A 17-month-old boy was admitted to the Department of Neurosurgery, Children’s University Hospital in Cracow due to aggravating problems with balance. Starting a week earlier, the parents noticed that the boy had a tendency to tilt the head to the right and would choke when drinking. Neurologic examination found the following deficits: cerebellar disturbances of balance illustrated by the boy’s staggering and inability to walk, compulsory tilting of the head to the right, slight right pyramidal hemiparesis, left VI and VII cranial nerve palsies, bulbar signs demonstrated by choking, weak pharyngeal and palatal reflexes, and a suspected lesion in the visual field which could not be confirmed due to the child’s lack of cooperation. Computer tomography (CT) and magnetic resonance imaging (MRI) revealed an enormous tumor (44 × 35 × 45 mm in CT and 35 × 36 × 32 mm in MRI) in the brainstem, mostly in the left pons and medulla oblongata (Fig. ). The tumor was hyperintense in T2 and showed heterogenous contrast enhancement in T1 with narrowing of the fourth ventricle (Fig. ). There were no features of supratentorial hydrocephalus in spite of evidence of elevated pressure in the posterior fossa. The state of the child was unstable and the risk of its marked worsening due to operation was high. Even the biopsy bore a significant risk. As a result, the parents, after meticulous consideration of the pros and cons, did not give consent to neither an operation nor biopsy on assumption that the child would receive chemotherapy, which was immediately introduced. Consecutively, the child was transferred to the Department of Pediatric Hematology and Oncology, Children’s University Hospital in Cracow. Further diagnostics did not reveal any other foci of disease. Based on the clinical picture and neuroimaging, a malignant glioma of the brainstem was tentatively diagnosed and chemotherapy was introduced accordingly with two cycles of cisplatin, etoposide, and vincristine, and one cycle of cyclophosphamide, etoposide, and vincristine. Two months after first admission, the child showed signs of ataxia and decreased muscle tone. Soon, the child’s condition abruptly worsened with severe vomiting and a forced retroflexed position. Repeated MRI revealed enlargement of the tumor (52 × 50 × 54 mm) and supratentorial hydrocephalus due to compression of the cerebral aqueduct with transudation (Fig. ). The hydrocephalus was treated with implantation of a ventriculo-peritoneal shunt, and chemotherapy with irinotecan and carboplatin was introduced. The hydrocephalus remitted but the child relentlessly deteriorated as the tumor continued to enlarge and died 3 months after hospitalization.
An autopsy of the brain performed at Department of Pathology, Children’s University Hospital, revealed an enormous tumor inside left cerebellar hemisphere partially occupying the pons and also expanding to the midbrain. The tumor distorted the whole brainstem and cerebellum, pushing aside and compressing the fourth ventricle, but definitely not originating from it (Fig. ). This confirmed the previous MRI findings of a primary brainstem tumor (Fig. ). On cross section, the tumor showed uniform consistency, slightly lower than the normal brain tissue. The tumor had well marked borders with a smooth transition into adjacent apparently normal tissue.
Microscopic pictures represented a very conspicuous pattern with relatively paucicellular neuropil-like background with numerous and rather evenly distributed cellular densities populated with small undifferentiated cells (Fig. ). Rosettes formed another characteristic element of the tumor (Fig. ). Neuropil zones, in contrast to the cellular zones, showed immunopositivity to synaptophysin (Fig. ). Both in cellular zones and neuropil zones, there were scattered characteristic rosettes, totally negative to synaptophysin (Fig. ) but strongly positive to vimentin (Fig. ). The rosettes had an “empty,” homogenous core, but frequently with a delicate eosinophilic contouring reminiscent of ependymoblastic rosettes, though somewhat more delicate than those occurring in typical ependymoblastoma (Fig. ). The rosettes were also weakly positive for glial fibrillary acidic protein (GFAP) and for S100 protein (Fig. ). However, in neuropil zones, there were cells present with the immuno- and morphophenotype of astrocytes (GFAP+ and some S100+). Moreover, outside of the rosettes, synaptophysin-positive mature neuron-like cells were noted (Fig. ), and some were even strongly positive for synaptophysin, in spite of resembling astrocytes (Fig. ). Ki-67 expression was high but limited to rosettes (Fig. ) and cellular zones especially around the vessels. No areas of frank necrosis or any “pathological” forms of endothelial proliferation were noted. However delicate vessels were numerous, frequently surrounded by undifferentiated cells that do not conform to the description of “perivascular formations” or pseudorosettes.


A 40-year-old nulliparous lady, married for 23 years, came with a 6-month history of abdominal discomfort. She had no history of bowel or bladder complaints, weight loss, anorexia, or fever. Previous menstrual cycles were regular. On examination, the patient was afebrile, pulse 76/min, and blood pressure 130/80 mmHg. Abdominal examination revealed a mass of 24-26 weeks size gravid uterus, which appeared to be arising from the pelvis. On palpation, mass was of found to vary in consistency, non-tender, transversely mobile with irregular margins. The lower pole could be reached partially. On speculum examination, cervix could not be visualized, and no abnormal discharge was seen. On vaginal examination, the mass was felt in the pouch of Douglas pushing the cervix anteriorly. Forniceal fullness was present and it was non-tender. Routine blood analysis was within normal limits. Serum Human chorionic gonadotropin values were <0.10 milli international units per liter. Serum CA-125 (cancer antigen 125) value was 28 milli international units per liter. On ultrasonography, a well-defined, mixed echogenic lesion measuring 22 cm × 12.2 cm × 18.6 cm was seen in the midline posterior to bladder pushing the uterus anterolaterally toward the left side [], extending cranially up to mid-abdomen with multiple internal cystic changes []. There was moderate to gross internal vascularity. Minimal fluid with internal echoes was noted in the endometrial cavity. There were two fibroids measuring 1.5 cm × 1.5 cm and 3 cm × 3 cm in the anterior and left lateral wall respectively. The right ovary was not seen as separate from the mass. Left ovary was normal. There was mild hydroureteronephrosis in the right kidney likely due to ureteric obstruction by the right adnexal mass. Diagnosis of right ovarian neoplasm was made. On contrast-enhanced computed tomographic (CECT) scan of abdomen and pelvis, a large mixed density abdomino-pelvic lesion was seen in the pouch of Douglas with thick internal septations and solid components []. The septations and solid components showed significant enhancement on postcontrast images []. The CECT appearance was typical of cystic ovarian neoplasm. Final diagnosis based on the CECT scan of abdomen and pelvis was right ovarian neoplasm.
With a provisional diagnosis of malignant ovarian tumor, the patient was taken up for an exploratory laparotomy. Intra-operatively, an abdomino-pelvic mass of size approximately 24 cm × 18 cm × 8 cm was seen with variable consistency and increased vascularity, arising from the right side of the uterus pushing the ureter laterally. Right fallopian tube, ovarian ligament, and round ligament stretched over the mass. Right ovary was normal. Left tube and ovary were normal. The mass was loosely adherent to the small bowel loops. Two fibroids in the fundus of uterus measuring 2 cm × 2 cm and 3 cm × 2 cm and 5-6 seedling fibroids were noted per-operatively. As the tumor was distorting the pelvic anatomy, careful dissection was done to prevent ureteric injuries. Excision of tumor with total hysterectomy and bilateral salpingo-oophorectomy was done. Post-operative course was uneventful.
The surgically resected specimen included a large nodular cystic mass measuring 25 cm × 25 cm × 8 cm adjacent to the uterus in the right broad ligament with bilateral normal ovaries []. On histopathological examination, the cut section of the mass showed solid fleshy areas and cystic areas filled with hemorrhage. The uterus revealed two large and multiple small intramural fibroids. The largest one measured 4 cm × 5 cm and smallest 0.5 cm in diameter. Right fallopian tube was stretched and measured 14 cm and right ovary was 3.5 cm × 1.5 cm × 0.5 cm. Cut section of ovary showed cyst filled with serous fluid. Cyst measured 2 cm in diameter. The ligamentous membranous attachment to the lesion seen on the right side was noted to be the broad ligament. Left fallopian tube was 3.5 cm. The mass in the broad ligament stained with hematoxylin and eosin showed the endo-myometrium was composed of round to oval glands lined by columnar epithelium set in a compact stroma. Section studied from the larger parametrial mass showed a benign tumor with interlacing bundles of smooth muscle cells, scattered thick-walled blood vessels, and evidence of cystic, myxoid and hyaline degeneration []. Cervix showed a mild-to-moderate sub-epithelial lymphocytic infiltrate. There was no evidence of dysplasia or malignancy in the cut sections of the cervix. The final histopathological report was:
Proliferative phase endometrium Leiomyoma: Intramural and parametrial (mass arising adjacent to the uterus) Chronic cervicitis Bilateral adnexa with no significant pathology


A 69-year-old man with upper abdominal pain was referred to our hospital in August 2006 by a local physician. Upper gastrointestinal endoscopy revealed a Borrmann type II-like tumor in the posterior wall of the lower part of the stomach (). The biopsy specimen revealed well-differentiated adenocarcinoma, and immunohistological staining showed that tumor cells were positive for alpha-fetoprotein-producing (AFP). An upper gastrointestinal series radiography showed an elevated lesion with a central depression () in the lower part of the stomach. Laboratory examination revealed mildly elevated levels of aspartate aminotransferase (AST 67 IU/L), lactate dehydrogenase (834 IU/L), and alkaline phosphatase (565 IU/L). Serum level of carcinoembryonic antigen and CA19-9 was normal, but that of AFP was markedly increased (160,000 ng/ml).
Abdominal contrast-enhanced computed tomography (CECT) revealed a 14 cm long hypovascular tumor in the left lobe of the liver (). On the basis of these findings, we diagnosed AFPGC with synchronous liver metastasis.
Our initial treatment plan was to perform a distal gastrectomy and an extended left lobectomy of the liver. However, another liver metastasis was found in the right lobe of the liver by the intraoperative sonography. We limited the surgery to distal gastrectomy with D2 lymph node dissection and Billroth I reconstruction in August 2006. The resected specimen was determined to be a 2.8 cm long Borrmann type II-like tumor ().
Histopathological examination of the gastric tumor revealed a well-differentiated adenocarcinoma with neoplastic growth of hepatocyte-like cells (). The level of invasion was submucosal and 3 of 35 dissected lymph nodes were positive for metastasis. Intraoperative biopsy of the liver tumor showed features similar to those of the gastric tumor (). Immunohistological staining showed that tumor cells in both the gastric tumor () and the biopsy specimen from the liver tumor () were positive for AFP.
After surgery, we placed a catheter into the proper hepatic artery for HIA. Chemotherapy using oral tegafur/gimeracil/oteracil potassium (TS-1, Taiho Co. Ltd., Tokyo, Japan) (120 mg/body/day from days 1 to 21)/HIA-cisplatin (CDDP) (30 mg/body/day on days 8 and 21, every 28 days) was started at day 15 after the surgery. After 4 courses, CECT revealed that the size of the metastatic tumor in the left lobe had decreased to 53.6% of the peak (), and the small liver metastasis had disappeared. The serum AFP level had decreased to 2,910 ng/ml.
Six months after the surgery, the serum level of AFP began to increase, that is why we performed a left lobectomy. The resected specimen showed atrophic change in the left lobe and an odd-shaped soft liver tumor (). On cut sections, the tumor was yellowish and highly denatured (). Microscopic findings revealed degenerative changes and necrosis in almost all sections of the tumor. Viable cells were similar in structure to the microscopic findings of the primary gastric cancer. After liver resection, 9 courses of adjuvant oral TS-1/HIA-CDDP and oral TS-1/CPT-11 by drip infusion of vein (div) were administered.
Twenty-five months after the first surgery, we performed a partial lung resection because of new metastasis to the lung (). Four months after partial lung resection, radiotherapy was performed to treat 2 new lung metastases. We obtained complete response transiently, but another new lung metastasis and para-aortic lymph node metastases appeared immediately. To treat these metastatic lesions, we performed chemotherapy sequentially as follows: 6 courses of paclitaxel (PTX) (div); 2 courses of 5'-Doxifluridine (DFUR) (oral)/Docetaxel (DOC) (div)/CDDP (div); 5 courses of DFUR (oral)/DOC (div); 8 courses of intravenous injection of 5-fluorouracil (5FU)/Adriamycin/Mitomycin-C (FAM); 2 courses of TS-1 (oral)/CPT-11 (div); and 6 courses of PTX (div) ().
In spite of these combination chemotherapies, metastatic lesions became difficult to control at 54 months after the first surgery, and the serum AFP level rose to 84,434 ng/ml. Liver metastases () and mediastinal lymph node metastasis enlarged gradually (). The patient complained of respiratory distress resulting from bronchial oppression by enlarged lymph nodes. We could not use trastuzumab because immunohistochemical analysis revealed that human epidermal growth factor receptor type 2 was not expressed in either the primary lesion or the metastatic liver tumor. We proposed best supportive care, but the patient wanted to try a molecular-targeted drug. The administration of oral sorafenib tosylate (sorafenib) was begun at a dose of 800 mg/body/day using of a private import. After 2 months, his serum level of AFP dropped from 100,000 to 59,198 ng/ml. CECT revealed marked attenuation of vascularity at the liver metastasis, but a decrease in tumor size was not observed (). The mediastinal lymph nodes shrunk in size by 16.2% (). The patient's respiratory distress was completely alleviated, and stable disease was observed for a period of 4 months. The only adverse events observed were grade 1 hoarseness, increased AST, and hand-foot syndrome. The patient eventually died from cancer cachexia at 60 months after the first surgery.


A 55-year-old Asian male was referred to our department for the management of suspicious hypopharyngeal cancer diagnosed by endoscopic biopsy at a primary medical clinic. The patient was not present with symptoms related to hypopharyngeal disease, such as dysphagia or throat discomfort. He was a 40-pack/year current smoker and consumed alcohol on a regular basis (>3 times per week). He had previously undergone Ivor-Lewis operation for esophageal squamous cell carcinoma (pT1N0M0) 1 year ago. Pathologic review of the biopsy specimen from the referring clinic showed squamous cell carcinoma. Endoscopic examination disclosed a round slightly elevated hyperemic mucosal lesion, approximately 8 mm at the longest diameter, at the right pyriform recess (). NBI showed dilated, tortuous and irregular intrapapillary ca-pillary loops (IPCL), consistent with type V IPCL pattern described by Inoue et al. (). Contrast enhanced neck and chest computed tomography (CT) scans showed no evidence of focal lesions or lymph node enlargement in the head and neck region, and positron emission tomography-CT scans were unrevealing. Based on these findings we diagnosed stage I early hypopharyngeal squamous cell carcinoma (cT1N0M0). The patient refused extensive resection and endoscopic resection was planned. Endoscopic resection was performed under general anesthesia. A cap fitted, forward-viewing, sterilized endoscope (EG2001i; Pentax Medical Systems Corp., Tokyo, Japan) was used for the entire procedure. Lugol's solution was applied for demarcation of the lesion, and marking around the lesion was performed with the Dual knife (KD-650Q; Olympus, Tokyo, Japan). Saline solution with 0.001% epinephrine and 0.004% indigo carmine dye was injected with a 23-gauge needle (NM-200L-0423; Olympus) into the submucosa under and around the lesion for mucosal elevation. After sufficient mucosal elevation around the lesion had been achieved, a snare was introduced into the accessory channel and suction was applied. The lesion was ensnared and resected en bloc using the cap assisted endoscopic mucosal resection (EMR-C) technique. The total procedure time was 35 minutes, and no complications occurred during the procedure. The resected specimen measured 10×9 mm and an erythematous elevated lesion was observed in the center of the specimen ().
Immunohistochemical staining and pathologic analysis was consistent with squamous cell carcinoma in situ with p53 protein overexpression in the full thickness of the epithelial layer (). Both lateral and vertical resection margins were free from involvement of the tumor. The patient recovered without incident and was discharged on the 6th postoperative day. Six months later on routine endoscopic follow-up, a slightly depressed hyperemic lesion was observed at the opposite left pyriform sinus (). Although endoscopic biopsy was negative, NBI showed an irregular vascular pattern () and additional EMR-C was performed (). Final pathologic results showed low grade dysplasia with focal p53 protein overexpression confined to the lower one-third of the epithelial layer ().


A 70-year-old man, with a medical history of vagotomy for a peptic gastric ulcer (operated in another institution 34 years ago), had an incidentally discovered mass in the abdomen on plain chest radiography. The mass was 8 cm diameter and was located on left hypochondrium. The only symptomatology was mild bloating, started a few months before. The computerized tomography (CT) scan showed a heterogeneous and low-density mass, with peripheral calcifications, measuring 7.4 cm diameter. The presumptive diagnosis made by the radiologist was gastrointestinal stromal tumour (GIST) [].
Endoscopic biopsy provided no additional information about the mass etiology, evidencing signs of external compression without mucosal lesion.
The patient was then submitted to laparoscopy. During the procedure, dissection showed a capsulated structure. After violation of the capsule and drainage of debris, a retained sponge was discovered []. An aseptic plastic material was used to wrap it for laparoscopic removal.


A 53-year-old woman with a history of AN since adolescence was admitted to the psychiatric clinic at the Uppsala University Hospital due to psychotic behaviour and inability to take care of herself. There were reports of paranoid schizophrenia and personality disorders, but her contacts with the psychiatric clinic were sparse, and she used no medications. According to her relatives she had isolated herself the last two weeks, and over the last two months she had barely been eating at all. Twenty-four hours after admission to the psychiatric clinic she was admitted to the clinic of internal medicine and presented at the emergency room (ER) in a cachectic state with hypothermia (32.6°C). Her mental status was altered (reaction level scale 2), and she barely responded to questions, being close to stupor. There were clinical signs of severe dehydration and muscle atrophy. Her breathing was shallow with 30 bpm, whereas blood pressure and pulse rate were normal, 110/60 mmHg and 77 bpm (see for a summary of the initial physical examination). She denied alcohol and any substance abuse. An initial arterial blood gas analysis displayed pH 6.895, pCO2 0.93 kPa, pO2 22 kPa, and P-glucose 40.6 mmol/L. There were no signs of infection, and electrolytes were normal (see for a summary of laboratory screening). She was immediately admitted to the intensive care unit (ICU), where rehydration was initiated with warm fluid combined with re-warming with heated blankets. Bicarbonate (100 mL) was administered i.v. in order to reverse acidosis; pH increased to 7.1, and blood glucose decreased to 35 mmol/L. Six hours after ICU admission insulin infusion was started with initially 0.5 IU/h (0.0128 IU/kg) combined with 5 mmol potassium/h. The patient's pH was normalized 15 hours after admission to the ICU, and the blood glucose concentration was kept around 15 mmol until 36 hours after admission. Variations of pH, glucose concentration, and arterial pCO2 and pO2 during the first 24 hours have been displayed in . Laboratory screening at the ICU revealed a P-C-peptide concentration of 0.14 nmol/L (ref. 0.4–1.5 nmol/L), a HbA1c of 151 mmol/mol (ref. <46 mmol/mol), and presence of both glutamic acid decarboxylase (GAD) (74 IE/mL, ref. <5) and insulinoma antigen 2 (IA2) antibodies (69 kE/L, ref. <8).
Due to the risk of re-feeding syndrome, the intake of calories was restricted to 500 kcal over the first 24 hours and then increased when the patient was able to drink nutritional drinks. The risk of re-feeding syndrome is especially high in patients with a BMI <16, recent weight loss, and electrolyte abnormalities (). In order to prevent re-feeding syndrome, especially carbohydrates should be restricted whereas proteins and fats seem to be less of a threat (). However, in this case administration of i.v. glucose (120 kcal) in the first 24 hours could not be avoided, since plasma glucose concentrations had to be maintained around 15 mmol/L during the insulin infusion in order to prevent the risk of brain oedema. Remaining energy intake was administered per os as nutritional drinks with a balanced protein, fat, and carbohydrate content. A transthoracic echocardiogram was performed in order to exclude ventricular dysfunction and mitralic valve prolapse which can be observed after starvation and can potentially be lethal, especially in combination with re-feeding syndrome (). An expanded health history revealed that the patient had rapidly lost 10 kilograms of weight over the last two weeks and had polydipsia and polyuria. It was noted that her niece had T1D but there was no family history of diabetes.
As pH and blood glucose concentrations normalized, the patient improved; she was transferred to an internal medicine ward, and after optimization of the insulin regime she was transferred to the psychiatric clinic for continuous psychiatric care. She is currently on treatment with Insulin Glargine and Insulin Aspart, and on her last clinical control her fasting glucose was 16.1 mmol/L (ref. 4.0–6.0), HbA1c 82 mmol/mol (ref. 31–46), and her P-C-peptide had further decreased to 0.093 nmol/L (ref. 0.4–1.5). There have been no additional episodes of DKA, and her weight has increased to 55 kg with a BMI of 18.2 kg/m2 (ref. 20–25). In , there is a summary of laboratory screening six months post-DKA.


A 2-year-old boy was brought to the pediatrician in view of non-specific respiratory symptoms where mother thought that he had a brief episode of apnea during sleep. The child was otherwise completely asymptomatic. He was then referred for a chest X-ray [] that showed significant cardiomegaly and therefore an echocardiogram was obtained. This showed a giant right atrial aneurysm (7 × 6 cm) []. The tricuspid valve was anatomically normal with no stenosis or insufficiency. A small left-to-right shunt across a patent foramen ovale was also noted. Rest of the cardiac anatomy was normal. These findings were later confirmed by a cardiac magnetic resonance imaging (MRI) [Figures and ]. Slow-flow artifacts were seen within the lumen of the right atrium without intracavitary thrombus. To our surprise, the electrocardiogram was normal. It showed normal sinus rhythm with normal P wave morphology and no rhythm abnormalities.
We opted to proceed with surgical resection of the aneurysm after Discussions with the family, because of the risk of future thromboembolic complications and arrhythmias. Surgery was performed through median sternotomy. The pericardium was normal. A giant thin-walled aneurysm of the entire free wall of right atrium was noted []. After establishing cardiopulmonary bypass, the aneurysm was opened. There was no evidence of thrombus or inflammation within the right atrial aneurysm. The aneurysm was resected as two pieces measuring 2 × 2 cm and 7 × 3.5 cm respectively. After excision of the affected right atrial wall, the defect was closed and care was taken not to compromise the right coronary artery. Direct suture closure of the patent foramen ovale was also performed. Histopathological examination of the resected atrial tissue showed paper-thin walls [], and the pathological features included interstitial fibrosis and endocardial thickening with no evidence of thrombosis.
The postoperative course was significant for short non-sustained runs of ectopic atrial tachycardia that were controlled on Atenolol. He was discharged home 4 days after surgery. The chest X-ray [] performed following surgery showed significant reduction in cardiac size. An echocardiogram [] performed prior to discharge showed significant reduction in the right atrial size, trivial tricuspid regurgitation, and a small pericardial effusion. At the time of first post-operative visit to the clinic a week from discharge, the effusion had resolved and there were no new findings on the echocardiogram.


An 81-year-old lady presented to the on call general surgeons with abdominal pain and vomiting for 24 hr. Two years previously she had undergone a laparoscopic abdominoperineal resection for carcinoma of the rectum at another centre. She had not previously received radiotherapy and was a non-smoker. History, examination and abdominal X-ray suggested small bowel obstruction. On admission, there were no signs of peritonism, observations were normal and markers of inflammation were not significantly raised.
Although not known to have a hernia, she described discomfort and swelling in her perineum over the previous year that sometimes made it difficult to sit down. Direct examination of the perineum confirmed the presence of an irreducible but non-tender hernia (Fig. ). Conservative treatment with nasogastric drainage and intravenous fluids produced no clinical improvement. The following morning a CT of the abdomen and pelvis was performed which confirmed small bowel obstruction and revealed a cut off at the neck of a perineal hernia.
At operation, small bowel obstruction was found with volvulus of a loop of ileum around an adhesion at the neck of the perineal hernia (Fig. ). Around 80 cm of gangrenous mid-ileum was resected and a primary end-to-end anastamosis performed. After thorough washout of the abdomen and pelvis, the wide pelvic floor defect was bridged with Strattice™ Tissue Matrix, secured in place with 2.0 prolene sutures (Fig. ). An omental pedicle graft was used to cover the mesh and fill the pelvis. One abdominal tube drain and two perineal suction drains were inserted and removed over the course of the next 7 days.
The patient made an uncomplicated recovery and was discharged home. At 6 months follow-up, she remained well with no reported problems other than a slight increase in stoma activity. There was resolution of her previous perineal symptoms and no clinical recurrence of the hernia.


An 83-year-old female with a left upper lid swelling was referred by her physician. She reported having this gradually enlarging, painless swelling for the last 2–3 years, which was now causing her eyelid to droop. She had been a soft contact lens wearer for 14 years. There was no history of any ocular trauma or lid surgery. On examination, a freely mobile 2 cm × 3 cm cystic swelling was noted in the left upper lid, which was causing an upper lid ptosis of 1 mm []. The rest of the ocular examination was normal. A presumptive diagnosis of chalazion was made and an incision and curettage was performed. During the procedure, a soft silicone hydrogel contact lens with granulation tissue was noted at the base of the cystic lesion and removed []. On further questioning, it transpired that 13 years previously she had lost a soft contact lens from her left eye. She assumed that it has spontaneously fallen out and she never went to see an optician or an ophthalmologist. Following this incident, she resumed uneventful contact lens wear.


A 41-year-old male patient with no significant past medical history visited our hospital due to a painful erection that lasted for 6 hours. He had also been seen in the emergency department of another hospital 5 days prior with the same condition. On both occasions, he was treated by aspirating blood from the corpus cavernosum followed by conservative management. The patient was subsequently discharged after detumescence. In our hospital, the patient's vital signs were within normal limits, and physical examination revealed an erection with 90~100% rigidity.
A complete blood count was unremarkable, with a hemoglobin of 17.1 g/dl, red blood cell count of 5,240 k/ul, white blood cell count of 9,900/ul, and platelet count of 246 k/ul. Liver function tests indicated elevated aspartate aminotransferase (82 IU/L), alanine aminotransferase (110 IU/L), and alkaline phosphatase (189 IU/L). Erythrocyte sedimentation rate and C-reactive protein were also increased at 44 mm/h and 24.39 mg/L, respectively. Chemical tests, blood clotting tests, and urinalysis were within normal limits. A corporal blood gas test suggested ischemic priapism, with a pH=6.9, pCO2=146.5 mmHg, and pO2=37 mmHg. Testing for sexually transmitted infections revealed a venereal disease researchlaboratory (VDRL) titer of 1:128, rapid pasma reagin=2.5, and treponema pallidum hemagglutination test titer of 1:2,560 suggesting reactive syphilis. Other tests including Chlamydia and human immunodeficiency virus antibody tests were negative. Color Doppler sonography was not performed due to the irritable state of the patient.
The patient was treated with intramuscular benzathine penicillin G (2,400 kIU) 3 times at intervals of one week. Aspiration of blood from the corpus cavernosum resulted in detumescence, but priapism recurred on 3 occasions over a 4 weeks period. Although intracavernous injection of sympathomimetic drugs was performed, the condition of the patient did not improve. We chose to initiate surgical intervention using a cavernoso-glandular shunt, which was performed using a 1 cm incision along the glans (). Post-operatively, complete detumescence was achieved. Since the VDRL titer dropped to 1:64, the titer remained unchanged despite of penicillin treatment. Therefore, we performed a cerebrospinal fluid (CSF) examination to exclude neurosyphilis. On analysis of CSF during hospitalization, the VDRL titer was increased at 1:4. The patient refused further treatment or evaluation and was discharged. The patient died of pancreatic cancer at another hospital several months later.


The patient is a 50-year-old man who is presently admitted to psychiatric hospital because of severe MDD. He has suffered from MDD symptoms for 10 months consecutively with symptoms progressing from mild to incapacitating. He admits to having impaired concentration, marked fatigue, depressed mood with suicidal thoughts, insomnia, ruminations of worthlessness, and feelings of guilt. This is his first diagnosed depressive episode and he has had no drug treatment prior to this hospitalisation. He likely suffered from dysthymic disorder throughout his adult life. He is amicably separated and has three grown children who are doing well. His biological family suggests ADHD symptoms in a nephew and schizophrenia in an elderly uncle. He drinks alcohol occasionally, but there is no indication of misuse. He has mild obesity, and environmental allergies, but is otherwise healthy.


A 66-year-old woman was referred to our hospital for treatment of a gastric tumor. She had been diagnosed with PBC at the age of 60 years and had been treated with ursodeoxycholic acid. Although she had been stable and asymptomatic for several years, a gastric tumor was detected on upper gastric endoscopy performed due to abdominal discomfort after meals a month before visiting a local hospital. Abdominal computed tomography (CT) and 2-[18F]fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) showed the localized stage of malignant lymphoma in her stomach. Endoscopic biopsy examination confirmed diffuse large B-cell lymphoma (DLBCL). She showed good performance status (PS1) and normal serum lactate dehydrogenase level (189 IU/l, normal range 121–220 IU/l). She had been diagnosed with gastric DLBCL with stage II1 in the Lugano classification and showed low international prognostic index (IPI, 1 point) []. With regard to PBC status, she had no jaundice (serum total bilirubin 1.0 mg/dl, normal range 0.2–1.3 mg/dl) and showed well-preserved liver function (serum albumin 4.0 g/dl, normal range 4.0–5.0 g/dl; PT% 100%, normal range 80–120%), although biliary enzyme levels were slightly elevated (gamma-glutamyl transpeptidase 214 U/l, normal range 10–47 U/l; ALP 826 U/l, normal range 115–359 U/l). Her serum IgM and AMA-M2 titers were high (IgM 615 mg/dl, normal range 57–288 mg/dl; AMA-M2 70, normal index <7), reflecting PBC.
Prior to treatment for DLBCL, we performed a biopsy on the right lobe of the liver and found chronic non-suppurative destructive cholangitis with interface hepatitis consistent with PBC at Scheuer's histological stage II (fig. ). According to the risk score of PBC, her risk score before treatment of DLBCL was calculated as 5.5 and the estimated probability of survival for 24 months was >99% []. Thereafter, she was treated with chemotherapy, including rituximab (R-CHOP – cyclophosphamide/hydroxydaunorubicin/oncovin/prednisone, rituximab 375 mg/m2) plus radiation 40 Gy as shown in figure . After treatment with 8 doses of rituximab, her biliary enzyme and serum IgM levels decreased gradually and the AMA titer also decreased (fig. ). Moreover, we found that portal inflammation had decreased after rituximab treatment (fig. ). She finished R-CHOP plus radiation therapy and achieved complete remission of DLBCL. Thereafter, she was observed and remained in a stable condition for 24 months after diagnosis of DLBCL. She was asymptomatic and her PBC Mayo risk scores remained low for 24 months after diagnosis of DLBCL (fig. ). However, she showed marked jaundice and ascites without elevation of serum AMA titer 30 months after diagnosis of DLBCL. CT scan showed bile duct cancer at the bottom of the common bile duct in addition to cirrhotic changes such as ascites. Percutaneous drainage of the bile duct was immediately and successfully performed and her jaundice was partially decreased, but surgical resection or chemotherapy could not be done due to decreased liver function. Hepatobiliary scintigraphy using 99mTc-PMT showed disturbance of hepatocellular excretion of bile (data not shown). Therefore, her cirrhosis was estimated to be already established at the time when jaundice developed. She received the best supportive care and died from liver failure 3 years after diagnosis of DLBCL. Autopsy showed bridged fibrosis formation of the liver (fig. , Scheuer's histological stage IV) and lower bile duct cancer. Prominent infiltration of inflammatory cells was not found in the liver (fig. ).


A 44-year-old woman was admitted to our hospital in June 2011 complaining of arthralgia in both knees, elbows, and ankles. She had been diagnosed with hepatic cysts 10 years ago but was healthy before the appearance of bilateral polyarthritis of the knees 1 month before admission. She went to a nearby orthopedic clinic and reported temporary alleviation of the knee pain upon taking non-steroidal anti-inflammatory drugs (NSAIDs).
A physical examination showed mild swelling and tenderness in both ankles without limitation of range of motion. Laboratory studies showed elevated erythrocyte sedimentation rate (ESR, 39 mm/hr) and C-reactive protein level (CRP, 45.79 mg/L) (). White blood cell count was 8,600/L, blood hemoglobin was slightly low at 11.4 g/dL, and platelet count was 290,000/L. The serum angiotensin-converting enzyme (ACE) level was (29.0 IU/L; normal range, 7.7-29.4 IU/L). Aspartate aminotransferase (AST) and alanine transaminase (ALT) levels were slightly high at 37 IU/L and 60 IU/L, respectively. She was negative for rheumatoid factor and anti-cyclic citrullinated peptide (anti-CCP) antibody. Her C3 level was 144 mg/dL, C4 was 34 mg/dL, and antinuclear antibody was positive at a titer of 1:80 (homogeneous and fine cytoplasmic granules pattern), but specific antibodies were not detected ().
Knee, ankle, hip, and hand X-ray findings showed periarticular osteoporosis (). Ultrasonography findings suggested polyarticular inflammatory synovitis of both knees, elbows, ankles, with a combined partial tear of common extensor tendons in both elbows.
Chest X-ray findings showed focal mediastinal widening in both the hilum and the right upper trachea (), and chest computed tomography scan indicated multiple enlarged lymph nodes of the mediastinum and both hila (). The bone scan showed mildly increased uptake in bilateral hands, wrists, and other small joint areas ().
A mediastinoscopic lymph node biopsy was performed. The obtained tissues of mediastinal lymph nodes showed noncaseating granuloma of epithelioid cells and the presence of giant cells ().
Based on the bilateral hilar lymphadenopathy (BHL) with polyarthralgia, the patient was diagnosed with Lofgren's syndrome, although EN. NSAID treatment did not alleviate her symptoms for 1 week. Oral administration of prednisolone (10 mg/day) was initiated. The pain resolved within 1 week, and the levels of ESR and CRP were normalized after 2 weeks (12 mm/hr, 4.96 mg/L). The prednisolone dose was lowered 1 month later (5 mg/day). The pain and joint swelling were improved, but she sometimes felt mild pain in the joints, and BHL was still seen on simple chest radiography after 2 months. For these reasons, prednisolone administration has been continued for 8 months until now. No recurrence has been observed since then.


We present a case of a 13 year old boy with Loey's Dietz syndrome and a history of a David procedure (valve sparing aortic root replacement) when he was 9 months old with aortic valve and aortic root replacement at 2 years of age with subsequent post-operative complete heart block requiring pacemaker insertion. He ultimately underwent a Bentall procedure at 12 years of age. Upon routine cardiology clinic follow-up an incidental finding of a cystic structure in the liver was noted during echocardiography ().
He was asymptomatic from a cardiac and gastrointestinal standpoint. The remainder of his echocardiogram showed normal mechanical aortic valve function and normal left ventricular function. His pacemaker function was normal.
An abdominal CT was obtained to further characterize the lesion. Compared to a CT obtained 4years previously a new, well demarcated, heterogenously enhancing mass or aneurysm was identified between the lesser curvature of the stomach and the liver measuring 3.9 cm × 5.3 cm × 4.3 cm ( and ). A small branch off the left gastric artery appeared to enter the left anterior aspect of the mass ().
An abdominal ultrasound with Doppler performed after the CT scan was consistent with a patent aneurysm or pseudoaneurysm impressing upon the left hepatic lobe ( and ). Color Doppler flow into the aneurysm was demonstrated from the left gastric artery. No thrombus was identified.
Successful embolization of what was found to be a left gastric artery pseudoaneurysm () by interventional radiology was performed using 5 detachable GDC coils (Boston Scientific Corporation, Natick, MA) in the left gastric artery at the neck of the pseudoaneurysm ().
He was also noted to have a markedly ectatic superior mesenteric artery (SMA) with several irregular beaded second and third order vessels from the SMA thought to possibly represent manifestations of his disease. Two months following the coil embolization, an abdominal CT scan with contrast demonstrated the excluded large pseudoaneurysm with no enhancement noted within it. Nine months later, an abdominal ultrasound showed interval resolution of the embolized and thrombosed pseudoaneurysm without any residual abnormalities demonstrated in the area. There were no new aneurysms identified. He remains stable from a cardiac perspective as well.


A 13-year-old girl presented with a history of abdominal distension, back ache, and loss of appetite and weight of 3 months’ duration. Her medical history was otherwise unremarkable. On examination, an ill-defined mass of 10 cm × 8 cm size was palpable in the right hypochondrial region, extending 7 cm below the right costal margin and medially up to the umbilical region. Ultrasonography of the abdomen revealed a large, heterogeneous mass of 18 cm × 16 cm × 16 cm size arising from the right adrenal, infiltrating the right lobe of liver, and displacing the right kidney inferolaterally.
Fine-needle aspiration cytology (FNAC) of the adrenal mass showed clusters of malignant cells forming rosettes in an eosinophilic fibrillary background with high nuclear cytoplasmic ratio. In addition, scattered ganglion cells with eccentric-placed nuclei and prominent nucleoli were also present and features were consistent with ganglioneuroblastoma [].
The 24-h urinary vanillyl mandelic acid VMA level was 5.4 mg/24 h (normal is < 7 mg/24 h). Radiography of the lumbo-sacral spine showed mixed lytic and sclerotic lesion involving the L3 vertebrae [] and confirmed by computed tomography (CT) soft tissue with bone window []. To assess the extent of neuroblastoma and metastasis, PET-CT was done. It showed [Figures and ] increased uptake in the right adrenal and liver areas with metastatic involvement of L3 vertebrae. These imaging findings and FNAC reflected the diagnosis of ganglioneuroblastoma; stage IV (vertebral and liver metastasis). The patient was treated with intravenous zoledronic acid[] and carboplatin, vincristine, etoposide, and cyclophosphamide[] based chemotherapy regimen.


A 52-year-old woman presented with complaints of multiple asymptomatic skin-colored raised lesions on the trunk and extremities of 6 months duration, which were insidious in onset and progressive in nature, with the last lesion appearing 1 month back. Patient was a diagnosed case of Type 2 diabetes mellitus (DM) and rheumatoid arthritis (RA) since 2008, for which she was on regular treatment with tablet metformin 500 mg TDS, tablet glimipride 1 mg daily, tablet hydroxychloroquine 200 mg BD, tablet methotrexate 5-7.5 mg per week (total cumulative dose of methotrexate till now being 1300 mg), and tablet folic acid 5 mg taken daily except on the days of taking methotrexate. There was no history of photosensitivity.
Dermatological examination revealed the presence of multiple well-defined skin-colored to slightly erythematous papules coalescing to form well-defined plaques, few being annular in configuration, present on the flexor aspect of right forearm near the elbow joint, flexor and extensor aspects of left forearm near the elbow joint [], left upper arm, chest, and abdomen. The individual papules forming the plaques had elevated borders with depressed centers. The lesions were normoasthetic on examination. Rest of the general and systemic examinations were within normal limits.
The results of her complete blood count, erythrocyte sedimentation rate, liver and kidney function tests were within normal limits. Fasting blood sugar was 95 mg/dl and postprandial blood sugar was 124 mg/dl, which were essentially within normal limits. Histopathologic examination was suggestive of GA. There was mild thinning of the epidermis with dermis showing histiocytes arranged in interstitial pattern, presence of multinucleate giant cells, and mild perivascular lymphocytic infiltrate with no collagen necrosis [].
Based on the dermatological examination and histopathologic findings, the diagnosis of GA was made. The patient was treated with single dose of rifampicin 600 mg, ofloxacin 400 mg, and minocycline 100 mg (ROM) given once a month for 5 months. Patient tolerated the combination therapy well with no side effects. All the lesions subsided after 5 months of monthly ROM, leaving behind slight epidermal atrophy of skin at few sites []. There was no recurrence at 3 months of follow-up.


A 44-year-old woman had undergone radical hysterectomy and subsequent radiation therapy (50.4 Gy) for cervical cancer 8 years before this presentation. The final pathological diagnosis was MANEC, comprising small cells with a high nucleus-to-cytoplasm ratio growing in a trabecular pattern (fig. ). Although the patient had been treated with oral chemotherapy using tegafur-uracil, small bilateral pulmonary nodules appeared after 3 years. Percutaneous computed tomography (CT)-guided needle biopsy and histopathological examination were performed, showing neuroendocrine carcinoma (NEC). Pathological findings were similar to those of the previous cervical carcinoma, and immunostaining for thyroid transcription factor-1 (TTF-1) yielded negative results. TTF-1 expression is specific for the lungs, thyroid and brain and is used as a reliable marker for distinguishing between primary lung carcinoma and metastases within the lungs. Given these findings, metastatic lung cancer from the earlier cervical cancer was diagnosed. Some lesions in the lungs disappeared following combined chemotherapy using nedaplatin (CDGP) plus irinotecan hydrochloride (CPT-11). For some remaining lesions in the right lung, video-assisted partial lung resection had been performed. After surgery, she was treated with additional chemotherapy (CDGP + CPT-11, paclitaxel + carboplatin, docetaxel hydrate + cyclophosphamide) and followed up with (18F)fluoro-2-deoxy-D-glucose positron emission tomography/CT (PET/CT) every 6 months. A small number of new lesions in the left lung diagnosed on CT were resected with video-assisted thoracic surgery and pathologically confirmed as metastasis from cervical NEC. One year after this lung operation, the patient was referred to our department when PET/CT showed abnormal uptake in the pancreatic body (fig. , arrow) accompanied by slight back pain. Laboratory data on admission revealed no abnormalities in inflammation tests or tumor markers such as carcinoembryonic antigen, carbohydrate antigen (CA)19-9 and CA125. CT obtained in the arterial phase of enhancement showed a 20-mm faintly attenuating homogeneous round mass located in the pancreatic body (fig. , arrow). Endoscopic ultrasonography (EUS) revealed a well-circumscribed hypoechoic mass (fig. , arrow) in the pancreatic body and upstream pancreatic duct dilatation. Magnetic resonance cholangiopancreatography (MRCP) revealed stenosis of the main pancreatic duct in the pancreatic body (fig. , arrow) and dilated upstream pancreatic ducts. Brush cytology specimens seen under endoscopic retrograde pancreatography and repeated pancreatic juice cytology specimens collected through an endoscopic nasopancreatic drainage tube were negative. EUS-guided fine needle aspiration (EUS-FNA) was therefore performed, resulting in a diagnosis of NEC. As no extrapancreatic disease was detected on imaging, middle pancreatectomy was performed. Postoperative pathological examination showed atypical cells derived from small to large neuroendocrine cells with a high nucleus-to-cytoplasm ratio growing in a trabecular to solid and nodular pattern under hematoxylin and eosin (HE) staining, as were seen in the patient's cervical and lung tumors (fig. ). Rosette formation was also observed (fig. , arrow). High mitotic activity was seen, with 20 or more mitoses per 10 high-power fields (fig. ), along with positive immunostaining for synaptophysin (fig. ) and chromogranin A (fig. ) and negative results for TTF-1. The proliferative index indicated by MIB-1 was over 30% (fig. ). These results were compatible with secondary deposition from the cervical NEC. On the basis of the morphological features and results of immunohistochemical staining, metastatic pancreatic cancer from the cervical carcinoma was finally diagnosed. She has been followed up for 7 months since the surgery without any signs of local recurrence or other metastatic lesions.


In June 1997, a 52-year-old man without any medical history was referred to the department of gastroenterology because of gastric subepithelial tumor. Esophagogastroduodenoscopy (EGD) demonstrated 1 cm sized subepithelial tumor on the proximal antrum anterial wall (). Biopsies showed chronic gastritis only. He was admitted and diagnostic endoscopic mucosal resection was done (). The pathologic finding was diffuse aggregation of monomorphic lymphoid cells without any large cell or small cell component in the submucosal layer (). Immunohistochemical staining showed atypical lymphoid cells that were positive for pan B and lamda, but negative for pan T, bcl-2 and kappa. Ki-67 labeling index was 5%. These findings suggested his diagnosis was low-grade MALT lymphoma. Abdomen and pelvic computed tomography (CT), chest CT, bilateral bone marrow examination and F-18-fluorodeoxyglucose positron emission tomography (PET) were done and there were no involvement of disease. Although rapid urease test (RUT), serologic test for H. pylori, urea breath test (UBT) and H. pylori culture of gastric mucosa were all negative, he took triple regimen of H. pylori eradication including clarythromycin, amoxicillin, and proton-pump inhibitor (PPI) for 14 days. After the H. pylori eradication was completed, subtotal gastrectomy was done. In the pathologic review of surgical specimen, there were no residual tumor cells. We performed esophagogastroscopy, abdomen and pelvic CT and PET every 6 months for 2 years and then we performed the same examination every 12 months for 4 years. He had been followed without evidence of disease recurrence for 6 years, after which he stopped visiting our hospital. In May 2007, he was referred to our hospital again complaining of throat pain and neck mass. Neck CT showed multiple enlarged lymph nodes in bilateral cervical nodes (). The largest diameter of the involved lymph node was 6 cm. Excisional biopsy showed diffuse infiltration of large atypical lymphoid cells (), which were positive for CD 20, but not for CD 3, CD10, CD 56, ALK-1, or CD 21. Ki-67 labeling index was 90%. These findings suggested DLBCL. EGD and full staging evaluation showed no involvement of other site. He was diagnosed with stage IIA DLBCL and received three cycles of systemic chemotherapy including rituximab, cyclophospamide, doxorubicin, and vincristine (R-CHOP), followed by 3,600 cGy radiotherapy. He achieved and maintained complete remission until recent days.


In June 2005, a 55-year-old woman with hypothyroidism was referred to our department for epigastric discomfort from the department of endocrinology. EGD demonstrated mucosal surface granularity and 1 cm size mucosal elevation which had central depression on the lower body anterior wall (). Biopsy showed diffuse infiltration of atypical lymphocyte aggregation and glandular invasion of lymphocytes. Immunohistochemical staining showed atypical lymphoid cells that were positive for CD 20 but negative for CD 3, Ki-67 labeling index was 8%. Abdomen CT, chest CT, bilateral bone marrow examination, and PET were done for staging and there were no disease involvement at any other site. Pathologic diagnosis was low grade MALT lymphoma of the stomach at stage IE. RUT and UBT were positive. The patient took H. pylori eradication including clarythromycin, amoxicillin, and PPI for 14 days. Six months later, EGD was followed and confirmed complete histologic remission as well as endoscopic remission. RUT and UBT were all negative. In August 2006, 12 months after the first visit, EGD showed multiple erosions on the proximal antrum (). Biopsy showed recurred MALT lymphoma and H. pylori was also seen. RUT was positive, too. She took the second eradication of H. pylori infection with the same regimen during the same period of time. In November 2006, she visited our hospital complaining of palpable mass on both the lateral neck. Neck CT showed bilateral cervical lymph node enlargement (). Excisional biopsy showed diffuse infiltration of large atypical lymphoid cells. Immunohistochemical staining was positive for CD 20 and CD 79 but negative for CD 3. Ki-67 labeling index was 80%. These findings suggested DLBCL. In the staging work-up, there was no disease involvement except for both the neck nodes. EGD showed the second complete remission and RUT was negative. She was diagnosed with stage IIA DLBLC and took three cycles of R-CHOP followed by radiotherapy of 3,600 cGr. In April 2007, DLBCL and gastric MALT lymphoma regressed completely. RUT was negative. In December 2008, DLBCL and gastric MALT lymphoma remained in complete remission state and RUT was negative. Until recently she has maintained complete remission state of DLBCL and gastric MALT lymphoma and negative RUT.


A 35-year-old female presented with a nodular ulcerated swelling measuring 3 × 2.5 cm over the left lower leg since the past 5 years []. The nodular lesion was well-circumscribed, firm with surface ulceration. The patient attended Fine-needle aspiration cytology (FNAC) clinic with a clinical suspicion of malignancy. On physical examination, the lesion was not fixed to the underlying bone and no inguinal lymphadenopathy was detected.
Fine-needle aspiration was performed using a 22-gauge needle. Cytological smears were cellular, showing clusters of basaloid cells having round to oval nuclei, nucleated squamous cells, and anucleated squames. Few fragments of shadow cells adherent to basaloid cells were also noted [Figure and ]. A cytological diagnosis of pilomatrixoma was made.
Excision biopsy and histopathologic examination of the resected specimen showed typical features of pilomatrixoma with basaloid cells in the periphery, shadow cells toward the center, and a few foreign body-type giant cells [Figure and ].


A 55-year-old man presented for an elective laparoscopic Roux en Y gastric bypass for super obesity. His previous medical history included insulin dependent diabetes, hypertension, obstructive sleep apnoea, intervertebral disc disease and ischaemic heart disease. His admission body mass index (BMI) was 50 and this represented an improvement from an original BMI of >65 at clinic. Following an uncomplicated intraoperative procedure he developed severe lower abdominal pain on the first postoperative day.
Over a couple hours the pain became centralized and was relieved by lying prone. He became gradually distended and complained of being unable to pass urine but had no other abdominal symptoms. On examination he had a pulse of 120 beats/min and a blood pressure of 145/80 mmHg. He had a respiratory rate of 25 breaths/min and was saturating at 94% on 2 L O2 (via nasal cannulae). Clinical examination of his abdomen revealed that he was not obstructed and had no signs of peritonitis. However, a large supra-pubic mass was palpable. He had a large residual volume of urine (>800 mL) that was not amenable to urethral catheterization necessitating the involvement of a urological team to insert a supra-pubic catheter.
He continued to experience worsening abdominal pain throughout the second postoperative day. His abdomen became more distended causing him to become short of breath thus increasing his oxygen support (i.e. >4 L O2 via a face mask). An arterial blood gas demonstrated a developing lactic acidosis (i.e. >3.0) and a high leukocytosis (>18,000/mm3). Computed tomography (CT) chest and abdomen confirmed consolidation and a pleural effusion but no obvious abdominal pathology. This excluded the original clinical suspicion of an anastomotic leak (Figure ). Ultrasound (USS) was not performed.
Due to inconclusive imaging and the lack of a definitive diagnosis he was taken back to theatre for a mandatory re-laparoscopy. This revealed a non-specific fibrinous coating over the small bowel. On deeper inspection the anastomosis appeared intact and healthy (no anastomotic air leak was demonstrated). However, a large segment of necrotic omentum was found and subsequently divided and removed. No other abdominal pathology was identified. Pathological examination revealed vascular congestion and omental necrosis. The patient made a good recovery and was discharged home a couple weeks later.


A 33-year-old gentleman, diagnosed to have neurofibromatosis type 1 at 14 years of age, presented to us with a history of low back ache for 3 years associated with headache difficulty in walking, urinary urgency, and constipation for 6 months. On examination his height was 149 cm, weight 56 kg, BMI 25.2 kg/m2, head circumference 55.4 cm, pulse 80/minutes, blood pressure 130/90-mm of Hg, and no postural drop in blood pressure. He had multiple neurofibromas with numerous café au lait spots with smooth edges all over the body the largest of which was present over the right leg measuring 15 × 10 cm, axillary freckling was noted bilaterally. He had scoliosis of the spine. His ophthalmic examination revealed lisch nodules with bilateral ptosis. Neurological examination revealed mild spastic quadriperesis with brisk deep tendon reflexes and equivocal plantars. Screening MRI scan of the spine cord was however did not reveal any compressive lesions but it revealed a right suprarenal mass measuring 28 × 20 mm and also a hyperintense lesion measuring 21 × 14 mm, posterior to the left lobe of thyroid. He had biochemical evidence of hypercalcemia (calcium 12 mg/dl; albumin 4.5 g/dl) hyperparathyroidism (PTH 741 pg/unit). Left inferior parathyroid adenoma was confirmed by an ultrasound, parathyroid scintigraphy [], and SPECT-CT []. His 24-hour urinary metanephrine level was 356 mcg (normal range <350 mcg/24 hours) and normetanephrine level was 937 mcg (normal range <600 mcg/24 hours). Right adrenal pheochromocytoma was confirmed on the CT scan [] and MIBG scan. This patient had features of neurofibromatosis type I (NF-1) with a left inferior parathyroid adenoma and a right adrenal pheochromocytoma. After adequate preparation he was taken up for focused parathyroidectomy with excision of left inferior parathyroid adenoma and right laparoscopic adrenalectomy. The postoperative period was uneventful. Histopathology confirmed pheochromocytoma of the right adrenal gland and a left parathyroid neoplasm with atypical features like nuclear atypia and capsular/vascular invasion, []. Immunohistochemistry of the tumor showed a low MIB-1 index [].


A 66-year-old male, known diabetic for last 1 year was hospitalized with pain in right upper quadrant of abdomen for 20 days and breathlessness at rest for 5 days. No written reports of blood sugar levels were available with patient at the time of admission. On verbal enquiry, he reported that blood sugars were always below 140 mg%. His blood sugars were well-controlled on medical nutrition therapy (diet with low glycemic index). He had never taken any anti-diabetic drugs. During this admission, blood investigations revealed fasting blood sugar of 142 g/dl, white blood cell count of 12700/c.mm, platelets of 48000 lacs/c.mm. Renal profile showed blood urea nitrogen of 33.4 mg/dl, creatinine of 1.7 mg/dl, sodium of 129 meq/l, and potassium of 3.9 meq/l. Liver function tests revealed total bilirubin level of 6.6 mg/dl, direct bilirubin level of 5.8 mg/dl, alanine amino-transferase level of 75 u/l, aspartate amino-transferase level of 103 u/l, alkaline phosphatase level of 202 u/l, total protein level of 5.8 g/dl and albumin level of 2.9 g/dl. Hepatitis B surface Antigen (HBsAg) and anti Hepatitis C virus (HCV) were negative. Chest X-ray revealed right mid zone pneumonia and ultrasound abdomen was normal. Urine routine revealed 10-12 pus cells/high power field and later urine culture grew Escherichia coli. He was empirically started on i.v. moxifloxacin (400 mg) at admission and was managed symptomatically. Twenty four hours later i.v. cefepime/tazobactum combination was also added based on urine culture sensitivity report. After 16 h of hospitalization patient developed tremors, sweating and was found to have blood glucose level of 58 mg/dl. He was administered 10% dextrose intravenously, which relieved his symptoms. The patient had repeated episodes of hypoglycemia for subsequent 3 days (blood glucose levels 77, 44, 54 mg/dl) despite regular administration of intravenous 10% dextrose. Moxifloxacin was discontinued after 3 days, after it was suspected to cause the hypoglycemia. After stopping moxifloxacin, his blood sugar levels increased after 24 h and he developed hyperglycemia (blood sugars-273, 324, 298 mg/dl) and required insulin treatment. The patient was discharged on recovery after 10 days of hospitalization. At 1 month follow-up, his liver function tests and kidney function tests were normal. He continued to require insulin for glycemic control even 1 month after his discharge. His last blood sugars level was 115 mg% with insulin.


A 45-years-old male patient came to the Department of Endodontics with a chief complaint of swelling in the upper front teeth region (teeth #7 and #8). The patient had no medical contraindication to dental treatment. Dental history revealed an incident of trauma to the upper front teeth region 5 years ago. Clinical examination revealed discolored tooth #8 with an Ellis class 2 fracture. Both the teeth #7 and #8 were sensitive to percussion test. Upon radiographic examination gigantic periapical radiolucencies were observed at the apical region of teeth #7 and #8 [Figures and ].
The root canal treatment was performed using step back technique till an apical size of # 55 and #45 in relation to teeth #8 and #7 respectively. 5.25% sodium hypochlorite solution (Novo Dental Product Pvt Ltd, Mumbai, India) was used to irrigate the canals during the canal preparation. The root canal treatment was performed in three visits and calcium hydroxide was used as the intracanal medicament. The root canals were obturated using gutta percha (Dentsply maillefer Ballaigues) and AH 26 (Dentsply DeTrey GmbH, Philadelphia, USA) by the lateral condensation technique. After a review period of 6 months, expected healing did not occur. Hence, a periapical endodontic surgery was planned. Under local anesthesia (1:200000 adrenaline, DJ Lab, India), a full thickness mucoperiosteal flap was reflected by a sulcular incision starting from the distal of the tooth #9 to distal of the tooth #6. A large periapical defect was seen with complete loss of labial cortical plate. The lesion measured 15 mm × 16 mm × 16 mm corresponding to the length, width, and depth of the lesion []. Tissue curettage was done at the defect site followed by thorough irrigation using sterile saline solution. Using #702 tapered fissure bur (SS White burs), root end resection was performed in teeth #7 and #8 and Grey mineral trioxide aggregate (MTA) (ProRoot MTA; Dentsply, Tulsa, OK, USA) was used as the root end filling material. 20 mL of blood was drawn from the patient's antecubital vein and centrifuged (REMI centrifuge machine Model R-8c with 12 × 15 mL swing out head) for 10 min under 3000 revolutions (approximately 400 g) per minute to obtain the PRF. Commercially available HA bone graft crystals (Biograft HA, IFGL Bioceramics Ltd., India) were sprinkled over the PRF gel and together the mixture was placed into defect site []. PRF membrane was prepared with compresses and placed as two layers covering the edge of the defect []. Flap stabilization was done followed by suturing using 3-0 black silk suture material (Sutures India Pvt. Ltd, Karnataka, India). Patient was kept under the antibiotic (amoxycillin) coverage along with Affen PLUS Tab (Dr. Reddy's Lab, Andrapradesh, India) and 0.2% chlorhexidine gluconate solution as mouth rinse for a period of 5 days. Suture removal was done 1 week later and the healing was uneventful [].
Patient was reviewed at 3 months [], 6 months [], 1 year [], and 2-year period during which there were no symptoms of pain, inflammation, or discomfort. These follow-up visits included routine intraoral examinations and professional plaque control. Radiographically, HA particles have been almost completely resorbed and replaced with new bone at the end of 2 years []. Patient was completely satisfied with the results of the treatment.


A 24-year-old male reported to the department of conservative dentistry and endodontics with the chief complaint of pain in right lower back tooth region. On intra-oral examination, the patient had multiple carious lesions and multiple missing teeth with retained deciduous teeth. Intraoral periapical radiographs were done for mandibular molars and left maxillary molar. Single rooted morphology of molars was observed on the intraoral periapical radiographs. OPG was taken after the completion of root canal treatment. The OPG depicted an interesting picture []. It revealed altered morphology of all permanent teeth and numerous missing teeth. Realizing the importance of the findings of this case, a computed tomograph for upper and lower jaw was advised. Since only high resolution computer tomography (HRCT) facility was available at the university hospital, HRCT was undertaken [] and the findings of HRCT confirmed the diagnosis. HRCT and OPG images suggested that right maxillary lateral incisor, second premolars, and third molars were congenitally missing. Deciduous second molars were retained and showed normal anatomy and all permanent teeth had single roots [Figures and ].
Since hypodontia along with altered morphology of teeth was observed, thorough medical examination was advised. The medical examination and checkup revealed no contributory finding. So it was concluded that there was no association with any medical condition and could not be kept under the category of any known syndrome. The patient is a single child and oral examination of his parents revealed nothing significant. So a familial correlation, at least for now, could not be established.
The importance of this case lies in the fact that all permanent teeth were single canalled including all molars, which is a rare finding.[] Also the condition presented with no additional anomaly other than dental, so could not be associated with any syndrome.


A 66-year-old female, 5 days post-elective total knee replacement was noted on clinical exam to have a pulsatile swelling in the popliteal fossa. Angiography confirmed the presence of a 1.9 cm pseudoaneurysm arising from the proximal left tibioperoneal trunk, with a 3.3 mm neck []. Under real-time ultrasound (US) guidance and using sterile techniques, a 20 gauge 2.75 inch needle was introduced percutaneously into the pseudoaneurysm cavity and 1.5 ml of a mixture of 5,000 US units of thrombin and 200 mg of microfibrillar bovine collagen (D-Stat flowable Hemostat, Vascular Solutions Inc., Minneapolis, Minnesota) was injected []. This resulted in complete occlusion of the pseudoaneurysm within seconds which was confirmed via US. A further check US was performed on day 6, post-operatively, which once again confirmed no flow within the thrombosed pseudoanuerysm cavity [].
Arterial complications post-total knee arthroplasty (TKR) are a rare but recognized entity. In a large single-center study incorporating 13618 TKR's, 0.17% of the cases had arterial complications.[] Pseudoaneurysms are much rarer, with fewer than 20 cases reported in the literature. The proximity of the popliteal artery to the operative site during TKR exposes it to potential injury. Parvizi et al. found that indirect trauma, such as vessel stretching during dislocation of the knee joint was responsible for the majority of vessel injuries rather than direct arterial trauma.[] Techniques previously described in the literature for management of pseudoaneurysms post-TKR included percutaneous bland thrombin injection, endovascular stenting, endovascular coiling, and open surgical ligation. This is the first reported case of a popliteal pseudoaneurysm managed with a combined thrombin-collagen percutaneous injection (D-Stat Flowable®). This product was designed to accelerate the blood clotting process by facilitating conversion of fibrinogen to fibrin. It is approved for management and control of bleeding from vascular access sites, percutaneous drains, and tissue tracts and has been used successfully in the past to percutaneously occlude a giant splenic artery pseudoaneurysm.[] In our experience, the high viscosity of this preparation allows greater control of the injection, potentially decreasing the risk of intra-arterial thrombotic events which have complicated a very small proportion of thrombin injections.[] The avoidance of arterial access such as is required for endovascular stenting and coiling decreases the risk of concurrent arterial access site injury. Furthermore, many authors discourage the use of covered stents across mobile joints due to high occlusion rates (up to 21%) at 24 months.[] As the patency rates of stents are improving as newer generation stents are developed, there is the ongoing requirement for patients to be medicated with aspirin and/or clopidogrel, both of which carry their own inherent risks. Lastly, with this technique, the general anesthetic required for surgical repair is also avoided and, consequently the associated complications.
It is important to note that this compound is not routinely used for pseudoaneurysm management and even though our own limited experience with this product has been favorable, randomized trials comparing it with other treatments are required before it becomes an established therapeutic strategy. In practice, it is important to aim the needle away from the neck of the pseudoaneurysm when injecting the drug and to only inject small amounts at a time as we have found that the “snow storm” effect [] can limit visualization of the pseudoaneursym neck. A final note of caution is the possible development of factor V deficiency secondary to the development of bovine thrombin-induced antibodies which can cross-react with human factor V. This risk is also seen in the use of bland thrombin.


A 30-year-old woman complained of redness and pimples on the nose for 6 months. She had
an infiltrated erythematous plaque with papules, pustules and telangiectasia all over
the nasal area (). Histopathology showed
superficial and deep tuberculoid inflammatory infiltrate without caseous necrosis,
consistent with granulomatous rosacea. Fungi and acid fast-bacilli resistant staining
negative.
The disease progressed despite the rosacea treatment. The plaque became less defined and
larger (). There were four new
infiltrated erythematous-brownish sarcoidosis-like plaques on the left and right
eyebrows, on the right temporal region and on the left jaw ( and )
The investigation was extended to possible granulomatous infectious diseases. Chest
X-ray was normal, PB serology, syphilis serology, Montenegro reaction and tuberculin
test (PPD) were non-reactive. The histology report was sustained after new biopsies
().
After l year of follow-up, the patient complained of throat pain. In the oral cavity
there was a slightly verrucous lesion with fine granulation tissue and bleeding points
similar to strawberry-like stomatitis ().
In the middle of mixed inflammatory infiltrate there were some thick-walled spores with
double contour and multiple budding, compatible with PB ( and ). After treatment, the
patient achieved clinical cure.


A 33-year-old male patient resident of Manguinhos - Rio de Janeiro - Brazil was diagnosed 18 months ago with polymyositis and was irregularly taking methotrexate (15mg/weak) with prednisone (40 mg/day); however, he failed to attend medical follow-up visits regularly. One year ago, he was hospitalized with pulmonary tuberculosis. He was prescribed rifampicin, isoniazid and pyrazinamide and completed a six-month treatment period.
Six months later, he was admitted at the Hospital with painful warm nodules of various sizes on his body in addition to gummatous lesions developed two months earlier (, and ). He presented intermittent fever which responded to metamizole. There was throbbing pain in his limbs, aggravated by movement and alleviated by resting. He also complained of muscle weakness in pectoral and pelvic girdle.
At admission, he had tachypnea, tachycardia and fever. Lymphadenopathy was absent and the blood pressure was normal. The laboratory tests showed leukocytosis (21,100 leukocytes), thrombocytosis (573,000 platelets) and normocytic normochromic anemia. Lactate dehydrogenase (LDH) levels were 515 U/I and creatine phosphokinase (CPK) 282 IU/l. Chest x-ray revealed no abnormalities. During hospitalization, leukocytosis, thrombocytosis and normocytic normochromic anemia persisted, as well as increased LDH levels. Creatine phosphokinase returned to normal levels.
The differential diagnosis of the nodular disease mainly considered myositis and vasculitis, methotrexate-related panniculitis, Weber-Christian disease, subcutaneous panniculitis-like T-cell lymphoma,
erythema nodosum, bacterial, fungal and mycobacterial infections.
At admission antibiotic therapy with oxacillin was initiated against infection by Staphylococcus. However, no specimen has confirmed the suspicion of diagnosis. These results hampered the investigation for presence of
staphylococci bacteria
and made the decision to maintain or not the antibiotics difficult. The patient's fever and skin rash persisted despite the therapy. On the seventh day of treatment, the antibiotic was changed to vancomycin at 500 mg qid intravenously for 14 days; no improvement occurred in the patient's clinical condition.
The material collected from the lesions was studied for histopathology patterns, bacterioscopy and culture studies.
Ultrasound confirmed a subcutaneous structural alteration on the posterior region of the upper third of the right leg, with 8 cm in size and without liquid. The possibility of purulent material being accumulated inside the lesion could not be discarded.
Skin histopathology studies revealed acanthosis, edema of the reticular dermis, and mild infiltrate of lymphocytes with some neutrophils around vessels and skin adnexa. Panniculitis was suggested by the presence of subcutaneous necrosis of adipocytes, deposits of fibrinoid material and hyaline eosinophilic strands. Initially, aspirated secretion from the lesions was negative for acid-fast bacilli.
One week later, a new sample showed acid-fast bacilli (+ + +). Rifampicin 600 mg qd, isoniazide 400 mg qd, pyrazinamide 2 g qd and ethambutol 1.2 g qd were initiated. Within a five-day treatment period the patient improved considerably, the fever disappeared and he was discharged. Culture studies revealed growth of
Mycobacterium tuberculosis.
Lesions were totally cured after 6 months.


A local newspaper wrote, “According to the owner of the circus, a lion became excited by the noise of the audience during the evening show, escaped from its unlocked cage and attacked an eight-year-old boy. The victim was dragged about twenty feet. The four-year-old lion was called ‘Bang-Bang’ and weighed about 200 kg” []. The patient was sent to a rural hospital (approximately 150 km from the capital city) and was transferred to our hospital almost 5 hours after the attack.
Upon arrival to the emergency room, the patient was in poor condition: Extremely pale, no arterial pressure, semiobstructed airway, breathing difficulty, and unstable vital parameters (heart rate, 170 beats per minute; respiratory rate, 34 breaths per minute; Glasgow Coma Scale (GCS), 5/15 points; and hemoglobin, 5.5 g/dL). Orotracheal intubation was performed immediately.
The results of the clinical examination were as follows:
Head and neck: Multiple scalp wounds, with profuse bleeding; extensive bilateral fronto-parieto-temporal injury with brain matter escaping through perforations caused by the bite; right ocular globe with hyperemia, enlarged right pupil (8 mm), and upper and lower eyelid edema; wound at level of left orbit; and bleeding neck wound Chest: Open wound on left side, measuring approximately 15 cm; fractures of the fourth, fifth, and sixth costal arches; muscle, pericardium, and mediastinum exposed; dullness upon percussion; decreased breathing sounds on left side; and collapsed left lung Abdomen: Open wound in the left infraumbilical region, measuring 9 cm, with irregular edges; reduced bowel sounds; and abdomen taut upon palpation Extremities: No injuries.
He received 2 L of normal saline and 2 U of whole blood through two large bore peripheral intravenous line and vitamin K, improving his vital signs. In the initial cranial computed tomography (CT) scan, severe trauma was predominantly seen to the left of the skull, including dislocated fractures. Intracranial injuries with areas of intracerebral contusion and bleeding as well as subdural air accumulation in the areas where the lion's canines penetrated the skull were found [].
The child was sent directly to the surgical ward, received tetanus toxoid and then postexposure rabies prophylaxis, and was assisted by the medical team (pediatric surgeon, neurosurgeon, and cardiovascular surgeon).
The following procedures were performed:
Head: Surgical cleaning and detailed washing with normal saline and povidone iodine were performed throughout the extensive scalp injury, followed by the control of bleeding. Multifragment fracture of the left parietal bone, with depression and exposure of the brain matter through orifices situated at the bifrontal, biparietal, and occipital levels was found []. Small bone fragments were removed from the left parietal region, along with protruding injured extradural brain tissue. Only hemostasis was performed due to the hemodynamic instability with hypovolemic shock Chest: Surgical cleaning of thorax and thoracotomy, evidencing three rib fractures, no injuries to the lungs, pericardium, or diaphragm; chest tube left connected to water-sealed flask; left chest tube returned 400 mL of fresh red blood Abdomen: Median, supraumbilical, and infraumbilical laparotomy, revealing abundant free-moving milky fluid in abdominal cavity and swollen intestinal rings; cavity inventory performed, revealing absence of visceral injuries; laparotomy left contained with bag and compresses.
The patient was admitted to the intensive care unit under mechanical ventilation with sedation and analgesia (heart rate, 115 beats per minute; blood pressure, 100/70 mmHg; GCS, 4/15 points) []. Cefotaxime, cloxacillin, metronidazole, 20% mannitol, and phenytoin were initiated parenterally. The ophthalmological evaluation discarded injury to the left ocular globe.
On the fourth postoperative day (GCS, 6/15 points; left brachiocrural hemiparesis, 3/5), the patient progressed with fever and right basal pneumonia. New antibiotic therapy was instituted (ceftazidime, vancomycin, and metronidazole), followed by the closure of abdominal cavity.
On the sixth postoperative day (GCS, 10/15 points), mechanical ventilation and chest drainage were removed and 20% mannitol was suspended. Blood derivative products (packed red blood cells and fresh frozen plasma) were used because of low prothrombin time (52%) and thrombocytopenia.
On the ninth postoperative day (GCS, 12/15 points), Acinetobacter baumannii and Pseudomona aeruginosa were isolated from sputum/tracheal secretions. The patient was sensitive to imipenem and amikacin; hence, the consequent change of antibiotic. An occurrence of cerebrospinal fluid fistula in the left parietal region was found.
On the twelfth postoperative day, in the operating room, the correction of occipital depressed fracture and hermetic closure of the dura defect in the parietal region, with free autologous periosteal graft, was performed using interrupted 3-0 absorbable sutures.
On the fifteenth postoperative day (GCS, 14/15 points), the child was discharged from the intensive care unit. During his hospital stay, he presented behavioral disorders (obsessive–compulsive disorder and panic attacks) and was treated with clomipramine and clonazepam. Seven months later, he developed amblyopia without other neurological deficit.


A 61-year-old woman with a long-standing history of thyroid disease presented with focal neck swelling of around 4-5 cm. She had a background history of hypertension. The mass was mobile with tongue protrusion. Computed tomography scan of the neck showed a heterogeneous thyroid mass with multiple cystic areas and multiple foci of calcification []. Fine needle aspiration cytology of the lesion yielded papillary carcinoma. There were no clinical features of airway compression. Total thyroidectomy was planned by the otolaryngology surgeon. The patient was clinically and biochemically euthyroid prior to surgery.
Fiberoptic bronchoscopy was scheduled to rule out tracheal invasion before surgery. The patient was induced with inhaled sevoflurane, intravenous propofol and remifentanil. The LMA Classic Excel™ was inserted and hand ventilation was assessed which was found to be adequate. Spontaneous breathing was allowed on the supraglottic airway device. Bronchoscopic assessment of trachea was performed through 15 mm fiberoptic bronchoscope swivel connector (Smith Medical International Ltd, UK) under general anesthesia. After the completion of the bronchoscopy, the patient was paralyzed and the trachea intubated with a 7.0 mm ID endotracheal tube over a fibreoptic bronchoscope, using the LMA Classic Excel™ as a conduit. shows the view of the laryngeal inlet and epiglottis elevating bar through the LMA Classic Excel™. The surgeons proceeded with surgery uneventfully. En-bloc dissection and removal of the thyroid gland was done with excision of the strap muscles and the first tracheal cartilage which was adherent to the tumor was done. The trachea was successfully extubated at the end of the surgery.


A 3-year-old child weighing 9kg diagnosed with Morquio-Brailsford syndrome was posted for MRI and CT of cervical spine after she had developed sudden weakness of the upper and lower limbs with inability to walk. She was diagnosed as Morquio syndrome at the age of 1 year, with a delay in achieving developmental milestones. She had short neck, pectus carinatum thoracolumbar kyphoscoliosis with a suspected unstable cervical spine based on clinical deterioration and cervical spine X-ray []. Cardiovascular, abdominal, and hematological examinations were unremarkable. Airway assessment could not be done as the child was uncooperative. A high-risk informed consent was taken in view of difficult airway and possible cervical instability which could worsen cord compression during the procedure.
Intubation was done inside the operation theatre (OT) in presence of senior anesthesiologists and then shifted to the MRI suite. Different sizes of endotracheal tubes (ETTs), laryngeal mask airways (LMAs), proseal LMA, and pediatric fibreoptic bronchoscope were kept in the difficult airway cart. An intravenous (IV) access was secured and child was shifted to OT after administration of 0.1mg IV glycopyrrolate and 0.5mg IV midazolam. Baseline vitals were heart rate of 150/min, blood pressure 94/60mmHg and oxygen saturation (SpO2) of 99%. Anesthesia was induced with titrating doses of propofol along with 3% sevoflurane in 100% oxygen; ensuring spontaneous ventilation was maintained while monitoring SpO2 and end tidal carbon dioxide (EtCO2). A total of 10μg IV fentanyl was given and after adequate depth of anesthesia, a size 2 classic LMA which was shaped like a proseal LMA using stylet was gently inserted with manual in-line stabilization (MILS) []. LMA position was confirmed by adequate chest expansion and presence of capnogram. A right-angle tracheal tube connector with seal was attached to the LMA []. This allowed fibreoptic bronchoscope to be inserted through the LMA while the child was ventilated with 100% oxygen and 2%-3% sevoflurane.
A 4.0 mm uncuffed portex ETT was railroaded over the 2.4 mm Outer Diameter (OD) pediatric bronchoscope. Then the bronchoscope was introduced through the lumen of LMA into the trachea and the ETT was advanced over the bronchoscope. On connecting the breathing circuit to the ETT, though a typical capnogram was present but visualization through the fibreoptic scope showed that the ETT tip was just above the glottis, even on introducing the ETT to the maximum depth possible in the presence of right-angle tracheal tube connector. Eventually, the right-angle tracheal tube connector was removed and reintubation was done using similar technique, now ensuring that the tip of ETT was placed about 2cm above carina by fibreoptic visualization.
The ETT with LMA was fixed as a single unit after confirming the ETT tip position by visualization and auscultation. Care was taken to avoid kinking of the ETT at the point of exit from LMA. Throughout the procedure, saturation was maintained between 96% and 100%. A total of 3mg IV atracurium was then given and child was shifted to MRI suite on oxygen trolley with monitors. Anesthesia during the MRI and CT was uneventful, after which child was shifted to pediatric intensive care unit and was extubated in ICU. MRI showed cord compression involving the cervicomedullary junction, hypoplastic split atlas, and posterior atlantooccipital fusion []. CT confirmed hypoplasia of C1 vertebra, split atlas, and nonossification of the dens []. Child was then put on a cervical collar and eventually discharged.


Nineteen-year-old female was diagnosed as idiopathic bilateral bronchiectasis and developed end stage lung disease and respiratory failure. She was referred to King Faisal Specialist Hospital & Research Center in June 2012 for further assessment and possible LTx. During her routine pre-transplant work up, esophageal HRM 24-hour pH impedance monitoring and modified barium swallow were performed to rule out any major esophageal dysmotility or significant GERD, which could predispose her to recurrent aspiration pneumonia and pseudomonas colonization. There was no significant past history of heartburn, regurgitation, chest pain or dysphagia before the LTx. HRM study was performed with a 4.2 mm outer diameter solid state assembly with 36 circumferential sensors spaced at 1-cm intervals (Given Imaging, Los Angeles, CA, USA). Esophageal pressure topography data was analyzed using ManoView™ analysis software. Her pre-transplant, HRM parameters were within normal limits (). Ambulatory impedance pH monitoring study was performed before the LTx, after an overnight fast (off proton pump inhibitors [PPI] for 1 week) on an out-patient basis, using a ZepHr system (Sandhill Scientific, Inc., Highland Ranch, CO, USA), which was equipped with a catheter with 6 impedance electrodes and 2 pH electrodes (first at distal esophagus and second at just below the upper esophageal sphincter). Both the impedance and pH signals were recorded at 50 Hz on a 128 MB compact flash (SanDisk, Milpitas, CA, USA). No significant gastro-esophageal reflux was reported in pre-transplant 24-hour impedance pH evaluation ().
She underwent bilateral sequential LTx in October-2012 with no major post-operative complication and discharged after 2 weeks in stable condition on immunosuppressant drugs (mycophenolate mofetil, tacrolimus and prednisolone). She was re-admitted 4 weeks later, when she developed aspiration pneumonia with additional complaints of dysphagia, regurgitation and heartburn. Her second hospital course was again uneventful; she received parenteral antibiotics, PPI and discharged in satisfactory condition. It was suspected that her aspiration pneumonia might have been triggered by GERD secondary to esophageal dysmotility. She was advised to continue her immunosuppressant and standard chemoprophylaxis with valganciclovir, bactrim and itraconazole, in addition to esomeprazole 20 mg twice daily to suppress the symptoms of GERD. Azithromycin 3 times weekly was added as anti-inflammatory agent. The patient's HRM study was repeated 8 weeks after LTx, and her detail HRM datasets were reviewed according to the current Chicago classification. It showed typical spastic hypercontractile contractions (the greatest DCI = 12,437 mmHg · cm · sec) consistent with the diagnosis of Jackhammer esophagus. Surprisingly, her last esophageal HRM returned to normal at 12 weeks after LTx, as shown in the study of esophageal pressure topography with conventional line tracings () without use of any muscle relaxant or sedatives. The data included in the HRM study was LES resting pressure, distal latency, integrated residual pressure, DCI and distal esophageal amplitude at 3, 7 and 11 cm above LES and contractile front velocity before LTx, 4 and 12 weeks after LTx as shown in .
The 24-hour pH impedance monitoring study was repeated again on PPI after 8 weeks of LTx (because she was symptomatic on PPI) which was consistent with significant GERD. Gastric emptying study also after the LTx showed severely delayed gastric emptying for solid meal (97% retention at 120 minutes with stagnation of food at 4 hours delayed images) consistent with gastroparesis. Post LTx barium swallow and upper gastrointestinal endoscopy was normal. The impedance pH study (on PPI) was repeated 12 weeks after LTx and surprisingly, no significant abnormality was seen ().


A 69-year-old woman was admitted to our hospital because of weight loss and progressively worsening dyspnoea. Her previous medical history was unremarkable. She was admitted to the internal medicine department. A cardiologist was consulted because of atrial fibrillation with rapid ventricular response and an enlarged cardiac silhouette on her chest X-ray. On physical examination her blood pressure was 120/70 mmHg with a heart rate of 120 beats/min. She had engorged jugular veins and an enlarged liver without peripheral oedema. Her ECG showed atrial fibrillation and low voltage. No Q waves were noted. An echocardiogram showed a large pericardial effusion of more than 30 mm and a swinging heart (Fig. ). The left ventricle was small and hyperdynamic without wall motion abnormalities. There was no collapse of the right ventricle. The inferior vena cava was dilated without inspirational collapse. Because of imminent cardiac tamponade a pericardiocentesis was performed with the immediate evacuation of 800 cc of haemorrhagic pericardial fluid. Six hours after this procedure control echocardiography was performed which showed only a small amount of pericardial fluid, but now a poor left ventricular function with general hypokinesia and anterior and septal akinesia. T-wave inversion and Q waves were seen on the ECG in the anterolateral leads (Fig. ) but the cardiac markers were not elevated. She was treated with ACE inhibitors, diuretics and low-dose beta blockade. Analysis of the pericardial fluid showed malignant cells and on a chest CT scan a mass was detected. Subsequently, she was diagnosed with small cell lung carcinoma (SCLC) stage IV with metastasis to the pelvis, pericardium and brain and treated with radiation therapy. She was discharged and returned several months later with complaints of fatigue and progressive dyspnoea. Echocardiography again showed a large pericardial effusion; the left ventricle now had a normal systolic function without wall motion abnormalities. The ECG now showed normal R progression in the anterior leads with disappearance of Q waves (Fig. ). She was again treated with pericardiocentesis and was discharged the following day. After this episode she died at home as a consequence of her progressive lung carcinoma.


A 60-year-old postmenopausal woman presented to the outpatient department with symptoms of headache and occasional dizziness for the last 2 months with one episode of generalized tonic clonic seizure 2 days back. The patient was of poor health status with mild pallor. The patient, on enquiry, also complained about a lump in her left breast for the last six months. There was no history of nipple discharge. On examination, a firm 6 × 4 cm swelling was palpated in the upper outer quadrant of the left breast. The swelling was non-tender and mobile with fixity to the overlying skin but not with the chest wall. There was no axillary lymphadenopathy; and the other breast was found normal on clinical examination. Clinical examination and history did not reveal any other abnormality. The patient underwent laboratory and imaging studies with fine needle aspiration of the breast lump. CT scan showed a heterogeneously enhancing hypodense infratentorial lesion measuring 6.3 × 5.7 cm in left cerebellar hemisphere and vermis compressing and displacing the fourth ventricle []. Laboratory tests showed hemoglobin of 9.8 gm/dl with microcytic, hypochromic anemia. The chest X-ray was normal. During FNAC, two passes were made. The smears were stained with Papanicolaou and May-Grunwald-Giemsa (MGG) stain and showed a dispersed population of malignant pleomorphic, sometimes bizarre, spindle cells and few multinucleated tumor giant cells. There was no epithelial component in the prepared smears. The background was hemorrhagic []. A cytologic diagnosis of malignant sarcomatoid spindle cell tumor was made. The differential diagnoses suggested were undifferentiated high grade pleomorphic sarcoma, malignant phyllodes tumor and metaplastic carcinoma (spindle/sarcomatoid variant). The patient underwent wide excision of the tumor. No axillary dissection was done. The histopathologic examination showed a high grade sarcoma composed of pleomorphic spindle cells arranged in fascicles and vague storiform pattern. There were bizarre and multinucleated tumor giant cells. The mitotic count was 15/10 HPF with atypical forms. There were foci of dystrophic calcification []. Immunohistochemistry for Cytokeratin (CK), Epithelial Membrane Antigen (EMA), Smooth Muscle Actin (SMA), desmin and S-100 protein were performed with adequate controls and showed no immunoreactivity indicating a lack of differentiation. A stereotactic biopsy from the cerebellar lesion showed similar histological and immunohistochemical pattern. A diagnosis of primary high grade undifferentiated pleomorphic sarcoma of breast with cerebellar metastasis was made. The patient in the post-operative period deteriorated quickly and developed progressive ataxia and headache. The patient died 3 weeks later.


A 62-year-old man presented with complaints of multiple dark eruptions involving his lower limbs, back, chest, and parts of the upper extremities. The lesions appeared 10 years ago on the lower limbs and progressed to involve the other parts of the body over the past 3 years. There was no history of itching, trauma, chronic scratching or friction, no systemic symptoms and no similar complaints in other family members. On examination, multiple, discrete, hyperpigmented, papules were present on the shins, [] and similar smaller papules were seen on the upper back, chest, abdomen, upper arms and few on the thigh and gluteal region [Figure and ]. Histopathological examination revealed a hyperplastic epidermis and globular masses in the dermal papillae []. Congo red staining revealed amorphous, eosinophilic deposits in the papillary dermis []. Routine laboratory investigations including complete blood count, blood sugar levels, renal, hepatic, urinary analysis, and thyroid function tests were within normal limits.
A diagnosis of lichen amyloidosis was made on the basis of clinicopathological findings, and the patient was given topical clobetasol propionate and salicylic acid combination along with oral acitretin 25 mg/day.