A 26-year-old healthy man visited our outpatient clinic with presenting symptoms that included recurrent jerky movements, which had been occurring for the previous 3 days. Asynchronous jerky movements appeared in his left face and neck area and lasted approximately 5 minutes, with irregular frequency, over the 3 days before his first visit to the hospital. The jerky movement was observed to be a form of myoclonus. He also complained of an episode of paresthesia that had occurred 10 days previously, starting in his left hand and gradually spreading up and over his left arm, trunk, face, and leg for approximately 2 minutes. He was an office worker with no history of headache, stroke, or seizure. There was no reported family history of headache or other neurologic disorders. A neurological examination revealed no focal neurological deficit between jerky movements. Magnetic resonance imaging and magnetic resonance angiography had normal findings. Cerebrospinal fluid analysis was normal (opening pressure, 15 cmH2O; white blood cell count, 0/mm3; red blood cell count, 0/mm3; protein, 26.4 mg/dL; glucose, 63 mg/dL). Electroencephalography (EEG) findings were normal for both the awake and sleep-deprived conditions. The jerky movements spontaneously disappeared less than 1 day after admission without specific treatment.
The patient revisited the clinic 7 days after discharge, presenting with excruciating headache on the right orbitotemporal area. He had never experienced this type of headache previously. He reported that the headache occurred around the times of sleep onset and waking, and lasted for 1-2 hours. Unilateral autonomic symptoms including lacrimation, rhinorrhea, eyelid edema, and ptosis appeared on the same side as the headache focus. However, symptoms of nausea, vomiting, photophobia, and phonophobia were absent. Thus, we diagnosed this patient as having cluster headache and referred him to the outpatient clinic for commencement of the following medical treatment: verapamil (40 mg tid), valproate (300 mg tid), and zolmitriptan (2.5 mg prn).
The patient developed a third cluster headache attack, which was associated with dysarthria and hemiparesis. The clinical features of this headache were the same as for the previous two. The hemiparesis was contralateral to the side of the headache, lasted 3 hours, and occurred within 30 minutes of cluster onset. During hemiparesis, the left upper limb was Medical Research Council grade 3 and the left lower limb was grade 4. His deep tendon reflex was normal and the Babinski sign was negative. Repeat brain Magnetic resonance imaging and magnetic resonance angiography revealed no abnormal findings. Echocardiography and laboratory tests such as lipid profile and autoimmune antibodies, including lupus anticoagulant, rheumatoid factor, antinuclear antibody, antineutrophilic cytoplasmic antibody, and anticardiolipin antibody, were normal. Using 24-hour video EEG monitoring, we detected lateralized slow waves on the right hemisphere during a headache accompanied by hemiparesis (). The slow-wave activity normalized after the headache subsided with the aid of oxygen inhalation (). Overall the patient experienced a total of 16 headache attacks over a 4-week period, each of which lasted from 15 minutes to 3 hours, and with a frequency of one to five times per day. The headaches occurred mainly at night (6 p.m. to 8 p.m.) and during the early morning (4 a.m. to 6 a.m.). He experienced daytime headache attacks only occasionally, two of which were accompanied by hemiparesis and lasted for 3 hours and for 30 minutes. During headache attacks, the patient's pain was relieved by oxygen inhalation. In addition, 60 mg of prednisone was prescribed to reduce the severity and frequency of headache, and this proved to be effective.
The patient was discharged without any neurological deficit. He was prescribed verapamil and valproate for 2 months th-rough the outpatient clinic, and prednisone tapered over 5 weeks. He did not complain of further headaches and did not want to continue this treatment due to gastric side effects, so we inst-ructed him in the use of zolmitriptan and oxygen therapy for acute treatment. No headache episode occurred after his discharge.


A 41-year-old man was referred to our institute with a 1-year history of persistent headache and a mass in the left temporo-occipital region, the mass had developed in the last year and rapidly grown within 6 months.
The physical examination revealed a fixed, five centimeters solid mass. The neurologic examination on admission showed no abnormalties. The computed Angiotomography showed a large extrinsic solid lesion with epidural mass effect and contrast enhancement causing bone destruction. MRI showed a supra-infratentorial tumour location with invasion of the scalp and left transverse dural sinus ().
Endovascular embolization was not possible and the patient underwent surgery. Intraoperatively, the tumour was found immediately in the scalp appearing as a soft, reddish, highly vascularizated mass with dural invasion. The inferior and posterior borders of the tumour encased the left transverse sinus; the tumour was successfully dissected employing microsurgical techniques. Finally, a complete resection of the tumour was achieved. Postoperatively, the patient was extubated and observed in the surgical intensive care unit. He remained free from neurologic deficits. Histological examination revealed a thyroid differentiated neoplasm with positive staining for thyroglobulin and follicular carcinoma in immunohistochemical analysis ().
Postoperatively levels of thyroid hormones were normal. Radionuclide imaging with Tc99 demonstrated a thyroid mass. Radiological neck, chest and abdominal examination revealed no other metastatic localisations. The patient received 6 courses of treatment with Paclitaxel.


A 22-year-old woman was referred to our institution with a past medical history of wedge resection of the left lung for two incidentally detected pulmonary nodules at another hospital fifty months earlier. The initial outside multi-phase dynamic CT scan obtained at that time using a multidetector CT scanner (Sensation 16, Siemens, Erlangen, Germany) after intravenous injection of contrast media was available for review, although the information on the amount and the brand name of the contrast media was not available. The CT images revealed two nodules each in the left upper and lower lobes. These nodules were round in shape with a well-circumscribed margin, and maximum diameters of 22 and 20 mm, respectively. On non-contrast CT scan, the nodules revealed no calcification. On multi-phase dynamic CT images, the nodules showed peripheral nodular enhancement at the early arterial phase obtained 1 minute after intravenous administration of contrast media and a subsequent "central filling-in" enhancement pattern on the delayed scans (obtained 2 and 4 minutes after contrast enhancement) (), which was identical to the well-known enhancement pattern of hemangiomas of the liver. Wedge resection of those left pulmonary nodules was performed at the other hospital, and the review of the pathologic specimen revealed pulmonary capillary hemangiomas.
At 50 months follow-up, new pulmonary nodules were detected on a chest radiograph, and the patient visited our institution for further evaluation. The CT scan obtained at our institution using a multidetector CT scanner (LightSpeed VCT, GE Healthcare) after intravenous injection of 100 mL (3 mL/s) of nonionic contrast media (Iopamiron 300; Bracco, Milan, Italy) revealed two recurrent pulmonary nodules each in the left lower lobe and the right lower lobe with maximum diameters of 30 and 12 mm, respectively. These nodules also showed a round shape with a well-circumscribed margin. The larger one detected in the left lower lobe especially showed the prominent "peripheral nodular enhancement" pattern at post-contrast CT images obtained 40 seconds after contrast administration (), which was the same as the previous CT finding.
A left lower lobectomy and wedge resection of the right lower lobe was performed, and the gross specimens of the resected nodules showed well-demarcated hemorrhagic or vascular nodules (). Histopathologic examination revealed vascular tumors of a capillary growth pattern. These nodules were composed of anastomosing capillary vascular channels filled with red blood cells. These capillary lumina were divided by narrow trabeculae of hyaline stroma (), which were identical to those of the previously resected pulmonary nodules at the other hospital. The final diagnosis was recurrent pulmonary capillary hemangiomas, and the tumors showed no evidence of histologically malignant features such as cellular atypia or mitosis.


A 65-year-old man accessed our clinic with a non-healing ulcer over the right hand for the last 1 year. The lesion initiated as a pea-sized papule, which subsequently increased in size and ulcerated in the centre. The patient was a diagnosed case of generalized vitiligo for the past 30 years, for which, he had been treated with topical corticosteroids without any response. The patient did not receive any form of phototherapy or immunosuppressive therapy. He was a daily wager, working outdoors for prolonged hours. He had never used photoprotective measures. The patient denied exposure to arsenic, X-ray irradiation and occupational carcinogens. He vehemently denied any intake of Ayurvedic medications for vitiligo. There was neither a history of vitiligo nor any malignancy in other family members.
Cutaneous examination revealed an exophytic growth, 4 cm across, present over the dorsal aspect of the right hand, adhered to the underlying subcutaneous tissue []. The surface of the growth was marked by ulceration in the centre. The rest of the cutaneous and systemic examination was normal. The histopathological examination of the sections from the margin of the ulcer revealed the presence of a tumour infiltrating the dermis and the subcutaneous tissue in the form of solid nests and sheets. The tumour cells were polygonal with eosinophilic cytoplasm. The nuclei were vesicular with prominent eosinophilic nucleoli. There was abundant keratinization in the centre of solid nests []. A diagnosis of well-differentiated keratinized squamous cell carcinoma (SCC) was made. The ulcerated nodule was excised with a 1-cm margin and the lesion was allowed to heal by secondary intention. The patient was advised to avoid sun exposure, follow adequate photoprotective measures and report for any suspicious lesion. No recurrence was noticed after a follow-up of 1 year.


A 8-year-old boy with 45,XO/46,XY DSD with proximal hypospadias was presented to our hospital for further examination for a left inguinal mass. He underwent one-stage hypospadias repair for scrotal hypospadias and exploratory laparotomy in another hospital when he was one year old. At that time the right spermatic vessels and vas deferens, which ran into the left inguinal internal ring along with the left spermatic vessels and vas deferens, was found in the exploratory laparotomy, and the right testis was diagnosed as a vanishing testis via left scrotal approach because a similar structure like the nubbin was located cephalad to the left testis. After that, he was referred to our hospital for a small tumor positioned cephalad to the left testis. Physical examination revealed a normally positioned left testis and a small tumor cephalad to the left testis. Ultrasound showed a homogeneous mass like a normal testis cephalad to the left testis, so we suspected right TTE. We performed laparoscopic and left inguinal explorations to confirm the diagnosis. Laparoscopy revealed that the right spermatic vessels and vas deferens ran into the left inguinal internal ring along with the left spermatic vessels and vas deferens ,and we didn’t find any persistent Mullerian duct and right intraabdominal testis []. Subsequently, we found two separate spermatic cords and gonads in the left inguinal exploration []. The perioperative histopathological examination revealed normal testicular tissue, and therefore we performed trans-septal orchidopexy for the right testis under laparoscopic guidance.


A 72-year-old man was referred to our cardiovascular center with previous history of hypertension for 5 years and a 2-month history of worsening dyspnea on exertion that motivated him to seek medical attention. Atrial fibrillation was detected during a recent medical check up at a local clinic, and the patient was referred to our hospital and admitted for closer examination. The laboratory data was unremarkable. His electrocardiogram revealed new-onset atrial fibrillation compared with previous data. A chest X-ray film revealed no particular anomalies. Two-dimensional transthoracic echocardiography (2D TTE) showed 1.04×0.87 cm nodular mass attached to the aortic valve. The echogenic mass originated from the ventricular side of the non-coronary cusp of the aortic valve (). Based on these findings, a papillary fibroelastoma of the aortic valve was suspected. Because of his old age and atrial fibrillation, scheduled surgery was performed to avoid systemic embolization. After aortic cross clamp, the aorta was opened. Inspection of the aortic root revealed a normal structure. A mass was found on the ventriclular side of the non-coronary cusp. The mass was successfully excised from the aortic valve (). The tumor measured 1.5 cm and had a smooth surface with a spherical shape. The aortic valve was repaired rather than replaced after tumor resection. Intra-operatory transesophageal echocardiography showed normal left ventricular outflow without regurgitation and no residual mass.
Histopathological examination of the tumor revealed a benign soft tissue tumor mass. The margin of the mass was irregular but was easily distinguishable from the normal valve tissue. The tumor consisted of plump gray purplish myxoid stroma and slender and long spindle stromal cells. The stromal cells had eosinophilic scanty cytoplasms and dark small nuclei. Fibrocollagenous bands of aortic valve tissue were also noted in the myxoid stroma (Hematoxylin and eosin; ×200) (). The final pathologic diagnosis was a myxoma of the aortic valve. The patient's postoperative convalescence was uncomplicated and he has discharged on aspirin with regularly scheduled follow-up. The post operative echocardiogram showed normal aortic valve function. No residual tumor was evident on the postoperative TTE 7 months after surgery.


A 35 year old female patient reported with a complaint of swelling on the lower left jaw since past 2 months []. The swelling was gradually increasing in size. It was present on the body of mandible and extended along the inferior border of the mandible up to the submandibular region. It was bony hard in consistency, and tender along the mental nerve region. Intra-orally, the swelling was present in the buccal vestibule and was extending from 33 to 37 and approximately measured 3 cm × 1 cm. The overlying mucosa was normal in appearance. 35, 36 and 37 were mobile and tender on percussion. Patient also had a history of pus discharge from left ear, which resolved spontaneously. Lateral oblique radiograph revealed a well-defined radio-lucency of size approximately 6.5 × 3cm []. Computed tomography (CT) scan showed expansion of the buccal and lingual cortical plates of the mandible []. Provisionally, the lesion was diagnosed as fibro-osseous lesion/ameloblastoma. Surgical enucleation with curettage of the lesion was done [Figures , ]. Histopathological picture had shown highly cellular fields with some calcified areas. Cellular component was composed of fibroblasts arranged in different patterns. Calcified areas appeared to be composed of cementum like material [Figures , ]. The lesion was diagnosed as a cemento-ossifying fibroma. Patient was reviewed after 2 months of surgery, and the follow up revaled that the size of the swelling had decreased and healing had taken place.


A 59-yr-old man was admitted to our hospital because he had exhibited progressive lethargy and confusion since the early morning. He had often complained of fatigue over the previous 3 months. Three days prior to presentation, he had eaten a large amount of dog meat at a party, become nauseated, and vomited. The next day, he had eaten chicken and freshwater snails, had again become nauseated, and developed severe vomiting. On the day of admission, he failed to arise at his normal time and exhibited inappropriate behavior and drowsiness.
During his childhood, the patient had suffered recurrent abdominal pain and periodic episodes of convulsions, but he had not experienced any seizures in adulthood. On examination, the patient was in a semi-coma; his Glasgow Coma Scale score was 10/15, and he was disoriented in time, place, and person. His vital signs were stable, with a blood pressure of 150/80 mmHg, pulse rate of 84/min, respiratory rate of 24/min, and body temperature of 36℃. Laboratory investigations showed that he had hyperammonemia (143.8 mM), elevated liver enzymes (alanine aminotransferase, 179 U/L; aspartate aminotransferase, 91 U/L), a total bilirubin level of 1.91 mg/dL, and a blood glucose level of 106 mg/dL. His blood urea nitrogen and serum creatinine levels were 17.7 mg/dL and 1.01 mg/dL, respectively. Blood analysis revealed mild leukocytosis (10.41 × 103 leukocytes/µL) and a normal hemoglobin level, platelet count, and clotting profile. Serum electrolyte analysis showed mild hypernatremia (149 mEq sodium/L), but serum potassium and chloride levels were within normal range. Results of a toxicology screen were normal.
Further investigations revealed no evidence of gastrointestinal bleeding, and computed tomography (CT) and diffusion-weighted magnetic resonance imaging revealed no brain lesions. Abdominal CT imaging did not show any abnormality. The cerebrospinal fluid was normal upon examination.
Although hyperammonemia was suspected as the cause of the patient's mental changes, there was no evidence of chronic liver disease. Despite the administration of a lactulose enema, the patient's serum ammonia level increased to 370 mM and he remained confused, leading us to initiate acute hemodialysis. During the procedure, the patient had a generalized tonic-clonic seizure, for which 1 mg of lorazepam was administered intravenously. The seizure subsided, but the patient continued to move convulsively, leading to several additional injections of intravenous lorazepam. A diagnosis of nonconvulsive status epilepticus was made, and the antiepileptic drug levetiracetam was administered.
After the initial session of hemodialysis, the patient's serum ammonia level had decreased to 170 mM, but it soon rose to 228 mM. Plasma and urine amino acid analysis and urine organic acid quantitation were performed. Under suspicion of a urea cycle disorder, arginine (3 g) and sodium benzoate (3 g) were administered via nasogastric tube every 4-6 hr. Dextrose solution (10%) was supplied intravenously, and a protein-free formula was supplied via a feeding tube.
By the morning of the next day, the patient's serum ammonia level had decreased to 36 mM. However, because it rose to 107 mM by the afternoon, and the patient was still semi-comatose, hemodialysis was performed one more time. At that time, an electroencephalogram did not show any signs of epileptic discharge.
After the second hemodialysis session, the patient's serum ammonia level stabilized at less than 30 mM. A plasma amino acids analysis revealed elevated ornithine (196 µM; normal 19-81 µM), decreased citrulline (3 µM; normal 19-62 µM), and elevated glutamine and lysine. Urine organic acids analysis revealed highly elevated urinary orotic acid (603.5 mg/mg creatinine) and a mild urinary uracil peak. No liver biopsy or genetic analysis was performed.
The accumulated evidence led to a diagnosis of late-onset ornithine carbamoyltransferase deficiency. After 5 days, the patient's mental status returned to normal. He continued to receive sodium benzoate (3 g) and arginine (3 g) three times daily. The protein-free formula, which was administered continuously, was gradually changed to increase the dietary protein without a significant rise in serum ammonia levels (). The patient was discharged home after 2 weeks with instructions to continue the medication and formula. His family received special counseling on diet and emergency management before his discharge.


A 60-year-old male presented with complaints of on and off fever and lump in left upper abdomen of 1 month duration. Physical examination revealed pallor. Abdomen was soft, with no venous engorgement. There was a 10 × 7 cm lump; 5 cm below left costal margin in midclavicular line. The lump was nontender with smooth surface and sharp margins. Bowel sounds were present. Laboratory evaluation showed anemia and leukocytosis; electrolyte and liver function tests were with in normal limits.
General blood picture was suggestive of hemolytic anemia with spherocytes, target cells, and Howell–Jolly bodies. Chest X-ray, X-ray abdomen, and echocardiogram were unremarkable. Upper gastrointestinal endoscopy showed antral gastritis, suggesting segmental portal hypertension. Abdominal sonography and computed tomography (CT) showed splenomegaly with heterogeneous enlargement, subcapsular hematoma with multiple ill-defined nodules in the splenic parenchyma with minimal ascites, and left pleural effusion [].
Suspecting a splenic trauma in a congested enlarged spleen, a laparotomy with splenectomy was performed. On gross examination, spleen was enlarged in size with cut surface showing a circumscribed grey white area with diffuse hemorrhagic areas and multiple ill-defined nodules []. Microscopic examination showed the replacement of whole of the spleen by a vascular neoplasm composed of anastomosing vascular channels lined by atypical endothelial cells. Tumor was composed of solid areas and papillary fronds. These papillary fronds were lined by pleomorphic endothelial cells with pleomorphic hyperchromatic nuclei showing mitotic figures [Figures and ]. Areas of necrosis were also noted. The staining for CD31 was performed, which showed diffuse membranous positivity []. Based on these findings the diagnosis of angiosarcoma was made.


The 31-year-old woman was presented to our emergency room with an episode of resuscitated sudden cardiac arrest. She suddenly collapsed at her office, where, upon arrival, the emergency medical technician documented no pulse, and cardiopulmonary resuscitation was started and continued until the patient reached our hospital. An initial electrocardiogram revealed a normal sinus rhythm with left ventricular hypertrophy and a markedly prolonged QT interval (QTc=635 ms) (). In the intensive care unit, recurrent episodes of Torsades de Pointes developed, which were terminated spontaneously () or by DC shock (). Echocardiographic assessment revealed severe left ventricular hypertrophy with normal systolic function. The patient did have a past history of primary amenorrhea and had been treated for hypertension with hydrochlorothiazide and valsartan. Initial laboratory evaluation revealed severe hypokalemia of 1.6 mmol/L (reference, 3.5-5.5 mmol/L). Plasma renin activity was decreased (0.13 ng/mL/hr; reference, 0.15-2.23 ng/mL/hr), but aldosterone level was elevated (270.1 pg/dL; reference, 29.9-158.8 pg/dL). Plasma dehydroepiandrosterone (0.8 ng/mL; reference, 1.8-12.5 ng/mL) and cortisol (4.93 ng/mL; reference, 70-250 ng/mL) were decreased, while plasma 11-hydroxycorticosterone (76.0 ug/dL; reference, 8.0-30.0 ug/dL) and ACTH (420.5 pg/mL; reference, 7.2-63.3 pg/mL) were markedly elevated. Follicle stimulating hormone (30.9 mIU/mL; reference, 1.3-8.1 mIU/mL) and luteinizing hormone (19.6 mIU/mL; reference, 1.0-5.3 mIU/mL) were elevated; however, testosterone was undetectable.
Abdominal computed tomography showed bilateral hyperplasia of adrenal glands (). Chromosome analysis for karyotype showed a 46, XY normal male karyotype, and genomic polymerase chain reaction direct sequencing for CYP17A1 confirmed the diagnosis of 17α-hydroxylase deficiency. After treatment with parenteral replacement of potassium and dexamethasone concurrent with antihypertensive medications, including captopril (300 mg/day), cilnidipine (20 mg/day), and spironolactone (100 mg/day), no more Torsades de Pointes was noted and QTc was normalized ().


A 65-year-old female came to our outpatient department with multiple fleshy and vesicular skin lesions in the external genitalia of 10 years duration. She complained of oozing of copious clear fluid wetting her clothes, making her a social recluse. There was occasional pain and itching over the lesions.
She had been diagnosed to have squamous cell carcinoma of cervix 15 years ago for which she underwent a total hysterectomy and multiple sessions of radiotherapy. There was no history of tuberculosis or other pelvic infections.
On examination, multiple vesicles were seen over labia majora and mons pubis with few lesions exhibiting a verrucous appearance []. Labia majora was edematous and thickened. There was no tenderness on palpation. Inguinal lymph nodes were not enlarged. H and E section of the skin biopsy showed hyperkeratotic, acanthotic epidermis, with multiple dilated lymphatics in the superficial dermis that was consistent with a diagnosis of lymphangioma []. The patient was subjected to a total vulvectomy with double V-Y advancement flap for the reconstruction of labia majora. She is under regular follow up for the past 5 years and is recurrence free till date [].


A 44-year-old lady presented with multiple vesicles over her genitalia for the duration of 16 years. The lesions oozed out clear fluid. She had undergone a hysterectomy 17 years back for carcinoma of cervix that was preceded and followed by ten sittings of radiotherapy.
On examination, there were multiple grouped vesicles over the labia majora with discharge of clear fluid []. There was no inguinal lymphadenopathy. All the baseline investigations including ultrasound of the abdomen were normal. Skin biopsy showed hyperkeratotis, irregular acanthosis, and papillomatosis of the epidermis, with the papillary dermis showing many dilated lymphatics filled with pale, vacuolated secretions []. The patient underwent excision with double V-Y advancement flap cover and is on follow up.


A 24-year-old unmarried female patient presented to us with a persistent fistula in the right iliac fossa following an appendicectomy done in another hospital 6 weeks previously. On further questioning, she informed us that she had presented to this hospital with a 2-week history of abdominal pain, confined to the right lower abdomen. She complained of general ill health and occasional altered bowel habits for 6 months prior to this acute episode.
An enquiry with the doctors who had initially seen her revealed that at the time of presentation for acute appendicitis she was quite unwell with fever, tachycardia, and dehydration. Her abdomen was tender all over with guarding, more pronounced over the right lower half. Her white cell count was 24 × 109/L. An X-ray of her abdomen showed a few fluid levels and an ultrasound scan revealed free fluid in the abdomen with an associated right iliac fossa mass.
A diagnosis of pelvic peritonitis secondary to acute appendicitis was made by the physicians, and she was taken up for surgery after adequate resuscitation. The abdomen was approached through a right McBurney's incision. There was pus in the abdomen with an appendicular mass, and the appendix appeared oedematous, thickened, and congested. An appendicectomy was carried out followed by a thorough irrigation of the abdominal cavity. The abdomen was closed after placing a drain, which was removed on the 3rd post-operative day. In spite of antibiotic administration, she developed a wound infection and persistent pyrexia. She was managed conservatively with antibiotics and anti-inflammatory medication and eventually improved, except for a persistent purulent discharge from the wound. She then decided to seek our opinion and was admitted under our care for further investigation and management.
The appendicular histopathology was retrieved from the previous hospital and revealed transmural inflammation with granulomas suggestive of Crohn's disease. A computerized tomography (CT) scan of the abdomen carried out in our hospital showed pericaecal collection with an inflammatory mass in front of the caecum ().
The abdomen was surgically explored through the previous incision after excising the fistula leading into the caecum. An inflammatory mass associated with the caecum was noted. The appendicular stump had not healed, and was draining into a cavity which was communicating with the wound, indicating a complex enterocutaneous fistula. A limited right hemicolectomy was carried out and continuity established with a primary anastomosis of macroscopically-appearing healthy bowel of the ascending colon and terminal ileum. A specimen of the resected caecum revealed the cobblestone appearance of the mucosa, strongly suggesting the possibility of underlying caecal Crohn's disease (). The histology was reported as inflammatory bowel disease (IBD) consistent with Crohn's disease (). The patient's post-operative recovery was uneventful. She was referred to a gastroenterologist and was being treated with mesasalazine and a regular annual colonoscopy. When last seen at two years post-surgery she continued to remain in good health.


A 75-year-old man with no medical history presented with a mass located below his right retroareolar region of 2 months duration. There was no reported history of family breast cancer. On physical examination, bilateral gynecomastia was observed and the patient had a mobile, well-circumscribed, round mass in the right retroareolar region. It was 4 cm in diameter. There was evidence of clinical right axillary lymphadenopathy. The rest of the physical examination was normal; particularly, there were no abnormal findings in the left breast. Ultrasonography breast showed a lobulated and hypo-echoic lesion with well-defined margins on the right retroareolar region ().
Preoperative examination, consisting of thorax X-ray, ultrasound of the abdomen, and isotope bone scan was within normal limits.Excisional biopsy and frozen section analysis confirmed the malignant nature of the mass. A right radical mastectomy, including right axillary lymph node dissection was performed. Macroscopic examination showed a well circumscribed mass with friable consistency and mucoid texture. Microscopically, the diagnosis was based on the presence of tumor cells which arranged in nest, tubular and solid patterns and floated in abundant extracellular mucin (). The neoplastic cells expressed progesterone but not estrogen receptor, but they did not express HER 2/neu. Axillary lymph node metastasis was noted within two nodes (). The diagnosis of pure mucinous carcinoma with lymph node metastasis was performed. After surgery, the patient received chemotherapy and radiotherapy; he also received hormonotherapy (Tamoxifen). The patient is still free of recurrence 36 months after surgery.


A 12-year-old female patient reported to the Dental Care and Implant Center, Wardha, India, with the complaint of missing maxillary anterior tooth []. The child was physically healthy and had no history of dental and medical trauma. Examination of oral cavity revealed a healthy dental status with permanent dentition. The intraoral radiographs revealed that the permanent central incisor was vertically placed with dilacerated root []. Radiograph showed the presence of sufficient space between maxillary right central incisor and maxillary left lateral incisor.
A combined surgical-orthodontic approach was used to align the dilacerated incisor. Surgical exposure of the crown of the impacted incisor provided access for coronal bracket attachment []. The orthodontic treatment was provided in two phases. The first phase of orthodontic treatment involved an upper removable appliance with elastic traction to the incisor []. Following three months of traction of the tooth, it showed developing cross bite and distal angulation []. This developing malocclusion was corrected by placing inclined plane on the lower incisors. Simultaneously, bracket was placed on maxillary right central incisor along with elastic chain for correction of rotation []. This phase was completed in 10 months.
The second phase of orthodontic treatment was with an upper fixed appliance to correct the alignment []. There was no complication related to the traction and alignment of the tooth. The treatment in this phase completed in 2 months. The results after completion of the treatment were satisfying, with the tooth in correct esthetic and functional position with normal gingival contour and healthy periodontium [].
The patient was recalled for check-up every 6 months and remained symptom-free 2 years after treatment [].


A 55-year-old male presented with left-sided pleuritic chest pain for 2 months and progressively increasing shortness of breath with dry cough for 1.5 months. Chest pain was not relieved by simple analgesic. Shortness of breath was not associated with wheeze. Initially cough was nonproductive; later it became productive with scanty white mucoid expectoration, but there was no history of hemoptysis. He smoked 25 bidis daily for last 32 years, i.e., he was exposed to 8 pack year smoking. But there was no history of past exposure to asbestos. On general survey, mild pallor was present, but there were no clubbing and palpable cervical and axillary lymph node. His respiratory rate was 24 breaths/min, pulse rate 108 beats/min, and blood pressure 120/80 mm Hg.
Examination of respiratory system revealed decreased movement of the left side of the chest wall with ipsilateral fullness. Trachea was shifted to right. Vocal fremitus was diminished and percussion note was dull over all areas of left side. Vesicular breath sound was diminished and vocal resonance was decreased on the left side. Examination of abdomen did not reveal any lymphadenopathy, ascites, and hepatosplenomegaly. Other systems were within normal limit.
Complete hemogram and blood biochemistries were within normal limit. Left-sided homogenous opacity along with contralateral mediastinal shifting was seen on chest X-ray. Sputum for acid fast bacilli (AFB) and malignant cell were negative. A total of 500 mL of pleural fluid was aspirated from the left side and pleural fluid analysis revealed lymphocyte predominant, exudative, hemorrhagic pleural effusion with adenosine deaminase value, 17.5 U/L. Papanicolaou stain of pleural fluid revealed no malignant cell. On contrast enhanced computed tomography (CECT) of thorax a large heterogeneous mass with multiple areas of necrosis, occupying almost whole of left hemithorax was seen along with left-sided pleural effusion []. CT-guided fine needle aspiration cytology (FNAC) revealed spindle cell neoplasm. On histopathological section of CT-guided tru-cut biopsy, it was shown that there were sheets of spindle cells with plump nuclei, moderate degree of nuclear polymorphism, and mitotic Figures in more than one high power field – suggestive of spindle cell sarcoma []. However, immunohistochemistry revealed that tumor cells expressed epithelial membrane antigen, CD99, bcl-2 and calponin and were immunonegative for cytokeratin []. Hence, final impression from immunohistochemistry was primary synovial sarcoma of lung.


A 50-year-old post-menopausal woman was seen by a dentist for the pain in the left side of the upper lip. After thorough examination of the patient, the dentist diagnosed it as chronic periodontitis and pulpitis of the left upper second incisor. The dentist advised her oral hygiene and extraction at a later date. She was advised to take oral paracetamol 500 mg thrice daily for 3 days. Two weeks after this episode of visiting a dental clinic, the patient developed swelling of the upper lip [] with mild puffiness of the face. The patient was also suffering from osteoarthritis of both knees and used to take diclofenac sodium 50 mg twice daily or Ibuprofen 400 mg thrice daily frequently and irregularly by her own by purchasing over the counter (OTC). When she visited the same dentist again for consultation, it was diagnosed as angioedema of the upper lip, an allergic phenomenon[] due to nonsteroidal anti-inflammatory drugs (NSAIDs), and advised to consult a physician for further management.
When the patient was referred to the physician for the management of angioedema of the upper lip with the history of taking NSAIDs for arthalgia, she was advised to stop taking NSAIDs and was prescribed corticosteroids (methyl prednisolone) 10 mg thrice daily along with Ranitidine 150 mg twice daily. Then, the physician tapered the dose of steroids in 10 days in two clinical visits. When reviewed again after 15 days, she was asymptomatic and the edema of the upper lip subsided completely.
After a week she developed the similar swelling of the upper lip again []. This time there was no history of taking NSAIDs. She was again referred to the physician for review and found no obvious focus of infection. She was prescribed oral antibiotic amoxicillin 500 mg thrice daily anticipating subclinical soft tissue infection around the mouth and nose.[]
The patient was subjected to laboratory investigations[] to rule out soft tissue infections around the mouth (hemogram, blood sugar, and nasal smears). The C1 esterase inhibitor estimation to rule out angioedema was deferred by the patient due to its high cost. Since other routine investigation results were within the normal limits without any obvious improvement in her clinical conditions, the antibiotics were stopped and referred to another dentist for the second opinion.
The dentist reviewed and raised the suspicion of the periapical abscess and ordered for X-ray of upper incisors on the left side. The X-ray revealed the radiological features of periapical abscesses of the upper incisor tooth [] The patient was treated by immediate tooth extraction and exudate in the abscess cavity was drained.[] The patient was given paracetamol 500 mg thrice daily as an analgesic.[] The edema subsided, the patient's condition was restored clinically normal [Figures and ] and was able to do her routine work. She was reviewed periodically in dental clinic. When the clinical condition favored, the artificial tooth was fixed to fill the gap.


A 70-year-old female presented with complaints of painful swelling of the right side of the face and the presence of wormy creatures in the lids and orbit on the right side, of 4 days duration. Her sleep was disturbed due to their constant movement.
She had undergone bilateral cataract surgeries elsewhere 2 years ago and the right eye had been removed 8 months ago. No documentary details were available. The patient reported that she had sustained accidental injury to the right upper lid, about 10 days earlier. She did not take any treatment for the same. Enquiry revealed that the patient was from a rural background and had close contact with goats.
Examination revealed an elderly, unkempt lady with right-sided facial cellulitis. There were large, maggot infested ulcerations involving the medial parts of the right upper and lower lids as well as the ipsilateral lacrimal sac area. The right orbit was anophthalmic, foul smelling, and ridden with numerous maggots, some of which were present in multiple deep pockets of the lids and orbital tissue []. Ophthalmic examination of the left side revealed no abnormality other than pseudophakia. The presence of severe scalp pediculosis was noticed and a dermatologist's consultation was taken. ENT examination was normal.
Immediate treatment consisted of injection Tetanus Toxoid, oral diclofenac, and instillation of 0.3% gatifloxacin eye drops, four times a day in the right orbit. Laboratory investigations and X-ray orbits were normal. Manual removal of maggots was done several times a day, initially following turpentine wash, and later under topical anesthesia []. However, several maggots continued to remain deeply buried in pockets in the lids and orbit. Magnetic resonance imaging (MRI) suggested a suspicion of early erosion of the medial orbital wall on the right side. The retro-orbital compartment including the optic canal was normal [Figs. and ].
The patient was administered a single oral dose of 12 mg of ivermectin, following which she had a dramatic relief from pain. 1% ivermectin drops prepared by dissolving the tablet in distilled water was additionally instilled four times a day in the right orbit for a week. Dead maggots were easily removed and rapid wound healing ensued. The patient was discharged with a healthy socket [Figs. and ] and a louse-free head. At final count, over a hundred maggots were removed, varying from 10 to 15 mm in size. The parent fly could not be identified.


A 20-year-old unmarried woman from middle socioeconomic status with urban bac kground presented to the Department of Psychiatry. She was a known case of SLE with predominant skin manifestations since 5 years, and she was treated by a dermatologist. The patient discontinued medication after 2 years on her own, as there was an improvement of symptoms after attaining the menarche. Again, for 3 months the patient noticed frequent hair fall, itching over the scalp, and intermittent low-grade fever. The patient also had continuous withdrawn/dull behavior, suspiciousness, referential ideas, hearing of voices, decreased self-care, and occasional crying episodes in the last 15 days. The dermatologist started 30 mg/day of prednisolone (tablet) after the onset of psychiatric symptoms. On physical examination, the patient was found to be pale. There was dark pigmentation all over her body and sparse and thin hair over her scalp, but there were no neurological deficits or signs of meningeal irritation. On mental status examination, the patient was found to be poorly kempt, restless, irritable, had poverty of speech, third person auditory hallucination, delusion of persecution, delusion of reference, and blunt affect but she was conscious and oriented. Baseline biochemical and hematological investigations were within normal limits; antinuclear antibody (ANA) test was positive (index ratio was 3.5 on ANA detect); and rheumatoid antibody test was negative. The patient was diagnosed to have organic delusional (schizophrenia-like) disorder (International classification of diseases (ICD)-F06.2)[] secondary to SLE. Eventually the patient was started on 10mg/day of olanzepine (tablet). A significant improvement was observed in 2 weeks, and the patient was discharged. She remained free from psychiatric symptoms for the last 2 months.


A 17-year-old unmarried woman from rural background with lower socioeconomic status reported to the general medicine department. She had a history of 2 months of fever and joint pain and a history of 5 days of cough. She was diagnosed to have cutaneous lupus erythematosus for a year, and she was under 20 mg/ day of prednisolone (tablet) in divided doses. The patient was admitted and evaluated. General physical examination revealed pallor, icterus, hyperpigmentation all over her body, including face, erythematosus skin lesions, diffuse hair loss, depigmented patches over the scalp, and erosions in the buccal mucosa. Chest radiograph was suggestive of miliary tuberculosis, and an electromyographic study was suggestive of mixed neurogenic and myogenic pathology. Most of the baseline biochemical and hematological investigations (except Hb% 8 g% and total bilirubin 4 mg/dl) were within normal limits. Immunological investigations showed positive anti-UI/Sm ribonucleoprotein antibody (RNPAb), anti-Sm Ab, anti-ds deoxyribonucleic acid (DNA) Ab, anti-histone Ab, anti-nucleosomal Ab, and anti-ribosomal Ab. The human immunodeficiency virus (HIV) test was nonreactive. After the review by the immunologist, the patient was diagnosed to have SLE with myositis with military tuberculosis. She was treated with 200 mg of ciprofloxacin bid (injection), 100 mg of amikacin iv tid (injection), 100 mg of hydrocortisone iv bid (injection), 1 g of ceftriaxone iv od (injection), and 400 mg/day of ethambutol (tablet). The patient was free from psychiatric complaints for initial 1.5 months. For 15 days, the patient presented with muttering, smiles to self, occasional crying spells, irrelevant speech, anger outbursts, and decreased sleep. There was no alteration in medication, changes in dosages, or onset of any fresh physical complaints in these 15 days. There was no history of either SLE or major psychiatric illness in family. There was no history of psychosis, depression, or mania, but she was premorbidly well adjusted. Mental status examination revealed poorly kempt, irrelevant speech, second person auditory hallucination, reduced range of mood, and absence of insight. The patient was diagnosed as organic delusional [schizophrenia-like] disorder (ICD-F06.2).[] The patient was started on 10 mg/day of olanzepine (tablet) and increased to 20 mg/day. The patient showed 50% improvement in the first week, and she was discharged later.


A 35-year-old woman was diagnosed with SLE since 2 years, and she was admitted in dermatology department for acute cutaneous exacerbation of SLE. She was on 20 mg/day of predinisolone (tablet) and local skin applications for skin lesions. The immunological test revealed ANA positivity. Other baseline biochemical and hematological investigations were within normal limits. The patient was referred to psychiatric evaluation for the complaints of fearfulness and decreased sleep for 2 days. There were no significant family history, history of major psychiatric illness, and no similar or significant psychiatric illness. Mental status examination revealed that the patient had delusion of persecution, delusion of reference, irritability, and absence of insight. There was no diurnal variation or symptoms suggestive of delirium. There was no change in medication or doses prior to onset of psychiatric symptoms. The patient was diagnosed as organic delusional (schizophrenia-like) disorder (ICD-F06.2)[] and started on 10 mg/ day of olanzepine (tablet). The patient remitted from psychiatric symptoms in 1 week, and she was symptom-free even after follow-up of 2 months.


A 48-year-old man presented with acute obstructive cholangitis. He had previously been admitted several times for alcohol-induced chronic pancreatitis with acute exacerbation. His laboratory test revealed white blood cell count of 12,250/mm3, aspartate aminotransferase level of 83 U/L (normal, 0 to 40 U/L), alanine aminotransferase level of 160 U/L (normal, 0 to 40 U/L), alkaline phosphatase level of 919 U/L (normal, 40 to 250 U/L), total bilirubin level of 4.19 mg/dL (normal, 0.2 to 1.2 mg/dL), direct bilirubin level of 3.52 mg/dL (normal, 0.05 to 0.5 mg/dL), amylase level of 182 U/L (normal, 20 to 125 U/L) and lipase level of 127 U/L (normal, 13 to 60 U/L). Abdominal computed tomographic (CT) scanning with the reconstructed image revealed distal biliary obstruction that was caused by a pancreatic stone in the pancreatic head, and there was also pancreatic ductal dilatation and parenchymal atrophy of the pancreatic body and tail with multiple calcifications (). The diameter of the stone in the pancreatic head was measured to be about 1 cm. Compared with the previous CT taken 3 years ago (), the multiple pancreatic calcifications were further increased. Emergency duodenoscopy revealed swelling of the papilla and an impacted pancreatic stone in the papilla (). Precut papillotomy using a needle knife (Huibregtse; Cook Medical, Winston-Salem, NC, USA) was performed without preceding biliary cannulation. The precut incision was extended to the roof of the papilla in a 12 o'clock direction, and a whitish-yellow stone was exposed. The pancreatic stone was successfully removed using grasping forceps (Rat Tooth; Olympus, Tokyo, Japan). After additional sphincterotomy with a pull-type papillotome (Tri-Tome PC; Cook Medical), a large amount of dark-greenish bile juice gushed out. The rest of the stones in the pancreatic duct were not removed. The patient has rapidly improved after the endoscopic therapy and he has remained well since then.


A 75-year-old man was referred to Chungbuk National University Hospital owing to urinary frequency. He had a history of transurethral resection of the bladder for invasive urothelial carcinoma 8 years previously. The tumor was of a low grade and invaded the subepithelial connective tissue. However, proper muscle involvement was not evaluable because the specimen had no proper muscle component. The patient had been treated with Bacillus Calmette-Guerin (BCG) and had lived without recurrence. The patient underwent a transurethral biopsy under the clinical impression of recurrent urothelial cancer. There was widespread urothelial carcinoma in situ; therefore, radical cystoprostatectomy with orthotopic bladder substitution (Ghoneim) was carried out. Grossly, the mucosal surface of the urinary bladder showed multifocal flat erythematous lesions, and there were no remarkable lesions in the remainder. The microscopic findings showed multiple urothelial carcinoma in situ lesions throughout the mucosa of the urinary bladder and both ureters (). The left seminal vesicle was covered by large polygonal tumor cells, which were confined to the mucosa in single to several layers (). The tumor cells had large and hyperchromatic nuclei with distinct nuclear membranes and relatively abundant eosinophilic cytoplasm. In addition, bizarre-shaped tumor cells showing irregular hyperchromatic or smudged nuclei were occasionally identified. The tumor cells mostly involved the mucosa between the epithelial cells and the basal lamina of the seminal vesicle, a feature that is referred to as pagetoid spread. In some areas, the entire thickness of the mucosa was replaced by tumor cells. The mucosa of the ejaculatory duct and adjacent prostatic acini and duct were also scattered with tumor cells (). On the basis of the overall findings, we considered that the urothelial carcinoma in situ of the urinary bladder revealed mucosal spread to the seminal vesicle along the ejaculatory duct. In addition, there was an incidental prostatic adenocarcinoma, which was a small, solitary lesion with a Gleason score of 6. At the time of the surgery, the patient's serum prostate-specific antigen level was 0.87 ng/ml. The patient had no evidence of disease for 5 months after surgery.


A 45 year old diabetic male patient who has been taking oral antidiabetic medications for 12 years and is followed because of schizophrenia was admitted to the resuscitation department because of septic shock. On examination, the patient was obnubilated, the temperature was 39.5C° with tachycardia and a general abdominal tenderness. The laboratory test showed a hyperleucocytosis (23000), glycaemia (44 mmol/l), acetonuria (+), glycosuria (+++), and creatineamia (300 μmol/l). Abdominal echography revealed a slack alithiasic gallbladder with a 5 mm thick laminated wall, a hydatid cyst of the VII segment, 6 cm in diameter and non-complicated type I. The diagnosis of a complicated alithiasic cholecystis due to the septic shock was retained, and the patient was operated upon. A swollen gall bladder and an enlarged liver were found. The hydatid cyst was of a non-complicated type I. Cholecystectomy was performed and followed by resection of the cyst covering dome.
In spite of the post-operative antibiotic therapy (Cefotaxime and Gentamicin), the patient remained subfebrile and developed an excessive tenderness in the hypogastrium area and retention of urine. When examining the genitals, crepitants at the base of his penis was found. A CT scan delineated a distended bladder with circumferential intramural air and intravesical air-fluid level ().
A vesical probe was inserted and the urine obtained was turbid and haematic. We also noted pneumaturia, and the urine cystobiological tests revealed klebsilla pneumonia. The haemaculturs were negative. The patient was treated with imipenem and metronidazole, and had urine drained via a vesical probe. His diabetes was stabilized and the condition was improved. A CT scan performed after 15 day of antibiotic therapy showed a normal bladder and free of the emphysematous cystitis (). The vesicle probe was removed after 21 days. After a decline in 18 months, the patient presented well without recurrence.


A 65-year-old woman underwent surgery for gastric cancer four years ago and her passage was restored with jejunal interposition. After 42 months following this surgical procedure, she complained of dysphagia. Endoscopic examination revealed a stenosis in the anastomosis, in which a recurrent cancer was assumed; and so a silicon-coated wall stent (25-mm diameter, 10-cm length) was inserted.
Four weeks after stent application, she was admitted to our emergency department with a 48-hour history of abdominal pain, nausea and vomiting. Examination of the abdomen showed marked distension with peritonitis. An irregular mass was detected in the right lower quadrant. Her body temperature was 37.5°C. Laboratory findings showed a white blood cell count of 15600/mm3, hemoglobin 12.3 gm/dl, and a platelet count of 200.000/mm3. All other studies, including electrolytes and urinalysis were within normal limits. An abdominal plain roentgenogram showed air-fluid levels present in the small intestine. A computed tomography scan was ordered, and marked dilatation of the small intestine and the displaced stent in the right iliac fossa were observed (). Fluid resuscitation and gastric decompression via nasogastric tube was started immediately. After establishment of the diagnosis as mechanical bowel obstruction, the patient was taken to the operating room for emergency laparotomy.
During laparotomy, a 40cm segment of edematous, thick-walled terminal ileum was noted. The stent could be palpated through the thickened bowel wall. The small intestine segment was incised longitudinally 2-3 cm and the migrated stent was removed (). The patient recovered without incident and was discharged on postoperative day 3.


A 58 year-old North African man presented with a macroscopic hematuria of two weeks’ duration. Cystoscopy showed a 4 × 4 cm sessile mass in the bladder. Pathologic examination of the transurethral bladder resection was consistent with the diagnostic of poorly differentiated carcinoma infiltrating the muscle of the bladder and a radical cysto-prostatectomy was performed.
Grossly, there was a well demarcated tumour in the dome that measured 2 × 3 × 1 cm. Microscopic examination showed an undifferentiated carcinoma with a lymphoid stroma. Tumour cells were characterized by a syncytial growth pattern in a dense lymphoid stroma (Fig. , ).
Immunohistochemically, most of the tumour cells were positive for Epithelial Membrane Antigen (EMA) and cytokeratin. The surrounding cellular infiltrate was a mixture of B and T lymphocytes. Hybridization to Epsteïn – Barr Virus encoded RNA was negative. The patient remained alive and disease free eight months after the diagnosis ().


A 48-year-old woman 155 cm tall weighing 30 kg, with cystic fibrosis had surgery planned for single lung transplantation. Twenty-years earlier, she was diagnosed with cystic fibrosis. She needed right lung transplantation because of progressive dyspnea and recurrent respiratory infection after left pneumonectomy, which was due to a huge aspergilloma in the upper lobe of the left lung 5 years before her visit to our hospital. There was nothing particular about her case except for her lung disease and her vital signs and laboratory workup were normal before surgery. In addition, arterial blood gas analysis (pH 7.35, PaCO2 51.8 mmHg, PaO2 74.6 mmHg, HCO3- 28 mEq/L, BE 1.3 mEq/L, SaO2 94.2%) showed mild respiratory acidosis. Results of the echocardiography before surgery showed a left ventricular ejection fraction of 69%, an enlargement of the right atrium and ventricle, a mild tricuspid regurgitation, severe pulmonary hypertension, and minimal pericardial effusion. The donor was a patient with traumatic SAH and SDH and was fit for donating a lung based of their medical history and laboratory workup.
The cystic fibrosis patient had an enlargement of the right thorax and a left mediastinal shift (organs such as trachea, heart, aorta, vena cava etc.) as a result of the past left pneumonectomy, and it caused an abnormal positioning, twisting, and narrowing of the subclavian vein and internal jugular vein; therefore, a 9 Fr. central vein catheter (advanced venous access, AVA high flow device, Edwards LifeSciences, USA) was inserted into the internal jugular vein under angiography in the angiography room because of difficulty in performing the catheter insertion and due to the risk of vascular perforation ().
The patient arrived at the operating room and was administered oxygen 5 L/min by nasal cannula. Additionally, noninvasive BP monitoring, electrocardiography, and pulse oxymetry was immediately done after arriving at the operating room, and a BIS monitor was attached and a monitor was connected to the right radial artery catheter and a femoral artery catheter. Before putting the patient under anesthesia, her measured vital signs were a BP of 115/78 mmHg, a HR of 85 beats/min, and a SaO2 of 95%. To induce anesthesia, 6 mg of etomidate and 5 mg of vecuronium were injected and an internal diameter 7.0mm tracheal tube was inserted after manual ventilation with 100% oxygen. The ventilator was set to a FiO2 of 0.5, a PEEP of 5 cmH2O, a RR of 10 breaths/min and a TV of 350 ml using the volume-controlled mode. After that, through the center vein catheter, which was inserted into the right internal jugular vein, a 7 Fr. pulmonary artery catheter (Swan-Ganz CCOmbo catheter, Edwards LifeSciences, USA) was inserted into the central pulmonary artery, and at that time, the pulmonary artery pressure was 61/26 mmHg. To maintain anesthesia, 3.5 µg/ml of propofol and 3.0 ng/ml of remifentanil with target-controlled infusion were consistently injected and the injection speed of these anesthetics was controlled according to the change in vital signs, and cell saver was used during the surgery.
After positioning the patient in the supine position with both arms elevated, the surgery started with a clamshell incision. First of all, before the pneumonectomy, by inserting a catheter into the aorta and the right atrium, cardiopulmonary bypass was done during surgery to not only prevent unstable vital signs due to severe pulmonary hypertension and profuse bleeding caused by severe adhesion of the right lung as a result of recurring infection but also to oxygenize the blood after the pneumonectomy. Before the cardiopulmonary bypass was started, 10,000 units of heparin were injected, and 3 minutes later, the activated coagulation time was 450-seconds. After starting the cardiopulmonary bypass, the ventilator was taken off and 1,000 mg of methylprednisolone were injected after the pneumonectomy. Thereafter, immediately, the bronchus, pulmonary artery, and vein of the transplanted lung were anastomosed to the patient. At some point, blood pressure dropped 80/45 mmHg because of bleeding or pressing of the intramediastinal structure, but it was relatively stable maintaining a blood pressure of more than 100/50 mmHg and SaO2 of 100%. The ischemic time of the right lung was 75-minutes.
Anastomosis of each organ was done well and reperfusion was attempted after pulmonary vein anastomosis. To reduce increased pulmonary artery pressure and prevent reperfusion injury, a weaning off of the cardiopulmonary bypass was attempted after setting the FiO2 to 0.4, the PEEP to 5 cmH2O, the RR to 14 breaths/min, the TV to 300 ml, and the nitric oxide (NO) to 20 ppm by the volume-controlled mode connected to a prepared NO ventilator. The results of the arterial blood gas analyses after connecting the NO ventilator were stable showing a pH of 7.48, a PaCO2 of 41 mmHg, a PaO2 of 449 mmHg, a HCO3- of 30.5 mEq/L, a BE of 6.4 mEq/L, and a SaO2 of 100%. The results of the arterial blood gas analyses after weaning off of the cardiopulmonary bypass was a pH of 7.42, a PaCO2 of 47 mmHg, a PaO2 of 89 mmHg, a HCO3- of 30.5 mEq/L, a BE of 5.2 mEq/L, and a SaO2 of 97%; therefore, a FiO2 of 1.0 was maintained. presents the results of the arterial blood gas analyses during the surgery.
The blood pressure decreased to 75/40 mmHg during the process of reducing the flow to gradually wean the patient off the cardiopulmonary bypass; thus, fluids were injected into her body, and 10 mcg/kg/min and 0.02 mcg/kg/min of dopamine and epinephrine, respectively, were consistently injected. After that, the surgery was completed with no complications and the patient was transferred to the SICU with manual ventilation. Total anesthesia time was 6 hours 45 minutes; the operation lasted 4 hours; cardiopulmonary bypass time was 2 hours 25 minutes. The volume of total bleeding was 3,000 ml; the amount of total fluid infused was 500 ml of crystralloid solution and 1,100 ml of colloid solution. Total transfusion was 10 units of irradiated WBC-depleted RBC, 7 units of FFP, 10 units of PC. Urine output was 1,600 ml.
2 days after the surgery, the patient received a tracheostomy to take her off the ventilator (). Six days after surgery, she was transported to the general ward and seemed to get better using a home ventilator. However, 42 days later, she died of inhalation pneumonia.


A 28-year-old male suffered from a traffic accident while in a car. His mental status was confused but brain CT revealed non-specific findings. He had no known history of hypertension, diabetes, hepatitis, and pulmonary tuberculosis. He was an 8 pack-year current smoker. His family history was non-specific. On admission his vital signs were stable except for blood pressure of 100/70 mm Hg. A 12-lead electrocardiogram (EKG) showed normal sinus rhythm with 72 beat/min. The results of electrolyte panel and kidney function studies were all within normal limits. The aspartate aminotransferase (AST) was 70 U/L, alanine amino-transferase (ALT) was 47 U/L, creatinine kinase (CK) was 255 U/L and CK-MB was 28 U/L. However, serum level of troponin I was within normal limits. Initial 2-dimensional echocardiography (2DE) showed no regional wall motion abnormality with normal left ventricular ejection fraction, but chest CT angiogram (CTA) showed traumatic aortic dissection in proximal descending thoracic aorta/distal aortic arch around aortic isthmus with left pleural effusion (). Therefore, we performed a thoracic aortography, which showed an aortic aneurysm distal to the subclavian artery, for which a 24×112 mm valiant thoracic stent was implanted. Follow-up aortogram showed no evidence of leakage. Follow-up 2DE did not show evidence of turbulent flow or flow disturbance near the entrance of the left subclavian artery. Follow-up chest CTA showed vascular stent graft insertion state from distal aortic arch to proximal descending thoracic aorta and resolved state of previous traumatic aortic dissection (). After uneventful recovery, he was discharged on day 33 and has been followed up at the outpatient clinic.


A 52-year-old male suffered from a traffic accident in a car. He complained of left flank pain and had multiple left rib fracture. He had no known history of hypertension, hepatitis, or pulmonary tuberculosis, but had been on diabetes medication for 3 years. He was a non-smoker and non-alcoholic drinker, and his family history was non-specific. On admission his blood pressure was 70/40 mm Hg. EKG showed sinus tachycardia with 138 beats/min. The results of electrolyte panel and kidney function studies were all within normal limits. The AST was 219 U/L, ALT was 153 U/L, CK was 2113 U/L, CK-MB was 51.7 U/L and troponin I was 0.24 U/L. Initial chest CTA showed traumatic aortic dissection in distal aortic arch/proximal descending thoracic aorta with surrounding hematoma (). The patient underwent medical treatment for 3 weeks, but follow-up chest CTA showed no remarkable change of traumatic aortic dissection and increased amount of bilateral hemothoraces. Based on this information, we performed the thoracic aortography on day 24. Aortogram revealed dissecting aneurysm approximately 1 cm distal from the left subclavian artery ostium. Stenting was performed using a 26×112 mm valiant thoracic stent graft. Follow-up aortogram showed good expansion of the stent without dye leakage. Follow-up chest CTA showed a graft insertion state from the distal aortic arch to the proximal descending thoracic aorta and a resolved state of a previous traumatic aortic dissection (). After uneventful recovery, he was discharged on day 34 and has been followed up at the outpatient clinic.


A 62-year-old man presented with a 3 × 2 cm oval squamous cell carcinoma (SCC) of the left distal leg (Fig. ). As the defect after excision of the tumor was expected to be too big to be closed primarily, and in order to avoid skin grafting, the TopClosure® 3S System was applied in a noninvasive manner for gradual stretching of the skin for 16 days until sufficient skin laxity was achieved to enable surgical resection of the lesion with primary closure (Fig. ). Preoperative skin stretching enabled complete excision of the tumor and primary edge-to-edge wound closure in layers with sutures and staples. Because the skin defect still required closure under relatively high tension, the TopClosure® 3S System was applied over the dressed closed wound and tightened with minimal force in order to secure wound closure by reducing tension on the suture line (Fig. , d). The patient was only confirmed to bed for one postoperative day and could fully ambulate with the attached TopClosure® 3S System during the rest of the preoperative and postoperative periods. Figure shows complete wound closure with a satisfactory aesthetic scar.


A 52-year-old woman with a depressed lower abdominal scar following TRAM breast reconstruction requested revision of the scar (Fig. ). The TopClosure® 3S System was applied noninvasively prior to surgery in order to stretch the skin to avoid excessive tension on the newly formed scar. Two 8-mm TopClosure® 3S Systems were positioned at the edge of the 7 × 8 cm depressed scar (Fig. ). In an attempt to reach a quick approximation, minimal blistering was observed on the underlying skin of the distal ends of the AP (Fig. ). This required removal of the systems only 2 days following application and attachment of another system at an alternative, adjacent site. Skin was now stretched by gradual approximation of the plates from 7 to 0.5 cm over a 5-day period. During surgery, the scar was excised and wound edges were sutured in layers. Gauze was placed over the wound with two 8-mm TopClosure® 3S Systems positioned across the incision edges for an additional 6-day period to support and secure closure, relieving tension from the wound’s edges, thus enhancing the aesthetics of the scar (Fig. ). The blisters at the attachment site were treated topically, resulting in no residual marks. Figure shows complete wound closure with a satisfactory aesthetic scar.


A 26-year-old male patient sustained a fourth-degree high-voltage electric burn with severe soft tissue damage to his left lower limb. Following demarcation of the necrotic tissue at high left leg level, a below-knee amputation was performed. Wound edges were sutured in layers and stapled (Fig. + c). Two 8-mm TopClosure® 3S Systems were attached invasively to the skin by staples, 3 cm from the wound edges as substitute to tension sutures (Fig. , c). Gauze was placed over the wound, and the ASs were then inserted into the L/RMs and lightly tightened to support and secure closure by relieving the tension from the wound edges and distributing the stress away from the scar (Fig. ). The wound was topically treated periodically by temporary removal of the ASs and reapplying them after dressing change. The postoperative period was uneventful and the wound healed well (Fig. ).


A 12-year-old girl presented with a congenital nevus on her right thigh (Fig. ). As excessive tension was expected with direct primary closure, TopClosure® (6 mm) was applied 5 days prior to excision and was gradually stretched daily (Fig. ). Following application of topical anesthesia, the lesion was excised and closed in layers under tension (Fig. ). In order to secure the wound closure and to improve the aesthetics of the scar, TopClosure® was applied for an additional 2 weeks following surgery (Fig. , g), resulting in a narrow aesthetically pleasing scar (Fig. ).


A 29-year-old woman presented with a dehisced, infected, abdominal surgical wound following dilatation and curettage for a missed abortion that was complicated by acute massive retroperitoneal hemorrhage (Fig. ). The wound was treated by combined regulated negative pressure-assisted wound therapy (RNPT) and an 8-mm TopClosure® 3S System that was used to approximate wound edges (Fig. , c). Wound dressings were changed every third day by releasing the AS, cleansing the wound and applying the RNPT again. The TopClosure® 3S System was attached to the skin by staples and could be cleaned together with the wound with every change of dressing (Fig. , e). As the patient refused additional surgery to close the wound, further treatment was provided in the outpatient clinic. The wound was closed by approximation of the wound edges by the TopClosure® 3S System with a final acceptable result (Fig. ).


An 88-year-old woman presented with a relatively large oval SCC (2.5 cm in diameter) of the left distal leg (Fig. ). As the defect after excision of the tumor was expected to be too large to be closed primarily and in order to avoid skin grafting, two 8-mm TopClosure® 3S Systems were applied in a noninvasive manner for gradual stretching of the skin over a period of 12 days until the sufficient skin laxity was achieved to enable surgical resection of the lesion with primary closure (Fig. ). During surgery, the patient underwent cyclical skin stretching using the TopClosure® 3S Systems for 2-min skin stretching followed by 1-min relaxation, for about 30 min. Preoperative and intraoperative skin stretching enabled complete excision of the tumor and primary edge-to-edge wound closure in layers, under tension with sutures and staples (Fig. ). Two TopClosure® 3S Systems were applied over the dressing for two days. The patient was only confined to bed for two postoperative days and could fully ambulate with the attached TopClosure® 3S Systems during the rest of the preoperative and postoperative periods. Figure shows complete wound closure.


A 28-year-old male presented with worsening of right knee pain since 9 months which started after blunt trauma to the knee. Immediately after trauma there was no swelling and the patient was able to walk. He was disabled due to repeated attacks of knee pain. On clinical examination, there was a small infrapatellar cystic swelling in the anterolateral region, which increased during extension of the knee and decreased with flexion of the knee. There was no collateral and cruciate ligament laxity and McMurray's test was negative. There was no joint effusion clinically detected. Range of movements was terminally restricted and was painless in all directions. X-ray and haematological parameters were both normal. Magnetic resonance imaging (MRI) showed well defined lobulated lesion which was of low signal intensity in T1-weighted images and of high signal intensity in T2-weighted images arising from HFP, suggestive of ganglion cyst arising from it that was extending inside the knee joint []. Rest of the knee joint was normal with no meniscal tear or continuity of the lesion with meniscus. Arthroscopic examination using high portal was done [], but it was not possible to resect the lesion through arthroscope; so, open resection and complete excision of the cyst was done. Histopathologic examination confirmed the diagnosis of ganglion cyst. Postoperative period was uneventful and the patient was given physiotherapy for strengthening of quadriceps. Cincinnati knee scoring improved from 300 to 380. At 2 years of followup, the patient is completely asymptomatic with full painless range of movements.


A 36-year-old female presented with long standing history of left knee pain. On clinical examination, there was minimal medial joint tenderness, and there was no palpable swelling or ligament laxity. McMurray's test was suggestive of meniscal injury. Movements were terminally restricted. X-ray was normal. MRI showed altered signal changes grade II in medial meniscus. There was well defined hyperintense in T2 and hypointense in T1, lobulated lesion arising from HFP, indicating a cystic swelling which was extending in the knee joint. There was no continuity with the meniscus of this lesion []. The patient was started on physiotherapy regime as she was not willing for any surgical intervention. With conservative management, she responded well and Cincinnati knee score improved from 280 to 360. At followup of 18 months, the patient has good range of movements and no complaints.


A 12-year-old female child came with intermittent knee pain and swelling below patella since 6 months. She had taken treatment from other physicians, but without any relief. When the child came to us, there was knee synovitis on examination with small cystic swelling present around the inferior pole of patella just lateral to it []. There was no ligament laxity and knee alignment was normal. Preoperative Cincinnati knee score was 320. MRI of the patient revealed well defined swelling arising from HFP, which was hyperintense in T2 but isointense in T1-weighted images, suggestive of hemangioma or low-flow venous malformation (the swelling was extending in the joint and anterior and lateral to patellar tendon). The patient underwent open surgical resection of the swelling. It was a reddish-blue colored, well demarcated swelling arising from HFP encircling patellar tendon extending inside the joint [] which was completely removed. Histopathologic report confirmed it to be hemangioma. Patient was started with knee range of movements and strengthening exercises. At followup of 1 year, the patient is completely asymptomatic with Cincinnati knee score of 390.


A 63-year-old woman, a known case of non-Hodgkin's lymphoma, underwent whole body FDG PET-CT scan for surveillance. She had Stage I disease involving the left axillary lymph nodes at the time of diagnosis and had completed chemotherapy a year ago. She was in remission since then. The patient had complained of lumbago for the past 4 months. The FDG-PET scan (Maximum Intensity Projection- ) showed vertical linear FDG uptake in the medial to bilateral sacroiliac joint and horizontal uptake connecting the vertical lines (H-shaped uptake) in the sacral region. This ‘H’-shaped increased FDG uptake resembled the typical Honda/Butterfly sign, classically described in bone scans in cases of SIF. There was no evidence of abnormal FDG uptake in left axillary region (site of primary disease) or anywhere else in the body. The axial and coronal maximum intensity projection (MIP) CT images [Figure and ] show lucent fracture line (arrow) with breach in the anterior cortex of right sacral ala. The fracture line is away from the right sacral foramina and medial to the sacroiliac joint. There was no history of trauma to sacroiliac region or any history of a fall. We concluded the scan abnormality as SIF.
A correlation with nuclear magnetic resonance (NMR) imaging of sacral spine was performed. Short tau inversion recovery (STIR)-weighted axial and coronal images [Figure and ] through the sacrum revealed complete diffuse hyperintensity of the right sacral ala suggestive of bone marrow bruise/edema. There was also mild hyperintensity in the soft tissue anterior to the right sacral ala suggestive of changes related to bone injury. Bilateral SI joints and bilateral iliac bones appeared unremarkable. These features were consistent with SIF. The patient was referred to a rheumatologist for further management and is being treated conservatively.


A 74-year-old man with a history of hypertension and on regular medication for 10 years had accelerating angina for 2 weeks. Six months earlier, he had accelerating chest pain for 1 month, which necessitated admission. His right coronary angiogram revealed diffuse severe stenosis involving the proximal to distal right coronary artery (RCA) (), which was treated with two overlapping DESs extending from the distal to the proximal RCA (3.0×38 mm and 3.0×30 mm, Endeavor™ Resolute™ stent; Medtronic Vascular, Santa Rosa, CA, USA) (). A platelet function assay showed 588 ARU and P2Y12 inhibition was 244 PRU (34%). He was discharged with dual antiplatelet therapy.
Six months after the index procedure, he had recurrent chest pain and a repeat angiogram showed diffuse ISR with Thrombolysis in Myocardial Infarction (TIMI) 2 distal flow () with pressure damping despite the use of a 5 Fr guiding catheter. Percutaneous coronary intervention was attempted. After balloon dilation with a 2.0 mm balloon, intravascular ultrasound revealed diffuse neointimal proliferation, but neither stent fracture nor overlap free segments were present. Sequential predilation of the distal to proximal RCA was performed with a 3.0×20 mm balloon. After balloon dilation with a 3.0 mm balloon, the RCA showed a stent-like result (less than 20% diameter stenosis with TIMI 3 flow) (). DEB ballooning was done at the mid to distal RCA with a SeQuent® Please (B. Braun Medical Inc., Melsungen, Germany) paclitaxel-eluting balloon 3.0×30 mm deployed at 8 atm/40 seconds (). After DEB ballooning, a no-reflow phenomenon developed with chest pain (). After intracoronary administration of 5 mg of nicorandil (dissolved in 10 mL of 0.9% saline), the distal flow was restored gradually. The final angiogram, taken 5 minutes after development of the no-reflow, showed TIMI 3 flow ().
The following cardiac enzyme and troponin remained within the normal range. The patient had an uneventful recovery and was discharged home on the following day.


A 46-year-old, Asian, male patient was diagnosed with AIDS with pneumonia in December 2010. HIV-encephalopathy was suspected on magnetic resonance imaging diffusion imaging and JC/BK virus polymerase chain reaction on the cerebrospinal fluid revealed negative findings. The patient refused antiviral treatment and did not return for scheduled appointments for HAART after he was diagnosed.
Two months later, the patient developed a confused mental state and gait disturbance. The patient started HAART with Combivir (GlaxoSmithKline, London, UK), Reyataz (Bristol-Myers Squibb, New York, NY, USA), and Norvir (Abbott, Chicago, IL, USA). The patient's HIV viral load was 826,000 copies/mL and CD4+ count was 6.59 cells/µL at admission.
Ten days later, multiple papules which were skin-colored and dome shaped in variable sizes were present on the face and neck (). The lesions were confirmed as MC by a dermatologist. Some lesions were surrounded by inflammation and varying size of erythema. Some evolved with central-umbilication. At this point, the CD4+ count was 20.28 cells/µL and the serum HIV viral load was 73,200 copies/mL. Some large lesions were curettaged.
In spite of increasing CD4+ lymphocyte count from 6.59 cells/µL to 20.28 cells/µL after 10 days of HAART, the CD4+ count was still under 200 cells/µL, suggesting a severely immunosuppressive state. The patient continued with HAART and was discharged. The skin lesions spontaneously healed and there were some residues.


A 65-year-old female presented with a swelling in the anterior chest wall of 6 months duration, which was gradually increasing in size. She also complained of fever and loss of weight. On examination, the swelling measured 6 × 4cm and was non-tender. She had no organomegaly. Her hemoglobin was 8 gm% and the erythrocyte sedimentation rate (ESR) was 110 mm at the end of 1st hour. The chest radiograph showed erosion of the manubrium sterni. FNAC performed on the swelling showed features of plasmacytoma.


A 60-year-old male presented with a diffuse swelling of 3 months duration on the anterolateral aspect of the chest wall on the left side. On examination, the swelling was diffuse and measured 12 × 10cm. His hemoglobin was 9 gm% and ESR was elevated to 80 mm at the end of 1st hour. Bence Jones protein was absent in the urine. Skull imaging showed lytic lesions. The swelling was subjected to FNAC.
The aspirated smears from the swellings in the chest wall were stained by hematoxylin and eosin (H and E) and Giemsa stains. The smears in both the cases were cellular and composed of plasmacytoid cells arranged singly and in clusters with varying pleomorphism. Binucleation of the cells was seen.
In the first case, the smears predominantly showed plasmablasts with prominent nucleoli along with few plasma cells showing moderate pleomorphism []. Plasma cells containing eosinophilic inclusion of immunoglobulin (Mott cells) were also observed in a good number in the smears [].
The second case on FNAC showed mainly mature-looking plasma cells and cells with nuclear atypia, pleomorphism, binucleation, open chromatin and basophilic cytoplasm [Figure , ]. Abnormal mitotic Figures were also seen.
The tumors of plasma cells can manifest as multiple myeloma, solitary plasmacytoma and extramedullary plasmacytoma. Multiple myeloma usually presents with low back pain and pathological fracture, but it can show varied presentation such as swelling of the jaw, orbit, and tonsil.[–] Swelling on the chest wall is an unusual presentation of multiple myeloma and can be a diagnostic dilemma for the clinicians.[] In such cases, a cytological diagnosis based on the FNAC of the swellings will definitely be very helpful in guiding the clinician to investigate further for confirmation.
On FNAC, the smears of plasmacytomas are cellular, composed of plasmacytoid cells showing pleomorphism with bi and multinucleation.[] We found similar appearing cells on the cytology smears of the second case. However, the smears in the first case showed predominantly plasmablasts with prominent nucleoli and eosinophilic intracytoplasmic inclusions with few plasmacytoid cells. In some cases, the cytology smears of myeloma show a mixture of plasmablasts and plasma cells in various stages of maturation with nuclear atypia, moderate pleomorphism and basophilic cytoplasm.[–] The first case showed the presence of anaplasia, increased plasmablasts and less number of mature plasma cells, and therefore the cytological evaluation was challenging in comparison to the second case where there were more of mature plasma cells.
In these two cases, a differential diagnosis of chondrosarcoma, primitive neuroectodermal tumor (PNET) (or Askin tumor), and metastatic carcinoma was considered at FNAC. Chondrosarcoma on cytology shows tumor cells with oval nuclei and vacuolated cytoplasm with small pink granules embedded in a pink matrix.[] PNET is seen in a younger age group and aspirates show a dissociative pattern of small malignant cells having a high nucleus: cytoplasmic (N: C) ratio with fine chromatin and lack nucleoli. The cytoplasmic vacuolation and membranous cytoplasmic blebs are noted occasionally. Metastatic carcinoma was ruled out due to lack of prominent nuclear pleomorphism and any cohesive arrangement of the cells.
In both the cases, the bone marrow aspiration showed more than 30% of plasma cells and urine was positive for Bence Jones protein in one case. The serum protein electrophoresis revealed an “M” spike in both the cases.
Multiple myeloma presenting primarily as a swelling in the chest is quite rare. The presence of increased plasmablasts, anaplasia and unusual location of the tumor are some of the challenges faced during the cytological evaluation. FNAC definitely offers an early and accurate method of diagnosis in such unusual sites of presentation of the disease, and thus is essential in helping the clinicians in clinically unsuspected cases.


A 56-year-old female with cancer in the right breast underwent a modified radical mastectomy in December 2001. At that time, the patient was diagnosed with breast cancer in stage T2N1M0. The tumor was positive for estrogen and progesterone receptors on immunohistochemical staining, and negative for HER2 overexpression by fluorescence in situ hybridization. Postoperatively, the patient was treated with adjuvant chemotherapy of cyclophosphamide, doxorubicin, and 5-FU every 3 weeks, for 6 cycles duration from December 2001 until May 2002, and hormonal therapy (toremifene citrate) from May 2002 until April 2006.
In April 2006, a follow-up bone scan revealed metastasis to the left scapula (), for which the patient was treated with doxorubicin and docetaxel every 3 weeks for 3 cycles. A follow-up bone scan revealed exacerbation of the metastasis, and in March 2007 (). At that time, the patient received intravenous (IV) zoledronic acid (4 mg) to prevent skeletal complications.
In October 2010, the patient accidentally tripped while walking and subsequently, felt pain in her right hip. Because she had no problems walking, she did not receive any specific treatment. A follow-up bone scan 1 month later demonstrated a focus of increased tracer uptake; however, the patient had no complaints of hip pain (). The finding was thought to be a bone metastasis, and zoledronic acid was administered on an ongoing basis.
In March 2011, the patient underwent X-ray evaluation at a local clinic because of intermittent right hip pain that was negative for fracture (). The following April, she developed pain in her right hip while jogging and had problems walking. Subsequent X-rays showed a right subtrochanteric fracture () and she underwent an open reduction with internal fixation (using compression hip screw & autogenous iliac bone graft) (). A bone biopsy of the fracture site demonstrated acute and chronic inflammation.
In addition, the left femur was observed to have cortical thickening and beaking on X-ray (). Therefore, treatment with zoledronic acid was stopped. The patient was treated with zoledronic acid from March 2007 until April 2011. Then, she was seen on routine follow-up in August 2011; the patient was using a wheel chair and the incomplete union of her right subtrochanteric fracture remained.


A 90-year-old Caucasian woman was found to have a multinodular white iris mass in the left eye (OS) and was referred for iris metastasis. Seven years previously she had uncomplicated cataract extraction with placement of posterior chamber intraocular lens. There was no history of postcataract iritis. There was no history of trauma or cancer. Visual acuity was 20/30 the right eye (OD) and 20/70 in the left eye (OS). There were white linear multilobular lesions in the inferior anterior chamber angle []. The cornea was clear without precipitates and the posterior capsule was intact. There was no gelatinous vitreous prolapse. Vitreous asteroid hyalosis was noted. The fundus was otherwise unremarkable. Anterior segment optical coherence tomography (OCT) disclosed iridoendothelial adhesion inferiorly with multifocal dense opacities causing posterior shadowing, consistent with calcification. These findings were consistent with asteroid hyalosis in the anterior chamber. Observation was advised.


An 80 year-old healthy Caucasian woman without a previous history of systemic cancer was found to have possible iris metastasis versus endophthalmitis. In the 1970s, she had cataract extraction both eyes (OU) without lens implantation. Two years previously, secondary anterior chamber intraocular lens was placed OU. There was no history of postcataract iritis. Visual acuity was 20/400 OD and 20/70 OS. In the affected OS, there was corneal epithelial edema, anterior chamber lens, and an open superior peripheral iridectomy. Fine, linear, bead-like, white lesions within prolapsed vitreous gel in the central anterior chamber was noted and large, bare calcified bodies were seen inferiorly in the angle, unattached to gel. [] Vitreous asteroid hyalosis was noted. A diagnosis of anterior segment prolapse of vitreous hyalosis was rendered and observation was advised.


A 10-month-old boy was brought to our department for an asymptomatic solitary, 3-mm size, firm white nodule over the heel of the left foot that had been present since birth []. The nodule had slowly grown in size during the first few months of life and then remained static. There was no discharge from the nodule. The infant had been delivered normally. The family history and medical history were unremarkable. General and systemic examination was normal. The infant had normal serum mineral values and renal function tests.
The nodule was excised and sent for histopathological study. Microscopic examination revealed massive compact hyperkeratosis and acanthosis of the epidermis. Dark basophilic-stained granular calcium deposits were found within cystic spaces in the dermis []. Von Kossa stain confirmed the presence of calcium []. Based on the clinical features and histopathology, we arrived at a diagnosis of subepidermal calcified nodule. On follow-up there has been no recurrence.


A 41-year-old man was referred to the outpatient clinic of Dermatology, Medical College, Thiruvananthapuram, by surgical gastroenterologist for evaluation. He presented with asymptomatic skin lesions over the abdomen and front of neck of 3-year duration. For the last one and a half year he is having early morning fatigue, tremor, sweating and difficulty in holding objects. He was earlier diagnosed by a neurologist to have absence seizures and was put on anticonvulsants but there was no relief. He also gave a past history of two episodes of ureteric colic 6 years back. General examination was normal except for an increased arm span (height -165 cm, arm span - 179 cm) and an elevated blood pressure of 160/100 mm of Hg.
Dermatological examination revealed multiple discrete skin-colored soft as well as firm dome-shaped smooth papules of 0.5 cm diameter over the abdomen and lateral aspect of trunk []. A single 2 × 1 cm hyperpigmented macule with serrated margins was seen over the right side of chest []. Multiple discrete brownish macules as well as hyperpigmented and erythematous papules were seen over the anterior and lateral aspect of neck and pre-sternal area []. A single whitish papule of 0.5 cm was seen over the right lower gingival mucosa []. Examination of other systems was normal.
With these findings, the possibility of steatocystoma multiplex or neurofibroma or collagenoma with angiofibroma and café-au-lait macules was considered. In view of the late-onset cutaneous tumors and associated systemic symptoms, the patient was subjected to detailed investigations. The routine blood and urine examinations as well as liver and renal function tests were normal. Serum calcium level was 11mg% and serum phosphorus was decreased (1.9 mg%; normal value: 2.5 - 4.5 mg %). Random blood sugar was 51mg%. In view of the repeatedly low blood sugar values, serum insulin was measured. It was found that serum insulin was elevated (112.47 μIu/ ml; normal value: 2-25 μIu/ml) with an increased insulin/glucose ratio (2.205) and a slightly raised C-peptide level (4.16 mg/ml; normal value 1-3 mg/ml). The detection of hyper insulinemia prompted us to do a detailed endocrinological evaluation. Serum parathormone was elevated (151.4 pg/ml; normal value: 15-75 pg/ml) whereas the other hormones like thyroid hormones, cortisol, gastrin and prolactin were normal. Ultrasonographical examination of neck revealed a hypoechoic lesion of 5.3×11 mm seen posterior to the right lobe of thyroid suggestive of parathyroid adenoma. Visual field examination, OGD scopy and X-ray skull were normal. Plain CAT scan of the abdomen showed a tumor involving the body and tail of pancreas and multiple calculi were noted in the left kidney. CAT scan of head was normal but MRI brain showed a space-occupying lesion in the pituitary suggestive of pituitary adenoma [].
Skin biopsy of the papules over the abdomen showed normal epidermis with increased dense connective tissue in the dermis which on subjecting to Van Gieson's stain- a mixture of Picric acid and Acid Fuchsin- took up red color confirming the diagnosis of collagenoma []. Erythematous papule on the pre-sternal area for which a provisional diagnosis of angiofibroma was made, showed hyperkeratosis, acanthosis and dilated blood vessels in the upper dermis with perivascular infiltrate which was more in favor of seborrhoeic keratosis rather than angiofibroma. Thus a final diagnosis of MEN type 1 was arrived at. The patient underwent parathyroidectomy and enucleation of the pancreatic tumor. The serum insulin level was normalized and his hypoglycemic symptoms disappeared after the surgical treatment.
MEN type 1 is a complex hereditary disorder encompassing pancreatic and pituitary tumors and hyperplasia of the parathyroid glands. Hyperparathyroidism is the most frequently expressed endocrinopathy in the syndrome which may also include on or more of the following: pancreatic tumors like insulinomas and gastrinomas, pituitary tumors like pinealomas, acromegaly, cushing's syndrome, thyroid tumors, carcinoids and hyperprolactinemia.[] The term MEN was introduced by Steiner et al in 1968 to describe disorders with a combination of endocrine tumors. These are complex genetic syndromes caused by activation or inactivation of different types of genes known to be involved in the regulation of cell proliferation and they include MEN 1, MEN 2, von Hippel - Lindau syndrome, Neurofibromatosis type 1 and Carney complex. Steiner and his colleagues also reported that the syndrome could be divided into two classes, MEN 1, (pituitary, pancreatic and parathyroid involvement) and MEN 2 (thyroid, medullary cancer, adrenal medullary and parathyroid disease).[]
Wermer in 1954 first described the classical triad of pancreatic, pituitary and parathyroid tumors which is now known as MEN 1. Hence MEN 1 is also known as Wermer's syndrome. It is inherited in an autosomal dominant pattern and is caused by inactivating mutation of the tumor suppressor gene MEN 1 on chromosome 11q13 which encodes a peptide called menin. Its prevalence is 1 in 30,000. Among cases showing biochemical expression of MEN 1, 90-97% exhibit primary hyperparathyroidism; pancreatic tumors are manifested in 30-80%, and pituitary tumors are manifested in 15-50%.[] The cutaneous lesions seen in MEN 1 include angiofibromas (88%), collagenomas (72%), café-au-lait macules (38%), lipomas (34%), confetti-like hypopigmented macules (6%) and gingival papules (6%). Collagenomas are skin-colored to slightly hypopigmented firm, round to oval papules and nodules of 0.2-2cm diameter, mostly located on upper part of trunk and neck and show an increased amount of collagen and normal or decreased elastic fibers on histopathological examination.Other conditions where collagenomas are seen include Tuberous sclerosis, Birt-Hogg-Dubé syndrome, Down syndrome, Chronic myelocytic leukaemia, Syphilis, Cowden's syndrome, Proteus syndrome and Encephalocraniocutaneous lipomatosis.[]
The constellation of these cutaneous findings like collagenomas, angiofibromas and gingival papules do not establish the diagnosis of MEN 1 as they are also seen in other genodermatoses like tuberous sclerosis (TSC) and Birt - Hogg - Dubé syndrome (BHDS). shows the overlapping clinical features seen in these three disordersBHDS is an uncommon autosomal dominant genodermatosis characterized by a triad of skin tumors - fibrofolliculomas, trichodiscomas and acrochordons - together with an increased risk of renal tumors and spontaneous pneumothoraces. BHDS should be considered, along with tuberous sclerosis and multiple endocrine neoplasia type 1 in the differential diagnosis of multiple facial angiofibromas and collagenomas, particulary when the onset is in adulthood.[] Angiofibromas seen in MEN 1 are clinically and histologically indistinguishable from those observed in TSC.[] However, angiofibromas in patients with MEN 1 tend to be smaller and fewer in number than in those with severe TSC. In the former, they also tend to appear on the upper lip and its vermilion border, regions usually spared in patients with TSC.[] So a thorough search should be made for other associated cutaneous tumors and systemic involvement before arriving at a final diagnosis.
Angiofibromas and collagenomas (single or multiple) have 50-65% sensitivity and 92-100% specificity for MEN 1. The combination criteria of multiple angiofibromas (more than three) and any number of collagenomas had the highest sensitivity (75%) and specificity (95%). This criterion has greater sensitivity than pituitary or adrenal disease and is comparable to hyperparathyroidism in some studies of patients with MEN 1 with gastrinoma.[] These cutaneous tumors also help in the presymptomatic diagnosis of MEN 1 because, even though the symptoms of endocrine tumors are delayed until the fourth decade, the cutaneous tumors make their first appearance in the teenage years itself. In addition, symptoms of hormone dysregulation may mimic non-endocrine diseases including neurologic or psychiatric disorders. Depression often associated with hyperparathyroidism and hypercortisolism.[] Confusion and abnormal behavior characterize hypoglycemic episodes in patients with insulinomas and they are often unrecognized or mistaken for seizures or psychiatric disorders.[]


A 50-year-old male diagnosed presumptively as onychomycosis presented with a white superficial lesion on the right index finger. The lesions appeared 4 months back. There was no history of itching, pain or discharge from the lesion.


A 70-year-old male presented with erythematous scaly plaques on the left little finger since 3 years with no itching, pain and discharge from the lesion.


A 45-year-old male presented with interdigital plaques on the right foot since 5-6 years with no itching, pain or exacerbation.
Direct wet mounts revealed hyaline, septate, branched hyphae. Culture on Sabouraud's dextrose agar grew white, cottony colonies which later turned pink in color. Lactophenol cotton blue mount of colony revealed septate, branched hyphae with microconidia and many sickle shaped macroconidia []. The fungus was identified as Fusarium solani. All the cases were treated with oral Itraconazole 200 mg per day for 2 months and significant clinical and mycological recovery was achieved in all three cases.