the diagnosis of lichen planopilaris. Comedo-like lesions on the entire scalp, face, and trunk; cystic and acneiform inflammatory lesions on the trunk; and scaly atrophic erythematous patches on the scalp were also observed. There was no lymphadenopathy or hepatosplenomegaly.
Biopsy specimens taken from the comedo-like lesions on the trunk, erythematous patches on the palmar and plantar regions, and purple macules on the axillary region revealed pilotropic cutaneous T-cell lymphoma without follicular mucinosis. Atypical cells comprising CD4+ T-lymphocytes could be observed within the follicle epithelium (). Sweat glands and ducts were also infiltrated by these atypical lymphocytes displaying the CD4+ phenotype (). Immunohistological examination and T-cell receptor β-chain gene rearrangement analysis of the skin biopsy specimens showed a clonal population of T-cells. Skin cultures taken from the pustules revealed the presence of Staphylococcus aureus. Routine laboratory investigations for systemic involvement, such as complete blood count, peripheral blood smear examination, and blood biochemistry, were unremarkable. Results of whole-body computed tomography were normal. The patient had no systemic involvement.
The patient was administered with 25 mg/d of acitretin with psoralen and ultraviolet A (PUVA) therapy thrice every week for 4 months along with a short-term oral antibiotic therapy (1,000 mg amoxicillin clavulanate, twice a day, 10 days) for staphylococcal superinfection. Significant improvement of skin lesions and relief of itching were observed at the end of 4 months. No atypical lymphocytes were observed on control biopsy specimens. Although PUVA therapy had been planned to be continued for residual clinical disease, the patient refused any further treatment. He has been followed-up for 7 years, exhibiting stable skin disease and no systemic involvement. No comorbid hematologic malignancy was observed.


A 57-year-old man presented with acneiform lesions that he had since childhood and loss of body hair and scalp hair. Dermatological examination revealed loss of body hair, eyelashes, and eyebrows, and some uninvolved scalp hairs. Generalized comedo-like lesions were observed prominently on the head and trunk. Cystic lesions were more prominent on the ears and scalp, and hidradenitis suppurativa-like lesions were observed on the axillary and inguinal regions (). Biopsy specimens taken from cystic lesions on the left arm revealed folliculotropic MF with follicular mucinosis (). Immunohistochemical analysis showed that the intraepithelial and perifollicular infiltrate mainly comprised CD3+ and CD4+ lymphocytes, with few CD8+ cells.
No clinically palpable lymphadenopathy or systemic involvement was detected. Routine laboratory investigations for systemic involvement were unremarkable. This patient also received 25 mg/d of acitretin with PUVA therapy thrice every week for 4 months. No atypical lymphocytes were observed on control biopsy specimens. No comorbid hematologic malignancy was present. Modest clinical improvement and relief of itching was observed. The patient has been followed-up for 6 years, and had not experienced any systemic involvement. No improvement in body hair was observed, but the patient has been relieved of itching.


A 13-year-old boy was brought to the emergency room with multiple injuries following a motor vehicle accident. He was intubated because of low Glasgow Coma Scale (GCS; 5/15). Pupils were equal and reactive, blood pressure 100/55 mmHg, pulse 110 per min, oxygen saturation 94% on 0.6 FiO2, and respiratory rate 16 per min. Trauma X-ray series showed fractures of the left femur and pelvis (stable fracture). Computed tomography (CT) brain showed mild cerebral edema with no midline shift. Patient was transferred to the intensive care unit (ICU) for further management.
Patient was sedated with midazolam 4-6 mg/h and fentanyl 100-150 μ/h. The ventilator (Dräger, Infinity C500, Germany) settings in the ICU were as follows; assist control mode, respiratory rate 14 per min, tidal volume 7 ml/kg (according to ideal body weight), inspiratory flow rate 45 l/min, positive end-expiratory pressure (PEEP) 5 cmH2O, and FiO2 0.5. Patient's weight and height were 60 kg and 160 cm, respectively (ideal body weight (IBW) 57 kg). Lung mechanics revealed high peak and plateau pressure (35 and 30 cmH2O, respectively).
After 24 h, patient's pupil became unequal and CT brain showed right temporoparietal epidural hematoma. Evacuation of hematoma was done, and patient was transferred back to ICU with ICP monitoring device. Initial ICP and cerebral perfusion pressure (CPP) were 17 and 60 mmHg, respectively []. Post-surgery ventilator settings were assist control mode, rate 28, tidal volume 6 ml/kg, FiO2 1.0, and PEEP 8 cmH2O. Arterial blood gas showed pH 7.32, PaCO2 46, PaO2 65, HCO3 19 meq/L, and SpO2 was 93%.
After 48 h, patient developed features of severe ARDS[] []. Tracheal aspirate was negative for any organism. Lung protective ventilation strategy was started as follows; ventilatory rate 32 per min, tidal volume 5-6 ml/kg (300-350 ml), FiO2 1.0, and PEEP 16. This ventilatory setting led to increase in ICP from 17 to 28 and reduction of CPP from 60 to 45. Arterial blood gas showed pH 7.24, PaCO2 53, and PaO2 58. Cisatracurium was used at this stage to paralyze the patient with 0.3 mg/kg/h as maintenance dose after bolus of 0.2 mg/kg. Train of four was also monitored. 20% mannitol (1 g/kg) was used 8-hourly for 24 h with monitoring of serum sodium (target serum sodium: 150-155 mEq/L) and serum osmolality (target osmolality up to 320 mOsm). Norepinephrine was used for 24 h for keeping mean arterial pressure (MAP) above 90 mmHg.
Patient was started on INO to improve pulmonary mismatching. INO was started after discussion with family members. It was started with 10 ppm, then gradually built up to 35 ppm. Next 12-24 h, PaO2 and PaCO2 improved. ICP also showed gradual improvement from 18 to 5. Blood gases were as follows: pH 7.37, PaCO2 40, and PaO2 128. PEEP reduced from 16 to 10 and FiO2 from 1.0 to 0.6 [ and ].
INO was discontinued after 1 week, and patient was weaned off from the ventilator. Post-extubation patient remained conscious with no neurological deficit. Repeat CT head was normal. Further stay in ICU remained uneventful, and then patient was transferred to the ward. Patient remained in hospital for 2 months as he developed an hospital-acquired infection and then shifted home.


An 80-year-old female patient reported to our department with a chief complaint of pain and swelling in the lower right back tooth region since past 15 days. Clinical examination revealed a huge swelling on the left side of the jaw []. The patient was aware of the swelling since 15 years. The swelling was gradual on onset, slowly increased over years to present size and was not preceeded by any trauma or tooth ache in that region of jaw. History of associated pain since 3-4 years was noted. No history of any loss of sensation or pus discharge was noted. The patient was a known hypertensive since 15 years and was on regular medication. The swelling was solitary, well-defined, firm in consistency, tender, and 12 × 12 cm in size extending from the right canine region to the left angle region. No evidence of lingual cortical plate expansion or perforation was noted. Based on the history and clinical findings, a differential diagnosis of osteoblastoma, cementoblastoma, calcifying epithelial odontogenic tumor, and OF was given.
Radiographic evaluation with OPG and lateral view of mandible showed a mixed radiolucent and radioopaque lesion with diffuse foci of calcifications throughout [Figures and ]. Patient underwent extraction of the complaint tooth and an incisional biopsy of the lesion was done and sent for histological evaluation.
Histopathological analysis revealed connective tissue stroma that was highly cellular and was made up of numerous fibroblasts and collagen fibers. Trabeculae of bone with osteocytes and globules of calcification amidst plump fibroblasts were noted [Figures and ]. Few endothelial lined blood vessels were also seen giving an impression of OF.
Correlating the clinical, radiological, and histopathological findings, a final diagnosis of OF was given. Treatment done was resection and reconstruction of mandible with reconstruction plate under general anesthesia [Figures and ]. Postoperative period was uneventful and no recurrence noted with follow-ups [Figures and ].


An 8-year-old Hispanic girl was referred to our institution for a growth in her right conjunctival fornix. Her mother had noticed the mass over the past few months and felt that it was enlarging. Our patient did not have any other medical conditions and denied undergoing prior trauma or surgery. Her only ocular history involved multiple, recurrent episodes of redness in the right eye that were diagnosed and treated as either bacterial or viral conjunctivitis by the referring physician. The mother also reported that she had discovered pieces of hair in her daughter’s right eye tear film over the past couple of years.
On presentation, the patient’s eye looked relatively normal, and she had uncorrected visual acuity of 20/20 in both eyes, with an otherwise unremarkable gross examination. Slit lamp examination of her right eye revealed a mildly injected palpebral conjunctiva with a moderate-sized, fibrous, tan-colored mass that had arborizing, centrally coursing blood vessels. Upon manipulation of the mass, a large tuft of normal appearing eyelashes emanated from the inferior conjunctival fornix ().
The mass was easily mobile, firm and non-fluctuant, so a decision was made to surgically remove the mass. The patient was taken to the operating room, and the base of the mass was carefully resected with sharp Westcott scissors. The tumor had a wide based pedicle and was easily removed. The specimen was placed in formalin and sent to pathology for analysis. The remaining conjunctival edges were easily reapproximated without tension, so no sutures were placed. On post-operative day one, the patient was comfortable and the conjunctival fornix was completely healed. She returned for her post-operative month one visit and had a pristine inferior conjunctival fornix, without any evidence of tumor recurrence.
The pathology specimen revealed nonkeratinized stratified squamous epithelium with multiple goblet cells and an underlying chronic mononuclear inflammatory cell reaction. The bulk of the lesion was made of relatively loose, irregular connective tissue with multiple small hair shafts in longitudinal and transverse cross-section and a small amount of adipose tissue. The pathology report was consistent with dermoid choristoma of the conjunctiva ().


Female patient, 52 years old, admitted at another service due to episodes of
recurrent syncope in 2010. At the time, she had suffered the recent loss of a
son in a car accident and was drinking a type of "tea for weight loss." The
electrocardiographic monitoring showed episodes of rapid ventricular tachycardia
( and ). Within hours, the patient developed electrical storm
and needed defibrillation for more than 50 times during a 24-hour period,
requiring sedation and orotracheal intubation. An attempt was made to suppress
the arrhythmias with isoproterenol, which was not successful.
Recurrence was observed in less than 6 hours after the start of drug infusion. A
temporary pacemaker was implanted in the right ventricle, performed on
stimulation without arrhythmia suppression. She was clinically stable after 48
hours of deep sedation and administration of verapamil at a dose of 240
mg/day.
The investigation showed no structural heart disease at the echocardiography, MRI
and coronary angiography. The 24-h Holter monitoring, performed after crisis
stabilization, showed 960 VE/24 h, with several of them showing short coupling
(+/- 280 ms) and the same morphology. There were no tachycardias. At the ECG,
the QTc was 400 ms. Electrophysiological mapping and attempted VE ablation were
not successful. The scarcity of extrasystoles during the procedure did not
induce arrhythmias when programmed ventricular stimulation was performed in the
apex and right ventricular outflow tract, as well as using 'short-long-short'
cycles for of VT/ VF induction under sensitization with isoproterenol. ICD
implantation was indicated and treatment with verapamil was maintained. The
patient remains asymptomatic during the two-year follow-up.


This is an asymptomatic, 18-year-old woman, the daughter of the patient described
in case # 2. During family screening, she was diagnosed with VE with a coupling
of approximately 300 ms (17 extrasystoles in 24 h) during a 24-h Holter
monitoring. Because the patient was asymptomatic and due to the low-density VE,
it was decided to prescribe verapamil 160 mg/day and keep her on frequent
clinical monitoring. She remains asymptomatic after a follow-up period of 48
months.


Female patient, 39 years old, with episodes of syncope preceded by sudden and
fast malaise, without trauma and unrelated to exertion, some occurring at night,
during sleep, at an approximate frequency of three times a week since March
2011. She sought medical attention a few times, having been diagnosed with
anxiety and prescribed anxiolytics, with no improvement of symptoms and
worsening of anxiety. On 07/10/2011, she had recurrence of syncope, with
characteristics similar to those described, when she was admitted at another
hospital for diagnosis.
The patient had no family history of cardiac arrhythmias or sudden death.
Physical examination was normal. The initial ECG showed no abnormalities. The
exercise stress test was negative for ischemia and di not trigger arrhythmias.
The echocardiogram showed a structurally normal heart (LV = 52/35 mm, LVEF =
60%; Aorta = 33 mm; LA = 35 mm; septum and posterior wall = 9 mm; preserved
biventricular function, no segmental alterations in contractility and mitral
valve prolapse with discrete meso-systolic leak). Cardiac magnetic resonance
imaging also ruled out structural heart disease showing preserved cardiac
chamber dimensions with normal biventricular function (LVEF = 70% and RVEF =
69%), without the presence of myocardial fibrosis.
The Holter monitoring showed several polymorphic VE with short-coupling interval,
some of them inducing nonsustained polymorphic ventricular tachycardia initiated
by extrasystoles with R-on-T phenomenon, with coupling between 350 and 370 ms,
and QT intervals within normal limits.
The coronary angiography ruled out the presence of obstructive coronary artery
disease. She was prescribed diltiazem 180 mg/day and a defibrillator implant was
indicated, performed in August 2011. As the VE persisted (density 2%) with
episodes of non-sustained polymorphic ventricular tachycardia, diltiazem was
substituted by verapamil 240 mg/day in September 2011. New 24-h Holter
monitoring showed a significant reduction in the density of arrhythmias
(<1%), increase in minimum coupling interval of extrasystoles to 370 ms and
absence of episodes of non-sustained polymorphic ventricular tachycardia, always
with a normal QT interval. The patient became asymptomatic, with no further
episodes of syncope or ICD therapies, during a nine-month follow up.


The present case report is about a 50-year-old female patient who had been diagnosed as having lung adenocarcinoma with multiple bone metastases was initiated on gefitinib therapy at an oral dose of 250 mg/d. She had a positive epidermal growth factor receptor (EGFR) mutation status. After 2 weeks of initiating therapy, the patient presented with ulcer over the palm []. The ulcers improved with stopping gefitinib for 2 weeks and also with the addition of topical steroids and antibiotics.


A 33-year-old male presented with a history of persisting right flank pain which started a 2-month before and before that intermittedly occurred in the last year. A 12 cm mass is detected on ultrasonography in the middle of the kidney at corticomedullary junction. Clinically, xanthogranulomatous nephritis is suspected as the patient meanwhile represented with signs of inflammation like fever, pain and high laboratory levels of inflammatory parameters in serum. Low density mass is described in computed tomography (CT) images. CT-guided biopsy and frozen section were made, which revealed pyelonephritis with spindle cell tumor of uncertain malignancy. The tumor part was too little, to be histologically differentiated. Patient was scheduled for open exploration of the right kidney (). Pre- and post-operative serum creatine was normal (84 umol/L). Intraoperatively, the kidney demonstrated with macroscopic necrosis that shined through the renal capsule. At two locations frozen section were taken, demonstrating inflammation and necrotic tissue. The necrosis was evacuated in order to drain the kidney, but finally and corresponding to the abdominal CT scan, the central part of the kidney was necrotic and renal pelvis was open, the preoperatively implanted DJ-ureteral catheter laid in the necrotic central part of the kidney. Consecutively, right nephrectomy was applied, which showed a 12×7×5 cm tumor () with vessel invasion. The tumor showed grayish white tissue with multiple blood spaces, necrosis and cysts. Pathologic confirmation was performed by immunohisto-chemical methods and in-situ-hybridization (FISH). Histologic examination of tumoral tissue, stained with hematoxylin and eosin, revealed solid cellular nests of monomorphic spindle cells with non-uniformly bounded cytoplasm in large areas and fascicles with cystic structures settled among them (). For the immunohistochemistry deparaffinized and rehydrated FFPE tissue sections (1-2 m) were stained after autoclave pre-treatment. Sections were processed in an automated staining system (Benchmark ULTRA, Ventana Medical Systems, Inc., Tucson, AZ, USA). Mouse monoclonal antibodies were used. FISH analysis was performed on FFPE tissue. Tissue was cut at 5 mm. First and last sections of each analysis were stained with hematoxylin and eosin (H&E) to delineate regions representing tumor and normal tissue. Tissue preparation for dual-probe hybridization was facilitated by ZytoLight FISH-tissue implementation kit according to manufacturers’ instructions (ZytoVision, Bremerhaven, Germany). Nuclei were counterstained with 4,6-diamidino-2 phenylindole (DAPI).
Immunohistochemistry was performed with an aim to detect the nature of the tumor cells and to differentiate the tumor and excluding the differential diagnosis which in these cases include sarcomatous renal cell carcinoma, nephroblastoma of the adult type, spindle /mesenchymal tumor of the kidney, dedifferentiated liposarcoma, rhabdomyosarcoma, leiomyosarcoma and neuroendocrine tumors. The tumor cells were positive for vimentin, CD99, BCL-2 and focally positive for AE1/AE3 and EMA. There was no reaction to CEA, desmin, chromogranin, synaptophysin, enolase, S-100, CD-10, CD-117, Caldesmon, bcl-2, β-catenin, PAX2, and WT-1. The cysts were lined by epithelium, which was focally positive for AE1/AE3 and for PAX2. In the molecular study of the study, there is sure rearrangement in the SYT-SSX gene (). According to these findings a diagnosis of monophasic spindle cell synovial sarcoma was done. CT imaging in April 2014 demonstrated no signs of metastatic disease. Patient is scheduled for adjuvant chemotherapy with 4 cycles of adriamycin and ifosfamid.


The present case report is about a 12-year-old female patient who presented medial foot and ankle pain related to recreational activities. A rigid mass on the medial side of the foot was perceived, followed by progressive functional incapacity. Due to the foot and ankle medial protuberance, local trauma was frequent; producing skin blisters []. Physical examination showed rigid hindfoot valgism, blisters and medial hindfoot prominences, causing pain when touched. shows foot and ankle X-rays.
The images show a talar head mass which deforms the normal anatomy. The mass exceeds the talar head limit, occupying the sinus tarsi space []. The joint irregularity clearly explains inversion-eversion blocking and pain during physical activities.
Considering the symptoms and functional limitations, the patient underwent surgical procedure, receiving medial and lateral approaches.
It was impossible to remove and model the anomalous tissue due to gross deformity and articular degeneration. Therefore, talo-navicular arthrodesis was performed after ample resection of the abnormal tissue [].
Histopathological analysis showed a tissue compatible with articular osteochondroma. Mitotic figures and cellular changes associated with malignancy transformation were inexistent. The main feature differentiating HED from classic osteochondroma is HED epiphysis involvement. Classic osteochondroma characteristically appears in the metaphysis [].
After surgery, weight bearing was prohibited during 8 weeks, followed by physiotherapy during 3 months.
At 2 years post-surgery, the patient presented no pain, no disease recidivism and complete arthrodesis healing [Figures and ].
The patient presented good aesthetic and functional outcome despite few limitations, especially regarding sport activities.
At 12 years post-surgery, when the patient was 24 years old, the last clinical and radiological evaluations showed no functional daily activity limitations and no signs of disease recidivism [Figures and ].
Although considered a successful treatment, the patient was still advised to maintain regular monitoring due to potential complications such as foot arthritis.


This was a case of a 48-year-old female patient who presented to the out-patient department (OPD) with the complaints of severe headache, dizziness and palpitation. She was a known case of essential hypertension and on drug treatment for hypertension. She was taking amlodipine (2.5 mg once daily) and metformin (250 mg twice daily) for 6.5 years. She has some difficulty with taste identification and stopped her drug amlodipine 25 days ago before presenting to OPD. On questioning the reason made for stopping the drug amlodipine alone and not metformin was logical for her, adding that her husband was diabetic and on metformin therapy but without any change in taste alteration. Hence, she herself stopped the other drug (amlodipine) she was taking. She also added that there was some improvement in loss of taste sensation. However, the actual complaint which brought her to the OPD was symptoms of hypertension which was due to the stoppage of the antihypertensive drug. On examination, she was found to be apparently normal. Her oral cavity was also examined for any pathology and was found normal. Clinical examination pertaining to central nervous system (including cranial nerve examination) and peripheral nervous system was also within normal. Vitals - Pulse rate - 98/min; blood pressure - 160/100 mm Hg; respiratory rate - 16/min and no abnormal deviation in auscultation findings. Since her blood pressure was high, she was again prescribed the same medication (amlodipine) with dosage increased to 5 mg once daily. At 8 days later, she presented with the similar complaint but with controlled blood pressure proving that amlodipine can cause dysguesia. Electrocardiography was within the normal limits except for changes pertaining to chronic blood pressure. The investigation also excludes any mineral/vitamin (zinc) deficiency []. Hence, she was asked to stop the drug and was changed on other medication which does not cause dysguesia namely cardio-selective β-blocker bisoprolol (2.5 mg). She was followed for next 15 days and 30 days for blood pressure and her status of taste sensation. It was found blood pressure was 110/70 mm Hg taken as average and she feels comfortable with moderate improvement in taste sensation.


A 62-year-old male with no comorbidities or previous ocular pathology examined in November 2012 complained of progressive blurred vision in his right eye over the past 5 years. The patient was known only for mild myopia and denied any history of previous ocular trauma.
Visual acuity was 20/60 in the right eye and 20/30 in the left eye, with a refractive error of −0.50 spherical in both eyes. Anterior segment examination of the right eye revealed nuclear and posterior lens opacities and incipient lens opacity of the left eye. No signs of previous trauma like angle recession, inferior angle pigmentation or phacodonesis were evident.
At fundoscopy examination, an oval, elevated retinal lesion in the superior nasal quadrant was noted, involving four clock hours, with no macular involvement (fig. ). No retinal breaks were identified, with a demarcation line bordering the lesion and with the retina slightly thinned. Left fundoscopy examination was completely normal.
A B-mode superior transverse nasal echography was performed with a 10-MHz probe. An oval, anechoic image was observed, compatible with a retinal macrocyst with smooth and well-defined borders (fig. ). Adjacent to the retinal macrocyst, there was a serous retinal detachment. A standardized A-mode echography showed a reflectivity spike higher than 98% (fig. ), thus confirming its retinal origin.
Combined scleral buckle and vitrectomy was the treatment chosen for the retinal detachment. Cataract surgery at the same surgical time was suggested but rejected by the patient. He was followed clinically and by ultrasound, first at 4 months after his initial evaluation (fig. ). His last examination was 14 months after presentation and revealed stable visual acuity with no clinical or echographic changes (fig. ).


We report a case of 28-year-old female weighing 40 kg, a known case of HS posted for laparoscopic splenectomy and cholecystectomy. She presented with pain abdomen and fever. She gave a history of jaundice since childhood. On physical examination, she had pallor, icteric sclera and splenomegaly. Rest of the systemic examination was normal. Her haemogram revealed haemoglobin (Hb) level of 6.2 g% and reticulocyte count of 18.5%. Peripheral blood smear showed spherocytes with increased osmotic fragility and Coombs test negative. Unconjugated bilirubin was 5.4 mg/dL, prothrombin time (PT), international normalised ratio (INR) was 27 s, and 2.0, respectively. Platelet count was 1.0 lakh/mm3. Rest of the liver function tests, creatinine and electrolytes revealed no abnormalities. Abdominal ultrasound revealed gall stones and splenomegaly. Patient was immunised with pneumococcal and haemophilus influenza vaccines. She was hydrated with 1.5 L/day for 2 days and received 5 units of packed cells, 5 units of fresh frozen plasma (FFP). Injection vitamin K (10mg, IV BID) was administered preoperatively for two days. Post-transfusion, Hb was 9.9 g%, platelet count was 1.0 lakh/mm3 and PT, INR was 18.1 s and 1.35, respectively. 3 units of FFP, 2 units of platelet concentrates and 2 units of packed cells volume were kept ready for intraoperative use.
On the morning of surgery, venous access was secured with a wide bore 16G intravenous (i.v) canula and infusion of Ringer's lactate started. She was pre-medicated with injection glycopyrrolate and fentanyl. After pre-oxygenation for 3 min, anaesthesia was induced with injection thiopentone sodium and succinylcholine was used to facilitate intubation which was done with 6.5 no. cuffed Portex® tube. Anaesthesia was maintained with O2, N2O, fentanyl, isoflurane and vecuronium. End tidal CO2 was maintained between 30 and 35 mmHg. Intra-operatively hypoxia, acidosis were avoided. Hypothermia was avoided by infusing warm i.v fluids, warm blood and by wrapping the patient's extremities with cotton. Patient received 3 units of FFP, 2 units of platelet concentrates, 1 unit of packed cells after clamping of splenic vessels. Surgery lasted for 4 h. She received 2 litres of crystalloids and her urine output was 425 ml. Blood loss was 400 ml. At the end of the surgery, port sites were infiltrated with 0.25% bupivacaine. Trachea was extubated at the end of surgery. The patient received 1 unit of packed cells post-operatively. I V paracetmol infusions and I V fentanyl were used to alleviate post-operative pain.


A 37-year-old woman presented with a 5-month history of chronic sinusitis without fever 1 year after allogeneic allograft bone marrow transplant for idiopathic aplasia. Computed tomography (CT) scan revealed right maxillary sinusitis and left focal basal pneumonia, without cavitation (). Bronchoalveolar fluid (BAL) revealed Actinomyces spp. in culture. The patient responded well to right maxillary antrostomy and high doses of intravenous and then oral amoxicillin for 5 months.


A 53-year-old man with long-term tobacco and alcohol abuse was admitted for asthenia and loss of 20 kg in 6 months. The patient had had a cough for several months, but never experienced hemoptysis. Physical examination revealed a right pulmonary crackling sound. A sinus tract, which followed a 3-week history of subcutaneous abscess, was observed in the right face of the thorax. Chest X-ray and CT scan revealed multifocal pneumonia with right pleural cavitation, in the face of the sinus tract (). Magnetic resonance imaging (MRI) revealed contiguous thoracic spine involvement, with paravertebral abscess (see “Extrafacial bone and joint actinomycosis”). BAL revealed A. viscosus in culture. The patient responded well to prolonged amoxicillin therapy (9 months).


A 43-year-old woman presented with lumpy jaw syndrome following root canal treatment of two right mandibular molar teeth (numbers 45 and 46). Tooth 46 was extracted (), followed by tooth 45 2 months later () as mandibular Actinomycosis due to A. naeslundii was diagnosed after surgical debridement and decortication. Prolonged high doses of amoxicillin (9 g/day intravenously over 6 weeks, followed by 6 g/day orally) was prescribed. One year later, despite continuation of antimicrobial therapy, the patient worsened, with extension of the osteomyelitis to the gonial angle and to the ramus (). New surgery was performed 3 weeks after discontinuation of amoxicillin therapy, and A. naeslundii susceptible to penicillin G was again found in cultures from perioperative samples. Amoxicillin was prescribed for 14 months, and, after 1 year of follow-up after amoxicillin discontinuation, no relapse was observed.


A 77-year-old woman with past history of breast cancer was admitted 7 years after radiotherapy for left mandibular metastasis with left mandibular pain, buccal-sided bone exposure, and sinus tract (). The patient had been receiving long-term trastuzumab therapy with bisphosphonate. As the patient was suspected to have chronic osteomyelitis with actinomycosis, left total hemimandibulectomy was performed. Pathology revealed suppurative osteomyelitis. No sample was sent for the microbiological diagnosis.


A 61-year-old woman with paraplegia was admitted for chronic and extensive back scar (20×20 cm). MRI showed back soft tissue infiltration, posterior epiduritis, and infection of the L2 and L4 vertebral bodies (). Laminectomy and excision of necrotic tissue was performed. A. meyeri grew in cultures with Escherichia coli and Staphylococcus aureus.


A 59-year-old woman with appendectomy as past medical history was admitted for acute peritonitis with fever. CT scan revealed an IUD, which had not been changed for 20 years, peritoneal effusion, and heterogeneous pelvic mass (). Coelioscopy showed intraperitoneal pus and a right pelvic mass, with inflammation of the adherent small intestine, without evidence of perforation. The IUD was removed, as salpingitis and endometritis were also found. As actinomycosis was suspected, intravenous high doses of amoxicillin were started. Streptococcus spp., Bacteroides fragilis, and Gardnerella vaginalis were found in cultures. Actinomyces spp. did not grow in cultures. Pathology found inflammation with sulfur granules suggesting pelvic actinomycosis. The patient responded well to prolonged oral amoxicillin therapy.


A 78-year-old man had a past history of endarterectomy, open surgery for deep gastric ulcer, abdominal wall implant for eventration, and cholangiocarcinoma. Following the surgery for cholangiocarcinoma, the patient experienced chronic enterocutaneous fistula. CT scan showed enterocutaneous fistula () and multiple large abdominal abscesses (). Surgical exploration revealed liquefaction of the wall implant. E. coli, Streptococcus anginosus, and Enterococcus spp. grew in cultures, but Actinomyces spp. did not, whereas pathology found aggregates of Actinomyces, typical sulfur granules, and filamentous bacteria (). A prolonged antimicrobial therapy with amoxicillin cured the patient.


A 50-year-old man with alcohol and tobacco abuse was admitted for aphasia and left hemiparesis. CT scan revealed a right temporoparietal lesion with perilesional edema (). Stereotaxic puncture revealed pus with Fusobacterium nucleatum and A. meyeri in cultures.


A 65-year-old obese man was admitted for left facial cellulitis with left pinna edema following ear acupuncture. MRI revealed abscess of the left earlobe (). Surgical drainage of pus revealed Actinomyces spp. in cultures. The outcome was favorable after 6 weeks of intravenous high doses of amoxicillin, followed by oral therapy for 4 months.


In February 2012, an 86-year-old female was diagnosed with bladder cancer extending to the urethra. Accordingly, the surgeon decided to perform a radical cystectomy followed by bilateral ureterosigmoidostomy (sec. Mainz type II).
The post-surgical period was complicated by recurring pyelonephritis and rectal incontinence. A multidisciplinary team of urologists, oncologists, and interventional radiologists came up with two possible alternatives. The first one was a new surgical approach in order to create a definite bilateral ureter-ileocutaneous anastomosis; the second one consisted of a percutaneous bilateral occlusion of both the ureters, coupled to bilateral permanent nephrostomic tube placement. As the patient refused to undergo surgery, the second alternative was performed.
Under USG-guidance, an inferior calyx of the kidney was punctured bilaterally by means of an Accustick introducer system (Boston Scientific, Natick, MA, USA). The procedure was then carried out under fluoroscopic guidance. A 6-Fr sheath (Terumo, Tokyo, Japan) was placed on a 0.035 stiff guidewire (Amplatz Super Stiff; Boston Scientific, USA) bilaterally. Antegrade pyelography was performed in order to exactly recognize both ureterosigmoidostomies. A 10 × 7 mm AMPLATZER vascular plug II was deployed bilaterally in the ureters, proximal to the anastomosis. Then, a 2.7-Fr microcatheter (Terumo, Japan) was bilaterally introduced through the sheath and the tip advanced into the AMPLATZER mesh. The microcatheter was subsequently flushed with 5% glucose saline before injecting 1.5 ml of NBCA mixed with ethiodized oil (Lipiodol UF; Guebert, Aulnay-sous-Bois, France) in a 1:1 ratio with subsequent immediate removal of the microcatheter. A slight caudal migration of NBCA was noticed on the left side, without any significant drawback. An 8-Fr nephrostomic tube (Boston Scientific, USA) was placed bilaterally in order to drain the urinary flow. Following the procedure, trans-nephrostomic bilateral antegrade pyelography showed immediate and complete ureteral occlusion on both sides []. The patient was discharged the following day. A week following the procedure, the patient underwent a new trans-nephrostomic bilateral antegrade pyelography confirming the complete occlusion of both ureters.


A 67-year-old male patient was referred to our hospital for further evaluation of a hepatic mass. He complained of general weakness and weight loss during the past 10 months. He was taking medications for diabetes mellitus, hypertension, prostatitis, and atrial fibrillation, and denied any previous intake of alcohol or smoking.
The initial laboratory findings demonstrated elevated serum alkaline phosphatase level (201 IU/L), while all other liver function tests were within normal limits, including aspartate aminotransferase 17 IU/L, alanine aminotransferase 15 IU/L, and total bilirubin 0.8 mg/dL. Serum alpha-fetoprotein and protein induced by vitamin K absence/antagonist-II levels were 16 ng/mL and 27 mAU/mL, respectively. Serologic tests for hepatitis B and hepatitis C virus were negative. Contrast-enhanced computed tomography and magnetic resonance images showed a hepatic mass with peripheral enhancement in segment 8 (). The center of the tumor remained poorly enhanced. A fluorodeoxyglucose positron emission tomography scan showed isometabolism in the hepatic mass (maximum SUV: 3.0) and diffuse hypermetabolism in the multiple bones. The hypermetabolic bone lesions were subsequently confirmed as involvement by multiple myeloma on bone marrow biopsy, urine/serum protein electrophoresis and immunofixation electrophoresis. The hepatic mass was revealed to be an adenocarcinoma on ultrasonography-guided gun biopsy, and a right anterior sectionectomy of the liver was subsequently performed.


Patient 1 is a 26-year-old male that was transferred from an outside hospital secondary to a firework blast to the face. He was described as alert but “restless” upon presentation. The outside hospital physician was not able to intubate the patient after 2 attempts and a cricothyroidotomy was performed at the outside hospital for airway protection. He was hypoxic and tachycardic the majority of the time at the outside hospital. During transport the patient became asystolic and his blood pressure dropped. En route, the patient was started on dopamine drip, and percutaneous pacing was initiated. Upon arrival at the emergency department (ED), his cricothyroidotomy was in place and he was in pulseless electrical activity. His Glasgow Coma Scale (GCS) score was a 3T. Advanced cardiovascular life support was initiated and 3 rounds of epinephrine were given without return of spontaneous circulation. The patient was pronounced dead shortly after his rhythm deteriorated to asystole.


Patient 2 is a 23-year-old male transferred from an outside hospital after being hit in the face from a firework blast. Upon presentation to our institution, the patient had a GCS score of 9T and he was intubated using direct laryngoscopy on the second attempt. Etomidate and succinylcholine were given as the sedative and paralytic. He had extensive injury to his forehead, frontal head, orbits, with exposed facial bones. His face was covered with abrasions and lacerations, his right cornea was burned, and the left eye was ruptured (). The patient’s vital signs were stable.
A computed tomography (CT) of the head and face revealed a 5 mm left frontal epidural with a complex frontal sinus fracture involving the posterior wall of the sinus. Extensive facial fractures were noted involving the paranasal sinuses, the nasal orbital ethmoidal complex, the planum sphenoidale, and the left greater wing of the sphenoid. The left eye was ruptured.
Ophthalmology, neurosurgery, and plastic surgery services were consulted. Ophthalmology took the patient immediately to the operating room (OR) for enucleation of the left eye. Neurosurgery found that there was no surgical intervention appropriate at the time, and plastic surgery deferred any reconstruction until the patient stabilized.
The patient was then admitted to the intensive care unit (ICU), started on dilantin for seizure prophylaxis, and continued on antibiotics for the open wound to the forehead. He was extubated on the third day in the hospital. The patient also sustained a large soft tissue defect to forehead, requiring debridement in OR on the sixth hospital day.
While an inpatient, the patient required pain control, intravenous antibiotic therapy, and physical and occupational therapy. He was discharged and his wounds healed, but he declined further surgery. This patient was lost to follow up after his initial outpatient follow up.


Patient 3 is a 30-year-old male who presented to the ED from the field with extensive facial trauma secondary to a firework blast to the face. His airway was secured immediately upon arrival due to an altered mental status with a GCS of 3 and poor oxygenation. A glidescope intubation with a size 7 tube was performed, with almost immediate replacement of a size 8 tube when an air leak was found. Exchange of the endotracheal tube was guided with the help of a bougie. He was hypotensive initially and had episodes of bradycardia. The hypotension responded to fluids. He had extensive facial blast injury with a large amount of tissue loss of the eyes, forehead and nose (). Both of the eyes were clinically ruptured. The maxilla was unstable upon examination. Focused assessment with sonography for trauma was performed and no free fluid was found. Ophthalmology, neurosurgery, and plastic surgery services were consulted.
CT head revealed diffuse cerebral edema with effacement of the 3rd and 4th ventricles, subarachnoid hemorrhage and bilateral frontal and temporal contusions. CT maxillofacial revealed multiple complex fractures as follows: frontal bone with a comminuted fracture involving the inner and outer table and displacement of both; large defect noted in the anterior and central skull base with destruction of the ethmoid complex and the sphenoid bone; left temporal bone fracture with involvement of the inner ear and mastoid sinus; bilateral orbital, pterygoid, zygomatic and maxillary sinus fractures; fracture of the hard palate with obvious diastases of the bone fragments; and a mandibular body fracture. In addition the CT of the face revealed bilateral globe ruptures with extensive edema of the ophthalmic nerves and extraocular muscles.
The patient was admitted to the ICU. Consultation of neurosurgery, ophthalmology and plastic surgery were obtained. Post injury the patient developed persistent high fevers and was treated with antibiotics, he developed central diabetes insipidus, and was minimally responsive with only a cough and a gag. Eventually he was able to intermittently move his upper and lower extremities with stimulus. No surgical intervention was planned for his injuries.
On hospital day six, the patient underwent tracheostomy, percutaneous endoscopic gastrostomy tube placement, and facial wound debridement, all of which were uneventful. On hospital day seven, the patient had an increasing pressor requirement and fevers. His responsiveness also diminished after the operating room and it was thought that he progressed to brain death. He was in the middle of a brain death workup when he went into cardiac arrest. After 3 cycles of cardiopulmonary resuscitation, the patient was pronounced dead.


Patient 4 is a 28-year-old male that was hit in the face by a firework described as a mortar. The patient was described as being combative during transport by emergency medical services. The patient was not speaking upon arrival, but saturating well on a non-rebreather mask. He was intubated in the ED due to the extensive facial trauma and the risk for loss of airway. Although the patient had lacerations on the mouth, oral intubation with an 8.0 tube was achieved using direct laryngoscopy while maintaining inline stabilization of the cervical spine. The patient was sedated and paralyzed with etomidate and succinylcholine. Glasgow coma scale was 10 (eyes 3, verbal 1, motor 6). Grossly, the patient had a laceration to the scalp, a frontal skull fracture, and avulsion of the left eye (). Vital signs remained stable throughout his ED stay and he was admitted to the ICU after his workup was completed.
CT of the head revealed 3 mm left frontal contusion and bilateral frontal and right parietal subarachnoid hemorrhage. CT maxillofacial revealed multiple facial fractures as follows: bilateral frontal and frontal sinus fractures with involvement of the inner and outer tables; floor roof and lateral wall fractures of the left orbit with absence of the left globe; comminuted ethmoidal, nasal bone and left maxillary sinus fractures; and left sphenoid sinus fracture.
Ophthalmology, Neurosurgery, and Plastic Surgery services were consulted. Ophthalmology removed the remainder of the left globe and orbital debris. Neurosurgery did not offer any surgical intervention and plastic surgery determined that the patient would need reconstruction once stable
On hospital day five the patient underwent tracheostomy and percutaneous endoscopic gastrostomy. The facial wounds were debrided and the midline frontal scalp laceration was repaired. Swallow evaluation done and a soft mechanical diet was started. No progression of his intracranial hemorrhages noted.
On hospital day thirteen, the patient was taken to the OR by ophthalmology and plastic surgery services to begin reconstruction. Left eye enucleation was performed first. The reconstruction of the face included open reduction internal fixation of the left orbital roof and lateral wall, the left maxillary sinus, frontal sinus, left maxilla, upper and lower eyelid reconstruction and repair of complex laceration. On hospital day #21, the patient was discharged home decannulated. This patient had two subsequent operations, the first of which occurred 12 days after discharge. He developed a 1.5 cm dehiscence present at the level of the left orbital rim with exposed plate. A pedicle flap was made based on the previous scar and the area was closed without further difficulty. The second operation was performed 68 days after discharge due to severe contracture development in the left upper and lower eyelid.


A 54-yr-old Korean man, who was a salaried office worker in Incheon, Korea, presented left scrotal pain for 6 yr in February 2009. The size and the general shape of the scrotum were within normal range, but intermittent pain has been the main symptom. The patient had no history of trauma or any scrotal operation. There was no history of hereditary disease or any other chronic disease except hypertension. No habit of ingesting raw reptiles such as snakes or frogs was reported. However, occasional drinking of not purified water from a spring during mountain climbing was revealed in the interview with the patient. The patient did not complain of fever or chilling sensation on the day of outpatient visit. There were no measurable weight loss, abdominal pain, and significant voiding difficulty. Only intermittent sharp and squeezing pain was present at the left testis. On the manual examination of testes, palpable mass, tenderness or redness, and wall thickening of the scrotum were not found. Laboratory tests were all within normal range, including complete blood count, blood chemistry, urinalysis, and testis-specific tumor markers. Ultrasonography of testis revealed a 0.4 × 0.5 × 0.4 cm sized poorly defined, heterogeneous mass with increased echogenicity in the left testis (). No posterior acoustic shadowing was observed. Instead, another hypoechoic ring was identified around the mass () forming a "tubule-in-a-tubule" appearance. On the color Doppler examination, moderately increased flow was noted in the periphery of the mass. At the time testicular teratoma was strongly suspected by our radiologist. Radical orchiectomy was performed under the impression of left testis tumor. Under the spinal anesthesia, about 7 cm long left inguinal incision was made. A 15-mL sized round firm testis was identified. En bloc resection of the left testis was done after thorough exploration verifying no other structural abnormalities were present. On the second day after the operation, patient was discharged with oral antibiotic medication without complication. Pathological examination of the resected tissue showed the sparganum was present in the testicular parenchyma within tunica albuginea and surrounded by abscess and necrotic materials (). And at close up view, tortuous recess of the bothrium, tegument, longitudinal layer of the smooth muscle, and loose internal stroma were seen and multiple calcarous bodies were present in the loose internal stroma (). This finding was consistent with the diagnosis of sparganosis in the tesitis. Further treatment was not given to the patient.


A 53-year-old Caucasian female with complaints of dry eyes and a burning sensation in the eyes presented to our ophthalmology clinic 5 years ago. Her medical history revealed that the symptoms had persisted for 10 years, with some relief with lubricating eye drops used every 0.5–1 h. Prior serology studies for antinuclear antibodies (ANA), Ro and La were all negative. She denied any symptoms of dryness of her mouth, and she had no other comorbidities. There was no family history of autoimmune diseases.
Clinical examination showed dry eyes, with slit lamp examination revealing thickening and hyperemia of the eyelids. Schirmer's test was very low at 3 mm in each eye. Her laboratory evaluation included a normal complete blood count and a comprehensive metabolic profile. ANA, anti-Ro and anti-La were all negative. She was put on GenTeal gel alternating with artificial tears (Restasis eye drops) four times/day. During the course of the next 5 years, she underwent multiple surgeries for persistent eye dryness, including three procedures of silicone punctual plug placement in each eyelid, permanent thermal punctual occlusion, and later, resection of the canaliculus due to repeat reopening of the punctum despite three permanent thermal occlusions and continued patient discomfort secondary to refractory dryness of the eyes. Additional evaluation to determine the etiology of dry eyes was done. It revealed the presence of antibodies to salivary gland protein 1 (Sp1) and parotid secretory protein (PSP), leading to the diagnosis of Sjogren's syndrome (SS).


A 68-year-old Caucasian female with a known history of rheumatoid arthritis (RA) presented to our ophthalmology clinic with complaints of persistent dry eyes and irritation for the last 25 years. She had tried artificial tears, Restasis eye drops and GenTeal gel with some relief. Her prior workup by her rheumatologist included antibodies to Ro and La, which were both negative. Her medications included methotrexate for her RA for the last 30 years.
On physical evaluation, she was noted to have dry eyes. Slit lamp examination revealed hyperemia and thickening of the eyelids. Schirmer's test was very low at 1mm in both eyes. Her laboratory evaluation in our clinic included antibodies to Ro and La that were negative and antibodies to ANA and rheumatoid factor, which were both positive. Evaluation of additional autoantibodies revealed the presence of antibodies to Sp1 and PSP, leading to the diagnosis of SS. Antibody testing for Sp1 and PSP in both patients was done at Immco Diagnostic Laboratory, Buffalo, N.Y., USA.


A 39-year-old female, with a history of anxiety, presented to our metropolitan hospital with a 3-day history of central abdominal pain radiating through to her back with nausea and vomiting.
Initial laboratory investigations revealed serum lipase of 640 U/l (a normal range of 8–57), bilirubin 9 µmol/l (<25), ALP 160 U/l (30–120), GGT 132 U/l (<51), AST 36 U/l (<41) and ALT 39 U/l (<41). Abdominal ultrasound demonstrated gallstones and a diagnosis of biliary pancreatitis was made. After 7 days her pain improved and was discharged home.
A planned elective laparoscopic cholecystectomy was performed 3 days after discharge. Cholecystectomy was uncomplicated and an intra-operative cholangiogram was unremarkable. After resection and delivery of the gallbladder, there was an ongoing accumulation of milky fluid within the hepatorenal space. The fluid appeared chylous and a midline laparotomy was performed with an aim to identify the leak site. At the base of the mesentery small blocked lymphatics could be seen (Fig. ). No definite site of chyle leakage could be identified. Two drains were placed, one in the gallbladder bed and other in the base of the mesentery. Analysis of the fluid demonstrated a chyle leak with triglyceride level of 410 mg/dl and amylase of 60. Serum triglycerides were 130 mg/dl.
Post-operatively, the patient was kept nil-by-mouth and given total parenteral nutrition. The patient was put on low fat diet 4 days later. The drains were removed on Day 15 post-operatively. The patient was well when seen again 6 months later.


A 21 year old male Nigerian student and a known
asthmatic presented to the Emergency Department
of Federal Medical Centre, Abeokuta, southwestern
Nigeria with a 10 hour history of cough productive
of whitish sputum, increasing difficulty with breathing,
wheezing and neck pain. He was diagnosed asthmatic
at the age of eleven and had been admitted on a few
occasions for acute exacerbations in the prior ten years.
He had salbutamol tablets regularly.
At this index presentation, he was noted to have
subcutaneous swelling and crepitus over the neck and
upper anterior chest region, bilateral and polyphonic
rhonchi with prolonged expiratory phase. He had a
respiratory rate of 36 cycles per minute, pulse rate of
120 beats per minute and a blood pressure of 120/
80mmHg. Other systems were essentially normal. The
chest radiograph showed low set hemidiaphragms and
bilateral basal emphysema in keeping with the known
asthmatic state. In addition, there was a linear lucency
in the region of the left border of the heart as well as
areas of lucency in the subcutaneous tissue of the neck
and region of the left side of the chest wall respectively
(see figure and ).
Laboratory findings including electrolyte levels were
within normal limits.
On the basis of the history, examination and
radiological findings, a diagnosis of acute exacerbation
of bronchial asthma with pneumomediastinum and
subcutaneous emphysema was made.
He was admitted into the male medical ward and
treatment offered included administration of
salbutamol inhaler, parenteral hydrocortisone,
aminophylline and augmenting as well as humidified
oxygen by nasal prongs. He made remarkable
improvement with resolution of the initial symptoms
about 48 hours after admission. He was discharged
after 5 days of admission on seretide inhaler and
remained in stable clinical state thereafter.


A 49 year-old man who presented with recurrent upper
abdominal pain of 9 years, and 6 months history of
right hypochondrial swelling and weight loss. He also
had history of early satiety and vomiting. No history
suggestive of upper gastrointestinal bleeding. Clinical
examination revealed wasting, pallor and ankle oedema.
He had hepatomegaly which was firm, irregular and
tender. Succussion splash was positive. An impression
of gastric outlet obstruction secondary to antral
carcinoma to rule out carcinoma head of pancreas
with hepatic metastasis was made.
Abdominal ultrasound scan revealed hepatomegaly
and a mass distal to the stomach.
Peripheral blood film examination revealed iron
deficiency anaemia and thrombocytosis, while serum
chemistry showed hypoalbuminaemia.
An upper gastrointestinal endoscopy revealed a mass
in the second part of the duodenum which was
ulcerating and vascular. Multiple biopsies were taken
for histopathology. The tissue was sectioned and stained
with haematoxylin and eosin. It showed spindle shaped
cells containing large nuclei with blunt edges and were
surrounded with mild to moderate cytoplasm. There
is loss of overlying mucosa . Features are in keeping
with gastrointestinal stromal tumour .
Exploratory laparotomy revealed a mobile mass in
the 2nd and 3rd parts of the duodenum which was
firm-hard, irregular, measuring about 12cm in
diameter. There were metastases to the liver and
paraaortic lymph nodes. He had gastrojejunostomy and
paraaortic lymph node biopsy for histology which also
revealed a metastatic gastrointestinal stromal tumour.
Postoperatively, patient was stable and he was
discharged home 11 days after . He is presently stable
and on follow up .He has been commenced on cyclical
cytotoxic chemotherapy using 5- fluorouracil.


A 14.7-year-old boy was presented to Chonnam National University Hospital because of extremely tall stature. His height was 192.0 cm (14 cm above the 97th percentile) and body weight was 70.5 kg (90th-97th percentile). He showed enlarged hands and feet, and prognathic mandibles. His body proportion was normal and his pubertal stage was mature (Tanner stage 5). Bone age was normal for chronological age according to the method of Greulich and Pyle. There was no family history of tall stature (father, 176.0 cm; mother, 167.0 cm) or any endocrine diseases. Laboratory investigation showed the following results; random serum GH, 38.4 ng/mL (normal range, 0-5 ng/mL); insulin-like growth factor 1 (IGF-1), 624.0 ng/mL (normal range for age, 220-616 ng/mL); IGF-BP-3, 6,301.6 ng/mL (normal range for age, 2,200-5,900 ng/mL); and prolactin, 8.94 ng/mL (normal range, 3-18 ng/mL). GH failed to suppress during an oral glucose tolerance test (OGTT; nadir serum GH, 22.7 ng/mL [normal range, <1 ng/mL]). Magnetic resonance imaging (MRI) of the brain revealed a 12-mm-sized pituitary adenoma (). Neither pituitary insufficiency nor visual impairment was present. Transsphenoidal surgery was perfor-med and a pathologist reported a pituitary adenoma with positive immunohistochemical staining for GH (). A pituitary MRI scan performed 4 months after surgery showed recurrence/residual tumor with a size of 5 mm and a basal GH level of 7.1 ng/mL (normal range, 0-5 ng/mL). Medical treatment with intramuscular injection of the long-acting somatostatin analogue octreotide LAR (Sandostatin LAR, Novartis Pharma AG, Basle, Switzerland) at a dose of 20 mg was given every 4 weeks. Six months after medical treatment, the serum GH levels increased further, and nadir GH during OGTT was 6.6 ng/mL (normal range, <1 ng/mL). Therefore, the patient underwent secondary surgery. Three months after reoperation, the GH level was 0.2 ng/mL and IGF-1 was 205 ng/mL (normal range, 220-616 ng/mL). During 2 years' follow-up, adrenal, gonadal and thyroid functions remained unchanged and annual MRI showed no signs of relapse. GH and IGF-1 levels also showed within normal range. His height was 200.8 cm (>97th percentile) at age of 16.7 years.


A 14.9-year-old boy was referred to our pediatric clinic due to extremely tall stature. His height was 197.3 cm (19 cm above the 97th percentile) and body weight was 101.5 kg (20 kg above the 97th percentile). He showed enlarged hands and feet, and prognathic mandibles. His body proportion was normal. The pubertal status was mature (Tanner stage 5). He showed advanced bone age of 16.0 years according to the method of Greulich and Pyle. There was no family history of tall stature (father, 175.0 cm; mother, 163.0 cm) or any endocrine diseases. Endocrine investigation revealed the following: basal serum GH, 9.3 ng/mL (normal range, 0-5 ng/mL); IGF-1, 518.0 ng/mL (normal range for age, 220-616 ng/mL); IGF-BP-3, 3,450.0 ng/mL (normal range for age, 2,200-5,900 ng/mL); and prolactin, 10.39 ng/mL (normal range, 3-18 ng/mL). GH failed to suppress during an OGTT (nadir serum GH, 9.0 ng/mL [normal range, <1 ng/mL]). A pituitary MRI scan showed a 6-mm-sized pituitary adenoma (). Transsphenoidal surgery was performed and histopathological examination demonstrated a pituitary adenoma with positive immunohistochemical staining for GH (). Three months after surgery, the GH level was 0.2 ng/mL (normal range, 0-5 ng/mL) and nadir GH during OGTT was less than 0.1 ng/mL (normal range, <1 ng/mL). Pituitary MRI scans at 6 months postsurgery showed no residual tumor. During 4 years' follow-up, adrenal, gonadal and thyroid functions remained unchanged and annual MRI showed no signs of relapse. GH and IGF-1 levels also showed within normal range. His final height was 206.0 cm (>97th percentile) at age of 19 years.


A 23-year-old pregnant woman (10 weeks of gestation) with mechanical mitral prosthetic valve (operated 5 years ago) was due to an exertional dyspnea admitted to our hospital. She did not notify her medical doctor about her pregnancy and had continued to intake 7.5 mg of warfarin on her own. The international normalized ratio (INR) was 2.4 on admission and the blood pressure was 130/80 mm Hg. The patient had a previous history of pregnancy loss at the 16th week. Transthoracic echocardiography on admission revealed increased Doppler gradients (maximum/mean gradient: 17/10 mm Hg) and decreased mitral valve area (MVA) of 1.5 cm2 indicating a prosthetic valve obstruction. Transesophageal echocardiography delineated a thrombus area of 1.5 cm2 on the mitral prosthesis (). Thrombolytic therapy (TT) with low dose (25 mg) and slow infusion (6 hours) of tissue-plasminogen activator (tPA) was administered as described recently. After TT, the MVA increased to 2.8 cm2 and the transmitral gradient decreased (maximum/mean gradient: 11/5 mm Hg) with a complete lysis of the thrombus (). An elective curettage was performed to avoid a potential embryopathy due to the relatively high dose warfarin consumption. Two years later, the patient suffered from two pregnancy losses at the 18th and 20th week respectively. Anticardiolipin immunoglobulin (Ig) G and IgM, lupus anticoagulant and toxoplasma antibodies were studied and showed all negative results. Factor VIII and fibrinogen levels were within normal limits. Genetic testing was performed using CVD Strip Assay Kit (ViennaLab Diagnostic GmbH) and no mutations were observed in the Factor V Leiden, factor V H1299R, prothrombin G20210A, factor XIII V34L, methylenetetrahydrofolate re ductase (MTHFR) C677T, plasminogen activator-inhibitor 1 4G-5G, GPIIIa L33P, ACE, ApoB R3500Q and ApoE. However, homozygous mutations of MTHFR A 1298C, and heterozygous mutations of β-fibrinogen 455 G-A were detected. One year ago, the patient became pregnant again and suffered another episode of obstructive PHVT in the 33rd week of her gestation despite strict anticoagulation (low molecular weight heparin with effective antifactor Xa levels in the first trimester and INR between 2.5 and 4 in the second and third trimester). The PHVT was again succesfully thrombolyzed (totally 50 mg of tPA). She delivered by cesarean section an alive and healthy newborn at the end of the 37th week of gestation.


A 38-year-old married female without any past or family history of psychiatric or neurological illness and with good premorbid functioning. She was harboring delusion of infidelity against her husband along with disturbed socio-occupational and biological functioning for the past 6 years. There was no history of substance use disorders, except excessive caffeine use, or any high risk sexual behavior. Organic psychiatric disorder or symptoms of schizophrenia were not evident. Detailed general physical examination including neurological evaluation did not reveal any abnormality except mild pallor. She reported regular menstruation and the last childbirth was 10 years back. Mental status examination revealed that she was having an uncooperative and aggressive attitude, delusion of infidelity, impaired judgment, and lack of insight. So, she was admitted to psychiatry ward with a diagnosis of persistent delusional disorder as per International Classification of Diseases, 10th edition. She was started on intramuscular olanzapine 10 mg/day. On investigation, her complete hemogram revealed a Hb of 11.5 gms/dL and a serum ferritin level of 30.2 ngm/ml (normal value for females is 10-291 ng/mL). Other routine hematological and biochemical parameters including blood sugar, renal, liver, and thyroid function were within normal limits. We also planned a vitamin B12 and folic acid estimation which could not be done due to lack of resources.
Within 4-5 days of starting olanzapine, there was a decrease in her aggression and she became cooperative for interviewing. However, she would report that she was having sleeping difficulty due to an itching sensation in both upper and lower limbs at night. She would rub her legs against bedclothes, tie pieces of cloth around her limbs, and ask for limb massage to get relief from such a discomfort. She would have such symptoms for hours after lying down at night. During daytime, she would, however, feel comfortable, relaxed, and was able to sleep. She was evaluated for sleep disorder-before this she never had any sleep complaint, neither was there any family history of sleep disorder. During sleep, there was no history of snoring, no abnormal limb or breathing movements. Ear, nose, throat examination was also noncontributory. She was having a thin built and a body mass index of 15.56. As she became cooperative, she was shifted to oral psychotropics (olanzapine 15 mg/day + clonazepam 1 mg at bedtime). Also oral iron supplementation in the form of ferrous fumarate 350 mg, vitamin B12, and ascorbic acid was started. To improve her sleep, general sleep hygiene practices were initiated, she was prohibited from taking any caffeine beverages at night and the clonazepam was further increased up to 2 mg at bedtime. But there was no improvement in her nighttime symptoms.
In view of her above symptoms and investigations, she was diagnosed to have RLS with an International Restless Legs Scale Score (IRLS) of 24 indicating high severity and olanzapine was considered to be the probable cause. Hence, the olanzapine was decreased to 10 mg/day and oral risperidone 2 mg was added at bedtime. On the next day, the severity of symptoms decreased to a RLS score of 12. No other change in medication was done during this period. So, after 2 days, olanzapine was stopped and risperidone was increased to 6 mg at bedtime. Thereafter, her troublesome symptoms ceased completely. Though she still harbored the delusions yet the conviction decreased and she became more cooperative with her husband and the treating clinician. Then after about a week of uneventful ward stay, she was discharged free of her sleep-related difficulties on risperidone 6 mg and clonazepam 1 mg at bedtime with the plan to continue her antipsychotic drugs and iron supplementation on an outpatient basis.


A 20-year-old Hispanic male with no past medical history presented to our institution complaining of generalized weakness for 4 weeks, progressive cough with yellowish sputum, as well as persistent shortness of breath for 3 weeks, and intermittent fever and chills for 3–5 days prior to admission. Family history was remarkable only for coronary artery disease in his father. He denied smoking, alcohol, and using illegal drugs. He had lived with his girlfriend for 2 years and stated to be monogamous over this period. He reported a total of two sexual partners in his life. He was born in Texas and had not traveled outside the US for the last 12 years. Physical examination revealed a cachectic and tachypneic patient in no acute distress with bilateral diffuse crackles and rhonchi. The initial laboratory workup was within normal limits. Computed tomography of the chest () showed diffuse tree-in-bud nodularity, bronchiectasis, and thick-walled cavities in the upper lobes and moderate right simple pleural effusion. The HIV-rapid enzyme-linked immunosorbent assay test was reactive. The acid-fast bacilli sputum test was positive, and the patient was started on combination treatment for tuberculosis. The patient improved clinically over the next 3 days. The HIV Western blot test was negative; the viral load was undetectable. The CD4+ and CD8 counts were low (124 and 185, respectively) with a low CD4:CD8 ratio of 0.7. Further workup including immunoglobulin A, immunoglobulin G, and immunoglobulin M levels, urine protein electrophoresis, and serum protein electrophoresis was unremarkable. The pleural fluid cytology revealed atypical lymphocytes consistent with the established diagnosis of pulmonary tuberculosis. Further imaging of the abdomen and pelvis showed no abnormalities.
In addition, 8 weeks after discharge the patient was seen in clinic. He was taking his medication to treat tuberculosis and had regular health care follow-up. He was found to be completely asymptomatic with a CD4+ count of 188 cells/mm3. The repeated HIV test was negative, and the viral load was undetectable. After finishing his treatment for tuberculosis, he was evaluated in clinic, had a CD4+ count of 212 cells/mm3, and remained asymptomatic.