A 68 year old male patient was presented to the outpatient department with a painful swelling on the dorsum of the right hand without any history of trauma. It was primarily considered to be cellulitis and treated with antibiotics for 10 days by the general practitioner. He had bronchogenic lung carcinoma which was diagnosed 5 months ago. He also had a bone scan done which did not reveal any metastases. Clinical examination revealed a palpable mass at the ulnar part of the dorsum of the hand (Figure ). The hand was warm, red and tender. Flexion and extension movements of the fingers were painful. There was no neurovascular deficit. No epitrochlear or axillary lymph nodes were palpable. The radiographs revealed a lytic lesion of the fifth metacarpal with destruction of its distal part (Figure ). Chest x-ray showed a large mass in the right upper lobe of the lung, in keeping with a bronchogenic carcinoma (Figure ). MRI of the hand revealed a large, solid mass originating from the fifth metacarpal and extending to the adjacent soft tissues (Figure ). Open biopsy showed metastatic carcinoma. The case was discussed with the Oncologist. We decided that, due to the broad extension of the mass to the hand, the patient should not receive any surgical treatment and he was referred to the oncology department.
The most common site of metastatic deposits to the hand is the distal phalanx. The incidence of metastasis to the metacarpals is 17%, phalanges 66% and carpal bones 17% []. Keramidas and Brotherston [] published a case with metastasis to both metacarpal and carpal bones, which is extremely rare.
It is rare to see bronchogenic carcinoma metastases to the bones distal to the elbow and knee. Floridis was the first to describe this rare entity in the United States in 1934 []. Depass (1958) was the first to report a metacarpal metastasis in the English literature [].
The lung is the most common source with 42% followed by the breast and kidney each of which account for 11% [,]. Other sources include colon, prostate, thyroid, oesophageal and bone cancers []. Men are more commonly involved than women []. Metastases in the hand are the first clinical sign of an undiagnosed tumor in only 16% of all metastases [].
The exact reason for this rarity of such metastases is not known. In 1889 Paget [] suggested the "seed and soil" theory for metastasis, which states that one needs to have both seed (i.e. tumor emboli) and good soil (or proper site) for this tumor emboli to settle down and grow. Prostaglandins have been implicated as possible chemotactic factors that influence cell migration and adherence to the skeleton []. Tumor deposits occur mainly in the bones hematopoietically active and multiply to produce typical lytic lesions or, occasionally, formation of reactive bone []. The infrequent development of metastases to the hand may be related to the smaller amount of red marrow present in these bones. Piney [] observed, as early as 1922, the absence of bone marrow in phalanges – spared metastasis. Recent trauma and increased blood flow have also been implicated as a nidus for tumor metastasis [,,]. Joll [] suggested that repeated trauma might play a role in reducing the local tissue resistance thus producing a fertile ground for "seed". Shinz [] pointed out that primary malignancies erode veins (systemic or portal) and tumor emboli are filtered by lung or liver. But in the case of bronchogenic carcinoma, the tumor erodes the pulmonary vein and thus has access to systemic circulation and, consequently, widespread metastasis. In contrast to this, the vertebral venous plexus of Batson permits mostly axial skeleton metastases [].
The patient usually presents with a painful, swollen, erethymatous and warm hand [,]. The x-rays show lytic bony lesion. The differential diagnosis includes gout, pulp space infection, osteomyelitis, septic arthritis, rheumatoid arthritis, tenosynovitis and reflex sympathic dystrophy [].
The prognosis of these patients is poor, with the median survival being usually six months []. This should be taken in account in the management of these patients. Radiotherapy and chemotherapy may be appropriate and amputation could be an option for lesions at the distal phalanx. Reconstructive surgery is not indicated due to poor prognosis [].


A 24-year-old woman was hospitalized in a state hospital because of confirmed bilateral ovarian cysts and ascites. Then she was referred to our hospital because of increasing abdominal girth and dyspnea of 1 week's duration. The patient's weight was 80 kg and height was 162 cm. The stimulation was performed by an external unit using a long protocol with follitropin alfa. The stimulation cycle had to be discontinued because of OHSS. The patient showed a severe form of OHSS with massive ascites, abdominal pain, dyspnea, blood pressure 80/40 mmHg, pulse rate 100 beats/min, plasma estradiol 4000 pg/mL, β-hCG 86 IU/L, hemoconcentration of 52%, hemoglobin of 18.0 g/dL, leucocytosis of 29 × 103/μL, PaO2 of 70 mmHg, PaCO2 of 40 mmHg, pH of 7.44, and oxygen saturation of 91.2%. The ultrasonographic examination revealed bilaterally enlarged multicystic ovaries and a large amount of ascites (Figure ).
Immediately after admission, infusion therapy was started, consisting of normal saline-infusion 0.9% 1000 ml (Pharmacia), glucose 5% 1000 ml (Pharmacia), four times 50 ml of human serum albumin 20%, and low-molecular weight heparin 5000 IU 2 × 1 per day. Body weight, abdominal circumference, intake and outputs, ultrasonography, and laboratory studies were monitored strictly daily. Renal function was supported using diuretics (Furosemide 20 mg ampoule and Furosemide tablet). Renal function was not disturbed and there were no serious changes in serum electrolytes. The patients' girth increased between days 2 and 7, and then abdominal paracentesis for the drainage of the massive ascites became necessary. The patient reported increasing dyspnea. The anteroposterior chest X-ray (Figure ) revealed right pleural effusion, whereupon a chest-tube was placed for treatment of pleural effusion.


42 year-old male patient, ASA III; positive anamnesis for hypertension, left ventricular hypertrophy, incomplete right bundle block, moderate mitralic and aortic insufficiency; end-stage kidney disease in dialysis for 5 years. Diffuse angiokeratomas, especially in lower limbs and inferior abdomen.


53 years old male patient, ASA III; myocardial infarction 3 years before, no revascularization or angioplasty. Hypertension, chronic obstructive lung disease with borderline spirometry and end-stage renal disease in dialysis for 8 years. Diffuse angiokeratomas in lower limbs, abdomen and thorax.
Anaesthesiological implications for both patients might be relevant: however, despite the underlying Fabry disease, standard anaesthesiological protocol for kidney transplantation was applied: propofol 1.5 mg*kg-1, cisatracurium 2 mg*kg-1 and fentanil 1.5 mcg*kg-1 were administered for anaesthesia induction, while maintenance was performed with sevoflurane 1–1.5 MAC and fentanil/cisatracurium boluses on demand. Accordingly to our protocol, fenoldopam continuous infusion was performed up to 3rd postoperative day; one of two patients received "nephroprotective" infusion at standard 0.1 mcg*kg-1 *min-1, while one patient received scalar doses up to 0.8 mcg*kg-1 *min-1 to control unresponsive hypertension. Cardio-haemodinamic management was standard 3 leads EKG, invasive blood pressure (difficult arterial cannulation in both cases) and in the second patient it included non invasive cardiac output measurement via PRAM® monitor (arterial waveform advanced analysis). Careful haemodynamic monitoring was useful for both intra-operative fluid management and to titrate anti-hypertensive therapy. Both patients received intraoperative transdermal nitroglycerine during surgery.
Interestingly, one patient showed unexpected laryngoscopic and intubation difficulty, despite normal antropometric predictive parameters. Ventilation was always granted and intubation was achieved at third attempt using McCoy blade and Frova Hollow introducer (Cook® – Bloomington – USA).
In both patients awakening was uneventful after uncomplicated surgery; extubation was performed in operative room and incentival spirometry was started immediately the day after surgery. Analgesia was granted with intravenous morphine.
In both cases renal function restored on day 2 and 3 post-transplant, respectively, with no needs for dialysis. At a follow up of 52 months and 13 months, respectively, both recipients are alive, in treatment with algasidase beta replacement therapy, and have an excellent renal function.


A 65-year-old female was relatively well before and had history of diabetes mellitus without medical control. This admission, she suffered from sudden onset of retrosternal chest pain at rest and came to our emergency room for help. The pain was tight in characteristic and radiation to the epigastric area. On physical examination, she had a blood pressure of 125/88 mmHg, heart rate of 77/minutes, and clear consciousness. Crackle could be heard in both lung fields and grade three holosystolic murmur was heard in the left lower sternal border. The blood tests revealed creatinine of 0.9 mg/dl, creatinine kinase of 251 IU/L, creatinine kinase MB of 31.11 ng/ml, and Troponin T of 0.800. The peak of serial cardiac enzymes values were as followed: creatinine kinase 500, creatinine kinase MB 40 and Troponin T 1.09 which noted 24 hours after onset. Chest X-ray showed mild pulmonary congestion. An ECG showed rightward axis, normal QT interval with QTc of 422 ms, and non-significant ST-T elevation (0.05 mv) at leads I, aVL, and V5 (Fig. ) without dynamic change in the serial follow-up ECGs. Under the impression of non-ST elevation myocardial infarction, conservative treatment with anti-thrombotic and anti-platelet regimens was prescribed. The echocardiography showed impaired global LV systolic performance with mid-septum hypokinesis and akinesis of mid-anterior to apical-anterior wall, but well preserved basal septal contractility. Cardiac catheterization was performed on the 7th day. Coronary angiogram revealed no significant stenonsis on coronary arteries and left ventriculography showed anterior wall akinesis, apical hypokinesis and slight basal hyperkinesis of left ventricle (Fig. ). The patient was improved after supportive care and follow-up echocardiography showed complete recovery of left ventricular systolic function without abnormal regional wall motion 2 weeks later.


A simple hepatic cyst was detected in the left lobe of the liver of a 25-year-old man in 1993 (Sadly, there was no imaging.). The patient was followed in our hospital, and no surgical treatment was performed although the cyst showed gradual enlargement. The patient was admitted to our hospital due to epigastralgia and for re-examination of simple hepatic cyst in 2004. In 1996 the cyst was unilocular, 6 cm in diameter without calcification, septa or papillary projections as observed by CT (Fig. ). In 2001 the cyst remained the same diameter but exhibited calcification and septa (Fig. ). An abdominal CT performed on admission in 2004 showed that the unilocular cyst had grown to over 10 cm in diameter with increasing mounting calcification, septa and thickening of the wall within the cyst (Fig. ). MRI revealed a partly low intensity, partly high intensity T1-weighted image, and high intensity T2-weighted image (Fig. ). US revealed a unilocular cyst over 10 cm in diameter and partial septa within the cyst (Fig. ). Abdominal angiography showed the tumor to be hypovascular and stretching of left hepatic artery. Endoscopic retrograde cholangiopancreatography (ERCP) revealed compression of the bile duct and no communication between the cyst and the bile duct was shown. The serum level of aspartame aminotransferase (AST) and almandine aminotransferase (ALT) were slightly elevated but tumor markers such as CEA and CA 19-9 were within the normal range. The cystic lesion was suspected of being a mucin-producing liver tumor, such as hepatobiliary cystadenoma or cystadenocarcinoma. Despite of these findings, we could not rule out the malignancy clearly. Therefore, in November 2004, left lobectomy of the liver with cholecystectomy was performed. Macroscopically, a resected specimen was a unilocular tumor filled with mucus. The cut surface of the tumor exhibited an elastic white-colored scar and yellowish papillary nodule (Fig. ). The unilocular cyst had a solid component with papillary projections, septa and calcification and was filled with liquid-like muddy bile. Histopathological examination revealed that the inner layer of the cyst was lined columnar epithelium exhibiting mild grade dysplasia and partially lined with papillary epithelium (Fig. ). Dense mesenchymal stroma was not detected. On the basis of these findings, hepatobiliary cystadenoma was diagnosed. The postoperative course was uneventful and recurrence of the lesion has not been observed. We believe this case provides evidence of a simple hepatic cyst changing into hepatobiliary cystadenoma over a 10-year period.


Our patient was a 50 year old female with a history of HIV on HAART (Highly Active Antiretroviral Therapy), who came in to our center for routine breast cancer screening. She had a significant family history of breast cancer in her sister, maternal grandmother and her aunt. Her last mammography was two years prior which only showed benign calcifications in both breasts and an enlarged benign-appearing lymph node, hence a BI-RAD 2 (Breast Imaging Reporting Data) designation was given. On the day of presentation, the patient was asymptomatic. She denied any B-symptoms (night sweats, fever, etc) or bone pain. Digital screening mammography was obtained which showed a new circumscribed nodule in the right upper quadrant measuring 2.3 cm, multiple circumscribed benign-appearing lymph nodes in each breast and bilateral axillary lymphadenopathy (fig. ). A BI-RAD 0 (incomplete) designation was given. Exaggerated right CC view showed multiple circumscribed nodules in the lateral right breast with the largest nodule measuring about 2 cm (fig. ). Sonographic evaluation was performed on the upper outer aspect of the right breast which showed an echo-lucent mass (fig. ). Adjacent to that mass was another 9 mm hypoechoic area and an area in the 10 o'clock position consisting of clustered microcysts measuring 6 × 4 × 7 mm (fig. ). A BI-RAD code of 4 was given for the suspicious lesions and fine-needle cyst aspiration was ordered. During the procedure, core biopsy was initiated because the mass was solid. Pathology results from the breast biopsy showed an aggressive high-grade B-cell lymphoma with positive immunostaining for CD20, CD10 and Bcl-6 (fig. ). The Ki-67 proliferation index was approximately 80%. PET/CT scan was ordered for staging which showed focal FDG accumulation in the right breast mass with ipsilateral axillary adenopathy (fig. and ). Right breast lumpectomy with right axillary lymph node biopsy was performed and the patient was started on systemic chemotherapy.


A 22-yr-old male, farmer by occupation was referred to our tertiary cardiac care institute for management of abnormal ECG changes mimicking acute coronary syndrome. He had sustained accidental electric burn injury (240V) while carrying out some work at home 2 hours prior to admission. He was thrown to the ground following an electrocution and was found to have brief period of loss of consciousness for few seconds with spontaneous recovery. He was immediately attended at a local hospital where ECG changes were noticed and referred to our institution for opinion and further management. He had no prior history of cardiac illness. There was no history of chest pain, palpitation or neurological deficits. There was no history suggestive of sudden or premature cardiac death in his family. He was not on any medications.
On physical examination, patient was conscious alert and had deep electric burn entry wound over the dorsum of left hand and an exit wound over the left ankle. The arterial blood pressure and heart rate were 120/80 mmHg and 78 beats per minute, respectively. Cardiovascular assessment was normal. Other systemic examinations were normal. Surface electrocardiogram done immediately following injury demonstrated a sinus rhythm with RBBB with coved ST segment elevation with negative T wave in V1 mimicking type 1 Brugada pattern and saddle back ST segment with upright T wave in V2 mimicking type 2 Brugada pattern with early repolarisation (). QTc was normal. Echocardiographic assessment revealed a normal left ventricular size and function, and no valvular pathology. Routine laboratory tests, including blood urea, serum creatinine, serum electrolytes and cardiac biomarkers were normal. Repeat ECG at 2 hours showed disappearance of above mentioned Brugada type pattern with persistent early repolarisation. Patient was observed in the ICU for rhythm disturbances for 24 hours. Repeat ECG after 24 hours was normal pattern. Since patient had no family history of sudden cardiac death and family members showing normal ECG and clear precipitating event that can result in above mentioned ECG changes he was not subjected for further investigations like induction with class I antiarrhythmics or electrophysiological studies.


The patient is a 70-year-old African American man with a history of bullous emphysema who presented with a 3-week history of severe left-sided chest pain to the emergency department. He had undergone a left upper lobe bullectomy at an outside associated institution 1 month prior to presentation for macrobullous emphysema with 4 prior spontaneous pneumothoraces. Chest computed tomography scan in the emergency department was unremarkable with no signs of pneumothorax, pulmonary embolism (PE), or pneumonia. Results from electrocardiographic and routine laboratory studies were within normal limits. The patient was admitted to the medical service for pain control and started on a patient controlled analgesia pump.
Approximately 48 hours after admission, the patient experienced an acute increase in chest pain and became tachycardic, tachypneic, and desaturated to 60% by pulse oximeter on nonrebreather oxygen supplementation. He was emergently intubated and transferred to the medical intensive care unit. Portable roentogram revealed a large right-sided pneumothorax (Fig ). Three attempts at right needle thoracostomy were performed without a notable rush of air or change in hemodynamics.
The thoracic surgery team was emergently consulted for chest tube placement. An air rush was noted upon entry in to the right hemithorax and the patient stabilized after chest tube placement. A sizable continuous air leak was noted upon chest tube insertion, as was a considerable amount of right anterior chest subcutaneous emphysema (SE) that tracked inferiorly from the infraclavicular sites of repeated attempts at needle thoracostomy (Figs and a). Of note, the patient required high amounts of ventilator positive end-expiratory pressure to maintain adequate oxygenation. A 2-cm “blowhole” incision was made below the right clavicle through the skin and prepectoral fascia in an attempt to allow subcutaneous plethora decompression.
No appreciable change in body contour or crepitance was noted over the next 48 hours; however, the patient's severe SE, which had tracked inferiorly into the abdominal subcutaneous tissues, had stabilized (Fig b). A topical negative pressure wound therapy (NPWT) dressing (VAC dressing, KCI International, San Antonio, Tex) was inserted into the blowhole incision and set at a continuous suction of 100 mm Hg (Fig ). Over the subsequent 48 hours. there was near-complete resolution of SE as supported by physical examination and repeat computed tomography scan (Fig ). Over the ensuing 4 days, the patient stabilized and was ultimately extubated. An air leak persisted but was adequately controlled by the chest tube. The patient underwent definitive right upper lobe bullectomy approximately 3 weeks later to remove bullae ruptured during attempted needle decompression and chest tube placement. He was discharged in good condition on postoperative day number 6 after a right thoracotomy and right upper lobe bullectomy.


A 70-year-old man presented with poor appetite and general malaise of two month's duration. Physical examination revealed mildly distended abdomen and hypoactive bowel sounds. Hemogram showed high white blood cell count (22070/μl) with 91.5% segment form and low hemoglobin level (7.0 g/dl). Biochemical examination showed elevated serum calcium level (2.7 mmole/l). Urinary analysis showed hematuria (numerous red blood cells per high power field in sediment examination). The serum prostate-specific antigen (PSA) was 100 ng/ml. A chest radiograph (figure ) showed diffuse osteoblastic bone lesions. In addition to dilated bowel loops with feces, an abdominal radiograph (figure ) showed multiple patchy osteoblastic changes of bones. A malignant disease with multiple osteoblastic bone metastases was highly suspected. Finally, prostate cancer was diagnosed with a prostate needle biopsy. He received bilateral orchiectomy and hormone therapy for stage IV disease. Unfortunately, he passed away 1 month later because of urosepsis.


A 3.3 kg full term African American female infant was delivered at a community hospital by cesarean section secondary to fetal bradycardia and suspected macrosomia to 37 years old G8P5 mother. The pregnancy was complicated by gestational diabetes mellitus and pregnancy-induced hypertension. Apgars were 8 and 9 at 1 and 5 minutes respectively. No neck mass was reported on prenatal ultrasound. The lack of maternal fetal medicine specialists at the community hospital might explain the quality of prenatal ultrasound. In the well baby nursery at the community hospital a right neck mass was noted. Due to history of spitting on regular infant formula, soy-based formula was started, but without improvement. She was transferred to the Regional Neonatal Center for further evaluation and management of the neck mass.
The baby was on room air for 1 week when she developed stridor. The neck mass had increased in size and a Pediatric ENT consult was obtained. An MRI demonstrated "a cystic lesion in the nasopharynx and right neck which inferiorly followed the course of the right carotid artery probably consistent with cystic hygroma" [fig. ]. The mass continued to increase in size so a follow-up MRI and MRA were done. MRI showed increase in size of right posterior retropharyngeal to right neck cystic mass measuring 4.1 cm and displacing the trachea and oropharynx to the left and the right carotid artery posteriorly and laterally [fig. ]. MRA showed the right common carotid artery displaced posteriorly and laterally without definite hemodynamic stenosis. But it is possible that neurovascular and respiratory structures of the neck were compressed during labor to cause fetal bradycardia. Carotid duplex ultrasound was recommended, which was severely limited due to displacement of vessels, presence of the mass and small size of the neck. To reduce fluid build up in the neck mass, the patient was started on 0.1 mg/kg of dexamethasone every 6 hours after the first MRI. She was later intubated for stridor and concern of potential airway obstruction. A team of pediatric otolaryngologists and pediatric surgeons was assembled to address the mass. The otolaryngologist decided to approach the mass via a cervical incision. Imaging studies demonstrated that the mass extended inferiorly to the aortic arch and a sternostomy had been planned. Intraoperatively the mass was found to be limited to the neck, so a sternostomy was not necessary. A hemithyroidectomy was required when the cystic hygroma was found to be inseparable from the right half of the thyroid. Dexamethasone was tapered over 2 weeks. She was observed for 24 hrs after the steroid taper. Endocrinology was consulted after the hemithyroidectomy. Initial thyroid function tests were normal except for mildly elevated T4 [13.9 μg/dL]. PTH was also normal [50.8 pg/L]. Genetics was consulted and chromosomal analysis showed 46 XX. The patient was noticed postoperatively to have a weakness of the right lower lip, consistent with neuropraxia involving the marginal mandibular branch of the facial nerve. This deficit is expected to correct with time.


In October 2006, a 35-year-old Swedish man presented to hospital in Stockholm with a two-day history of fever, sweats, headache and fatigue. He had a temperature of 40°C, but was otherwise in good general condition with a normal respiratory rate and blood pressure. Laboratory investigations revealed normal haemoglobin concentration (154 g/L), leucopaenia (2.2 × 109/L), moderate thrombocytopenia (58 × 109/L) and an elevated C-reactive protein concentration of 109 mg/L (normal < 10 mg/L). Plasma creatinine was normal, while plasma bilirubin (31 μmol/L) was just above the upper limit of the normal range and alanine transaminase (1.68 μcat/L) was slightly increased (normal < 1.20 μcat/L).
The patient had recently returned from a two-week holiday in Sarawak, Malaysian Borneo. He had trekked in the jungle of the Bario Highlands during the last seven days of his holiday, at altitudes between 800 and 1,400 m. He had not taken any anti-malarial chemoprophylaxis. His symptoms started 11 days after leaving the Bario Highlands. There was a strong suspicion of malaria, and although a rapid diagnostic test (Now® Malaria Test, Binax Inc., Maine, USA) was negative, oral mefloquine therapy was started while waiting for the results of the blood film examination. Malaria parasites were detected by microscopy in 0.1% of erythrocytes. The Plasmodium species could not be identified conclusively, but Plasmodium malariae was suspected, since late trophozoites were observed and the infected red blood cells were not enlarged (Figure ). The patient improved rapidly following mefloquine treatment and was discharged afebrile after two days. The lowest recorded haemoglobin and platelet levels were 95 g/L and 34 × 109/L, respectively. Two blood cultures were negative and a pulmonary x-ray was normal.
Due to the uncertain identification by microscopy, PCR assays for P. falciparum, Plasmodium vivax, Plasmodium ovale and P. malariae were performed in Sweden. These assays were negative, but microscopy results that were suggestive of P. malariae and the patient's travel history led to the suspicion of a P. knowlesi infection. A frozen blood sample, collected when the patient was admitted to hospital, was sent to the Malaria Research Centre at University Malaysia Sarawak for further analysis. The infection was identified as a single P. knowlesi infection following DNA extraction and examination by PCR assays for P. falciparum, P. vivax, P. malariae, P. ovale and P. knowlesi as described previously []. PCR amplification, cloning and sequencing of the A-type small subunit ribosomal (SSU r) RNA gene was also undertaken, followed by phylogenetic analysis as described earlier []. The SSU rRNA gene sequence of the malaria parasite from the Swedish patient was phylogenetically indistinguishable from those of other P. knowlesi isolates (Figure ).


A 70-year-old man was admitted to our hospital for treatment of gastric cancer. He had presented with a 7-month history of fatigue, intermittent abdominal pain, loss of appetite, vomiting and two episodes of haematemesis. He had lost 10 kg in weight during the last month, and felt he had no energy. The patient had no prior history of diabetes, hypertension, abdominal surgery or trauma. He had no cancer or weight loss. She didn't use any specific medication or drugs. The patient didn't smoke or drink alcohol. Physical examination revealed a temperature of 37°C, a pulse rate of 80 beat per minute (bpm), a blood pressure of 130/80 mm Hg. The patient was pale looking; the apex beat was in the right fifth intercostal space, midclavicular line. The heart sounds were normal. Nothing abnormal was found in the respiratory system. Abdominal examination revealed that the liver dullness was on the left side. There was a little tenderness in the epigastrium, but no mass was felt. On rectal examination the prostate was found to be moderately enlarged. The laboratory findings were: an hematocrit of 25%, an hemoglobin of 10 g/dl, a white blood cells of 8500/mm3, blood urea of 12 mg/dL, a creatinine level of 0,7 mg/dL. Dextrocardia was seen on the chest x-ray (Fig. ), the electrocardiography findings were typical of congenital dextrocardia. Endoscopic examination objectified a tumor with ulceration in the antrum of the stomach. Histological examination of biopsied specimens resulted in a diagnosis of well differentiated adenocarcinoma.
Chest X-ray revealed a right sided heart (fig. ). The abdominal CT showed Tumor thickened antrum gastric wall with situs inversus totalis (fig. ).
Based on the endoscopic and imaging findings we made a decision to perform a laparotomy. The abdomen was explored through a midline epigastric incision. There was complete transposition of the viscera (fig. ), the stomach and spleen lying on the right side, and the gall-bladder, the larger lobe of the liver and the caecum and appendix on the left. On the posterior wall of the antrum of the stomach was a large carcinoma 6 cm/8 cm (Fig ). There was no macroscopic serosal invasion or swelling of the lymph nodes. There was no evidence of glandular, hepatic, or peritoneal metastases. A subtotal gastrectomy Finsterer type was performed, with regional lymphadenectomy (D2), without splenectomy.
Histologic examination of the specimen showed a liberkunien middle differentiated adenocarcinoma, with serosal invasion and 3 lymph nodes metastasis (T3N+ according to international classification). The patient's postoperative course was uneventful. She was commenced on chemotherapy.


A 63-year-old Caucasian man presented complaining of right neck pain and dysphagia following a bout of violent coughing that he experienced on the previous day. On admission, he was haemodynamically normal with mild pyrexia. Physical examination revealed cellulitis extending from the right sternoclidomastoid region to the anterior upper chest with swelling and tenderness just above the right sternoclavicular joint. The blood tests showed leukocytosis and raised inflammatory markers. A plain chest X-ray showed a right pleural effusion. These findings raised suspicion of a spontaneous rupture of the cervical oesophagus. Oral intake was omitted and a nasojejunal tube was inserted for feeding along with empirical intravenous administration of penicillin, flucloxacillin and metronidazole. A computed tomography (CT) scan showed inflammatory changes behind the right sternoclavicular joint with small pockets of air behind the upper sternum, pleural thickening at the right apex with some adjacent lung consolidation and confirmed the presence of a pleural effusion (Figures and ).
A gastrografin swallow study showed a small irregularity of the lateral pharyngeal wall but not definitive contrast leak, and pharyngoscopy could not find any abnormality. Paracentesis of the sternoclavicular swelling was attempted under ultrasound guidance, but no micro-organisms were isolated from the aspirate. Nevertheless, because there was still a degree of uncertainty, it was decided to treat the condition with prolonged jejunal feeding and antibiotics. After 2 weeks on this regime, the inflammation resolved completely and the patient was allowed oral feeding and discharged home. However, 9 days after his discharge, the patient presented with the same symptoms. Another CT scan established the diagnosis of septic arthritis showing erosive changes within the right sternoclavicular joint (Figure ). The patient restarted a 6-week course of the same antibiotic combination but without restrictions in oral intake. Eventually, the arthritis settled and on follow-up 3 and 6 months later, respectively, the patient remained asymptomatic.


A 79-year-old Caucasian gentleman, with no history of any malignancy, underwent a left cemented total hip replacement (THR) for osteoarthritis and was symptom free post-operatively (Figure ). At 6 months he complained of pain in the left groin and thigh. Examination revealed painless hip movements. Radiographs of the left hip showed an osteolytic lesion in Gruen zones 2 and 3 (Figure ). Routine blood investigations showed an erythrocyte sedimentation rate of 90 mm/hr and C- reactive protein of 50 mg/dl. Aspiration of left hip did not reveal any organisms and a bone scan showed increased uptake of on the left femoral shaft, right scapula and the first lumbar vertebra. Bone chemistry, tumour markers, renal and liver assay were normal. An open biopsy showed erosion of the lateral cortex associated with friable soft tissue mass and profuse bleeding. Histopathological report showed a classical case of renal cell carcinoma. Further to this, a computed tomographic (CT) scan of the abdomen and chest revealed multiple nodules in lung fields, multiple nodules in liver, a mass on each kidney consistent with renal cell carcinoma and multiple skeletal lytic lesions.
The patient was then referred to the oncologist for palliative treatment and died 6 months later.


A 16 year old male student of average built presented to us with progressively increasing swelling in the outer aspect of left ankle for last two years. Patient also gave history of pain off and on. There was no history of difficulty in walking or restriction of movements at ankle. There was a globular swelling measuring 6 cm × 5 cm over lateral aspect of ankle on examination (Fig. and ). It was bony hard in consistency, smooth with ill defined margins and non tender on palpation. There was no distal neurovascular deficit.
Patient was subjected to anteroposterior, lateral and oblique radiography of leg with ankle. Radiography revealed a well defined bony exostosis, arising from the interosseous border of distal tibial metaphysis with erosion and impending fracture of fibula on oblique view (Fig. &).
The patient was initially put in an ankle foot orthosis. The nature and prognosis of the condition was discussed at length with the patient and his family and operative intervention was planned once an informed and written consent was obtained. An MRI scan was ordered once the decision was made to undertake operative intervention. It was consistent with a large, broad based benign osteochondroma arising from the lateral aspect of distal tibia with an uncalcified cartilagenous cap (Fig. ). This led to a marked pressure erosion of the distal fibula, which was only 5 mm thick at the narrowest point (Fig. ).
The patient underwent excision of the osteochondroma through an anterior approach without fibular osteotomy. Intra-operatively, the fibula was found to be quite thin and weak. However, its outer cortical shell was intact. The inferior tibio-fibular joint was stable. Histology confirmed the clinical diagnosis of osteochondroma with no malignant transformation. Post-operatively, the patient was mobilised, non-weight bearing in a below knee plaster, for four weeks. Further mobilisation was undertaken with a gradual transition from partial to full weight bearing. At one year follow-up, he had made a complete recovery with full return of ankle functions. The fibula had recovered the full thickness. There was no evidence of recurrence and he is still under follow up.


In March 2007, an otherwise healthy 41-year-old Caucasian female veterinarian from Instituto Lauro de Souza Lima, Bauru, São Paulo, Brazil complained of a slowly growing subcutaneous nodule on the inner side of her left hand middle finger. The patient did not recall any previous trauma in that particular anatomical area. The nodule had appeared 10 months earlier as a small hard cutaneous swelling on the proximal articular side of the middle phalanx, resembling a synovial cyst. The nodular skin lesion was very small and painless, therefore the patient did not seek immediate medical attention. In the following months after she had first noted the tissue swelling, the nodule increased in size and interfered with flexion of the affected finger. It was difficult to determine whether the nodule was attached to the skin or to the subcutaneous tissue at palpation. Clinical examination of the subcutaneous nodular lesion (~2.0 × 1.5 cm in diameter) by a surgeon led to diagnosis of a giant cell tumor of the flexor tendon, and surgical excision was advised. Physical examination revealed that the patient was in good general health and had no other similar skin lesions. Surgery was performed 10 months after the initial onset.
The granulomatous 2 cm × 0.5 cm × 0.5 cm excised mass was attached to the dermis, nerves and tendons of the affected finger. It consisted of a firm yellowish tumoral-like mass resembling a lipoma, with a smooth bright surface. Because of the initial diagnosis of a benign tumor, microbiological testing (including culture) was not requested. Histopathological examination of hematoxylin-eosin stained sections showed a granulomatous infiltrate constituted by histiocytes and giant cells filled with numerous thick walled yeast-like cells, either singly or in chains, characteristic of L. loboi (Figures and ). The majority of the fungal elements in the infected tissues showed clear cytoplasmic content, a morphological characteristic of viable L. loboi yeast-like cells. Methenamine silver staining showed spherical to oval yeast-like cells mostly uniform in size and arranged singly or in small chains of cells linked by small tube-like structures (Figures and ). In respect to treatment, it has been observed that after use of clofazimine and dapsone, which have antimicrobial as well as anti-inflammatory activity, in concomitant leprosy and Jorge Lobo's disease patients, Jorge Lobo's lesions became atrophic. Itraconazole was chosen as the antifungal drug because of its low toxicity, high affinity to skin and good results when used with clofazimine in a Jorge Lobo's disease case. To prevent recurrence of the lesion in the present patient, drug therapy with clofazimine (50 mg/day), dapsone (100 mg/day) and itraconazole (200 mg/day) was initiated immediately after surgical intervention and continued for 1 year.


A 29-year-old white woman presented with a tumor that had appeared on the right thigh two years before. Physical examination revealed a hard nodule about five centimeters in diameter with no signs of inflammation. Ultrasound showed a heterogeneous tumor measuring 5.0 × 6.5 × 4.5 cm and coarse calcifications within the tumor. Because of hemangiomas in upper extremities, the initial hypothesis was thrombosed hemangioma.
Histopathological analysis of excisional biopsy specimen showed fusiform and vasoformative tissue. Immunohistochemical analysis suggested a well-differentiated myxoid liposarcoma.
After the diagnosis of soft tissue sarcoma, complete tumor staging was performed using chest radiographs and CT, which showed lytic bone lesions in fourth and seventh anterior ribs, and nuclear MRI of right thigh, which showed a large, predominantly cystic and heterogeneous lesion. Therefore, radical tumor resection was performed. Material was sent to the Mayo Clinic, and the final diagnosis was a low-grade vascular neoplasm consistent with retiform hemangioendothelioma and not liposarcoma, as previously thought.
After one-year follow-up, the patient noticed a new tumor on the right thigh and inguinal region, and biopsy confirmed lymph node metastasis. Chest CT revealed several lung lesions, but etiology was not confirmed by biopsy. Chemotherapy with doxorubicin was initiated, and full clinical response was achieved.


A 3-months-old female was referred to our department because of suspicion of child physical abuse. She was born at term, a product of uneventful gestation. At birth her weight was 3310 gm, length of 51 cm and her occipito-frontal circumference was 33.5 cm. No irritability, feeding or pulmonary abnormalities, and fever were recorded. The mother was 29-year-old-gravida 2, married to a 33-year-old-unrelated man. She had no history of spontaneous abortions, stillbirths, prematurity and or polyhydramnios. No maternal use of medications or antenatal illnesses was reported. Physical examination of the baby showed normal facial features, no blue sclerae, normal nose but swelling of the mandible was noted. There were swellings of some of the long bones (right leg, and radius). Neither feeding abnormalities nor fever was present. Her chest was noted to be symmetrical and of normal appearance. Hands and feet were normal. There were no associated signs of abnormal skin stigmata such as fragility/extensibility and or ligamentous hyperlaxity was noted. Hearing, vision and neurological examinations were all normal. Laboratory studies including metabolic tests, which aimed to tests calcium, phosphorus, and vitamin D metabolism were normal. No specific genetic test has been done for this baby.
Anteroposterior radiograph of the skull showed massive sclerosis of the skull bone associated with significant cortical hyperostosis and enlargement of the mandible secondary to cortical new bone formation (fig ). Lateral skull radiograph showed sclerosis of the skull base (arrow) and hyperostosis of the calvaria (fig ). Coronal MRI imaging showed significant calvarial/facial and mandibular hyperostosis (fig ). Anteroposterior radiograph of the radius showed cortical new bone formation associated with subperiosteal thickening. Note marked bloating along the diaphysis with sparing of the epi-metaphyseal components associated with expansion of the bone marrow cavity and a persistent-like deformity (fig ). Anteroposterior radiograph of the tibia showed a thick and broad ballooning occupies the diaphyses (proximally and distally) there is gaining in the diameter comes from subperiosteal new bone apposition by intramembraneous bone formation (fig ).


A twenty-eight year old female patient presented to the breast clinic with a palpable painful mass on the upper lateral quadrant of the right breast, measuring 3 × 4 cm2. Upon further questioning, menarche occurred at the age of thirteen and it was stated that her menstrual cycle was regular. The patient denied use of contraceptive pills. No birth, miscarriage or abortion is mentioned. The patient was a smoker. There were no inherited diseases or family history of cancer.
A biopsy under general anesthesia was performed, which was positive; therefore an extended tumorectomy and radical lymph node dissection were performed. The histological testing showed invasive ductal carcinoma of myeloid type, grade III with 4/12 of the axillary lymph glands involved, as well as coexistence of carcinomatic emboli to the lymphatic vessels. The surgical margins were clear of neoplasmatic cells. The tumor was negative to PR and ER: 5% progesterone receptors, 10% estrogen receptors, 0% p53, c-erbB-2: score +3, 60%, ki 67. The patient received chemotherapy (clitaxel, epirubicin, paclitaxel, cyclophosphamide, methotrexate) for four months and radiotherapy for one month.
A year after the accomplishment of the adjuvant treatments she got pregnant and was under supervision thereafter. The pregnancy was uneventful and a healthy child with no health problems (normal physical and mental development) was born, by natural labor.
The patient breastfed her baby for three months. Routine follow-ups were performed every month, included ultra sound (U/S) cancer ratings, with normal findings.
Four years post-labor, the patient remains free of disease.


A thirty-four-year old female patient presented to the breast clinic with a palpable painless mass on the upper lateral quadrant of the right breast. Menarche occurred at the age of fourteen, as well as regular menstrual cycle was stated. No contraceptive pills use is mentioned. It was also mentioned that she has had children, no miscarriage or abortion. The patient was a smoker. Her family medical record was also free of inherited diseases or cancer.
The patient was submitted to immediate biopsy under general anesthesia (which was positive). Therefore, a broad resection of the quadrant of the right breast with respective lymphadenic care was performed. The histological testing showed ductal invasive carcinoma grade II with 2/13 of the axillary lymph glands involved. The surgical limits were clear of neoplasmatic cells. The tumor was positive to ER and PR: 90% progesterone receptors, 80% estrogen, 0% p53, c-erbB-2: score+1, 15% ki 67. The patient received radiotherapy for two months (40 sessions) and she was given anti-estrogen (Tamoxifen) for two and a half years, which was discontinued due to the occurrence of ovary cysts and menstrual disturbances.
Eight years after the accomplishment of the adjuvant treatments she got pregnant under supervision. The pregnancy was uneventful and a healthy child was born via a caesarian incision. The patient did not breastfeed her baby. Routine follow-ups were performed every month, included ultra sound (U/S) and cancer indexes testing, which both proved to be negative throughout the whole period.
Thus, twelve years post-mastectomy, during the check-up performed with bone CT scanning, deposits were found on the bones and the ultra-sound showed liver metastasis, measuring approximately 3 cm. The patient received chemotherapy (Fluorouracil, Adriamycin, Cyclophosphamide) for six months. After the six-month period she continued Tbs treatment (Noveldin) for three months.
Till present, according to the C/T scans, the metastasis in the liver measures 1 to 1,5 cm and the patient continues her treatment with Zometan for bone deposits.
Generally patients were monthly checked by clinical evaluation, cancer indices rating (CEA, Ca 125, Ca 153) and breast U/S during pregnancy and immediately after birth; Thereafter, they should be checked every three-six-nine-twelve months and then yearly with the addition of CT scanning of thorax, CT of upper and lower abdomen and bone scanning.


Upon abdominal computed tomography (ct) imaging performed to rule out nephrolithiasis, a 57-year-old woman was incidentally found to have a 5-cm renal mass. The patient subsequently underwent a right-sided nephrectomy, and pathology analysis of the surgical specimen revealed a 5-cm Fuhrman grade 2 clear-cell-type renal cell carcinoma. Imaging by ct of the chest, abdomen, and pelvis at that time showed no evidence of distant disease, and the patient was followed clinically by her urologist.
Roughly 2 years later, the patient presented to her primary care practitioner with vague complaints of fatigue and lethargy. Initial workup included a complete blood count, remarkable for a pronounced leucocytosis (30,000 white cells/μL, normal differential). Subsequent bone marrow examination was consistent with a diagnosis of chronic myelogenous leukemia (cml), and the patient was initiated on therapy with imatinib. The patient tolerated therapy with minimal adverse effects, and within 3 months of therapy initiation, she had mounted a complete cytogenetic response by peripheral blood Bcr–Abl mutation screening.
The patient continued to demonstrate a durable remission on imatinib therapy. However, at a time corresponding to roughly 4 years of imatinib therapy, the patient was noted to have onset of cough and shortness of breath. Imaging of the chest by ct revealed multiple pulmonary nodules, and ct-guided biopsy of a representative lesion was consistent with clear-cell carcinoma of the kidney. After a discussion of potential therapeutic options, the patient was initiated on therapy with bevacizumab while continuing treatment with imatinib.
Six months after initiation of the bevacizumab therapy, interval imaging data shows no significant change (), and the patient has experienced no toxicities related to either therapeutic agent.


A 60 year-old woman with permanent atrial fibrillation was referred to our University center for pacing system implantation due to symptomatic bradycardia.
Clinical history reported acute rheumatic fever in the childhood followed by, at 41 years of age, surgical mitral and aortic mechanic and tricuspid bioprosthetic valve replacements. Three years later degeneration of the tricuspid bioprosthesis, accompanied by grade IV regurgitation, required replacement with a new tricuspid mechanic valve (St. Jude n.31).
A guiding catheter was introduced and placed into the ostium of the coronary sinus via the left subclavian vein. Venography of the coronary veins was performed to establish detailed anatomy (). A bipolar endocardial pacing lead (Pacesetter Quicksite 1056T, St. Jude) was implanted in the anterior interventricular vein ().
Measurements at implantation reported pacing threshold 0.5 V at 0.4 ms, R wave sensing potential 20.7 mV, impedance 1160 ohms at 5 V. Electrocardiogram showed left ventricle pacing with right bundle branch block configuration. The lead was secured and connected to a single-chamber rate adaptive pacemaker (Sensia SESR01, Medtronic) placed in a subfascial prepectoral pocket and programmed to ventricular demand rate-responsive at 50 ppm. In day 2 after the implantation chest X-ray excluded lead dislodgement and controller based pacemaker measurements were stable. Six months later, the last available follow-up for this woman, all pacemaker parameters remained stable (threshold 0.75 V at 0.4 ms; R-wave sensing 19.1 mV; impedance 917 ohms at 5 V) in the absence of symptomatic arrhythmic events.


A 12-year-old female presented with vesicovaginal fistula of 5 years duration. She had genitourinary tuberculosis detected on histopathology after left nephroureterectomy and had received antituberculosis treatment for 9 months. Attempted vesicovaginal fistula repair 4 years ago had failed. On cystoscopy, bladder was hardly admitting tip of 14 Fr. sheath. A large vesicovaginal fistula was seen involving complete posterior wall of the bladder. Right ureteric orifice was not seen on cystoscopy. Anterior vaginal wall was 2 cm in length and 2 cm in breadth at the introitus. Posterior vaginal wall was 4 × 2 cm2. Biochemical parameters were within normal limits. Abdominal CAT scan revealed mild dilatation of right pelvicalyceal system. Patient underwent bladder augmentation with ileal ‘W’ pouch. Right ureter was reimplanted into the pouch. A 5-cm-segment of ileum was detubularised and used for anterior vaginal wall reconstruction. Uterus and tubes were seen attached by small cervix to vagina.


A 71-year-old man, who had colon carcinoma, visited his internist because of headaches and pain around the right eye. Roxoprofen, an anti-inflammatory agent, was prescribed. After 6 days, the patient was referred to our clinic because he was suspected to have early herpes zoster ophthalmicus (HZO). After informing him of the examination procedures, an informed consent was obtained to perform the tests.
External examination showed severely erupted skin lesions over the right ophthalmic branch (V1) of the trigeminal nerve. His visual acuity could not be measured because of a complete levator paralysis with severe dermatitis. However, when the eyelids were held open with an instrument (Desmar), a mild pseudo-dendritic keratitis was detected. Acyclovir eye ointment and antibiotic eyedrops were prescribed.
After 12 days, the eruptions on the skin and the swelling of the upper eyelid were slightly improved. But then a total ophthalmoplegia developed in the right eye. The pupil was dilated to 7 mm and did not react to light and accommodation. The cornea was clear although cells were seen in the anterior chamber. The fundus and optic disc were normal, however his visual acuity was reduced to 0.1.
T1, T2, and Gadolinium enhanced magnetic resonance imaging (MRI) was performed on the whole brain, and fat suppressed imaging for the orbit. Diffuse inflammation was detected in the images of the right orbit but not in the left orbit (Fig. ) in both coronary and horizontal sections. The orbital cavity had signs of inflammation, the ocular muscles were swollen although not in the muscle cone. The optic nerve in the retrobulbar region had an edematous ring-shaped image by T1-weighted with Gadolinium enhanced MRI. Although the optic disc appeared normal, an optic nerve involvement was suspected, and a tentative diagnose of orbital apex syndrome was made.
After 14 days, betamethazone eyedrops were prescribed for the iritis, and after 22 days, 30 mg predonisolone, was prescribed because his visual acuity had decreased to 0.06. After 40 days, his right optic disc was slightly pale. After 48 days, the stellate ganglion was injected with 6 ml of 1% mepivacaine hydrochloride to alleviate the post-herpetic neuralgia, and 27 weekly injections were given for half year. After 59 days, the adduction of the right eye was improved. But then, a 45 prism diopters (PD) right esotropia was observed at the primary position. He did not complain of double vision possibly due to the poor vision of his right eye.
After 108 days, the ptosis had improved, and pattern VECPs were recorded (check size 30 minutes, contrast 90%, reversal rate 3/sec). A response was not elicited by stimulating the right eye, while a P-100 component was clearly obtained by stimulating the left eye (Fig. ).
After 115 days, the levator function had improved and the difference in the eyelid opening was 3 mm. After 204 days, the right abduction palsy was slightly improved. After 291 days, the patient had a right ptosis of 4 mm, a slight abduction palsy, and a paralyzed pupil (7 mm). Optic atrophy was still present.


A 35-year-old female presented with profuse, irregular, intermittent vaginal bleeding for the past six months. One month prior to onset of the symptoms she underwent a laparoscopic tubal ligation at a peripheral centre. Her past obstetric history was non-contributory. General, systemic and gynaecological examinations were unremarkable except for the presence of significant pallor. Abdominal and transvaginal sonography showed presence of multiple vascular channels in the parametrium and myometrium with a bulky uterus and fluid in the endometrial cavity. Color Doppler imaging, suggested presence of arterio-venous shunting. Pelvic magnetic resonance imaging (MRI) [] revealed presence of multiple flow voids in the myometrium and parametrium, more on the right, with features suggesting blood within the cavity. A diagnosis of complex utero-adnexal arterio-venous malformation with suspected fistula was considered and pre-hysterectomy angio-embolisation was planned. Bilateral common, internal and external iliac artery injections were acquired by left femoral puncture []. Abnormal vascularity supplied by uterine arteries was noted. Selective injection of right uterine artery showed high flow AVF with early drainage into pelvic veins and inferior vena cava, with hypertrophied radial and spiral arteries. More distally abnormal uterine parenchymal blush with tuft of vessels was noted, with cross filling from opposite uterine artery injection as well. To close the high flow AVF first, histo-acryl glue [in a ratio of 1:1 with contrast media] was injected through a cobra catheter, which was placed in the right uterine artery, very close to the origin of the high-flow AVF. Glue was injected after confirming free flow and ensuring that no major branch was visualised on the angiogram. Deep catheterisation obviated the need for using a micro-catheter. A microcatheter-wire system was subsequently advanced in the major feeders to the myometrial low flow fistulae using cobra catheter as the guiding catheter. Polyvinyl alcohol [P.V.A] particles (average size 500-750µ) (Cook Bloomington Inc, USA) were injected from both uterine arteries to achieve occlusion of this malformation. The procedure was terminated when no further filling of malformation was seen from either side. Patient was discharged on the second post-procedure day. The patient opted against hysterectomy as she became asymptomatic. Follow-up MRI showed occlusion of abnormal vascularity []. Similar symptoms, however, recurred after one and a half years for which a second sitting of angio-embolisation was done. Hysterectomy was done on the fourth day of embolisation this time. Gross operative specimen and cut section showed few abnormal vascular spaces in the myometrium. The patient was asymptomatic till last follow-up.


A 55-year-old gentleman presented with the chief complaints of right upper abdominal pain along with fever with chills and rigor, and associated nausea and vomiting. On examination, there was mild icterus and tender right upper abdomen with guarding. Ultrasonography (USG) showed multiple hypoechoic space occupying lesions (SOL) in both lobes of the liver, largest one being 8 cm in diameter at the junction of both the lobes adjacent to the diaphragm. USG also showed dilated common bile duct CBD (11 mm) with a large stone obstructing the lower end. Hematological studies showed elevated bilirubin, liver enzymes, and leukocyte count. The provisional diagnosis was multiple pyaemic liver abscesses due to choledocholithiasis with cholangitis.
The patient was put on intravenous antibiotics. USG guided percutaneous transhepatic drainage of the largest SOL was done and an indwelling pig-tail catheter was placed in the cavity. An endoscopic retrograde cholangiography ERC with endoscopic sphincterotomy was done and the CBD was cleared of the stone. There was rapid recovery following this. The catheter initially drained pus and later bile which gradually decreased from 125 ml/day to 40 ml/day. Patient was sent home in a satisfactory condition with the indwelling catheter in situ and advice to report after a week. At home, after a few days, the catheter came off accidentally which he did not report or took seriously. A week later the patient started suffering from intractable cough with copious expectoration of green colored sputum which increased during sleep. The patient mistook the symptoms for respiratory infection as did the physicians he attended in his locality. Patient returned after a month to us with this presentation and was readmitted and investigations were carried out.
The sputum tested positive for bile. The USG showed a dilated CBD (14 mm) with sludge and heterogeneous echo pattern of the liver near the dome. Chest X-ray showed pleural effusion. Further imaging could not be done due to unaffordability.
The provisional diagnosis was biliptysis due to brochobiliary fistula secondary to residual bile duct obstruction.
Patient was subjected to laparoscopic exploration. Dome of the right lobe of liver was densely adherent to diaphragm and was not disturbed. The CBD was full of inspissated biliary sludge [] and tiny, crumbly, blackish calculi. CBD was cleared and a choledochoduodenostomy [] was done. The choledochoduudenostomy was done as a single layer interrupted sutures with 3-0 polyglactin. Concomitant cholecystectomy was added. The postoperative recovery was uneventful. The coughing gradually subsided over the next few days. The patient is doing well on 7 months follow up.


A previously healthy 9-year-old boy with normal growth and development presented with progressive pallor, peripheral oedema and respiratory distress. Examination showed pallor, pitting oedema, and respiratory distress. No lymphadenopathy, jaundice, hepatosplenomegaly or abdominal masses were present, and the remainder of the physical examination was normal.
Investigations showed hypoalbuminaemia; albumin 16 g/L, and total protein 27 g/L, with normal liver and renal function. The urine was normal with no proteinuria or haematuria. Haemoglobin (Hb) was 89 g/L, white blood cell count (WCC) 16.6 × 109/L, but neutrophils and lymphocytes and blood film were normal.
The chest X-ray (CXR) showed consolidation in the right lower lobe and an abdominal ultrasound scan was normal.
At gastroduodenal endoscopy, multiple raised large (2 to 3 cm in diameter) ulcerated tumours of the greater curvature of the gastric body were seen (Figure ), and numerous smaller tumours were seen in the second and third part of the duodenum but colonoscopy was normal.
Computed tomography (CT) of the chest and abdomen showed multiple exophytic lesions in the stomach, several filling defects within the jejunum, and a 5 cm long intussusception in the ileum (Figure ). Small lymph nodes were seen around the superior mesenteric vessels. Bilateral pleural effusions and atelectasis were seen in the chest but there was no mediastinal lymphadenopathy.
Gastric biopsies showed a diffuse lymphoid infiltrate (Figure ), immunohistochemistry identified a B-cell population and flow cytometry was positive for the B-cell markers CD20, CD10 and CD43 (Figure ). Fluorescence in situ hybridisation (FISH) was positive for the C-MYC, t(8:14) translocation of Burkitt lymphoma. The bone marrow aspirate, trephine biopsy and cerebrospinal fluid (CSF) were normal. No Helicobacter pylori infection was detected.
The histology, immunophenotype and FISH analysis of the biopsies, and the radiological findings were consistent with a primary gastrointestinal Burkitt lymphoma, Stage III, Group B.
Treatment was according to the UKCCSG consensus guideline for Burkitt lymphoma (2003). Induction chemotherapy with cyclophosphamide, vincristine and prednisone (COP) was given together with alkaline hydration and allopurinol, daily albumin infusions, frusemide and omeprazole.
No tumour lysis syndrome occurred, and there was a rapid rise in serum albumin and protein with resolution of clinical signs of protein-losing enteropathy.
Further chemotherapy included two courses of cyclophosphamide, vincristine, prednisone, cytarabine, doxorubicin and methotrexate (COPADAM) followed by two courses of cytarabine and methotrexate (CYM) and double intrathecal chemotherapy of methotrexate and hydrocortisone.
Our patient's clinical course and disease response were monitored by sequential endoscopic biopsy, histology and molecular analysis by FISH. The rapid clinical response was reflected in the rapid histological and molecular resolution of disease. Follow-up endoscopy showed complete resolution of the mucosal tumours with only residual mucosal puckering present. Abdominal CT cans were normal and, once in remission and having completed chemotherapy, the patient's ongoing disease surveillance was by endoscopy and repeat biopsy for FISH analysis. No molecular evidence of residual disease was detected, and he remains in clinical remission with complete resolution of the protein-losing enteropathy and no treatment related sequelae 4 years from initial diagnosis.


A 60-year-old man in good health visited our rehabilitation clinic complaining of left calf pain. He visited our clinic 10 days after the sudden onset of pain at the left medial aspect of the posterior calf during rigorous steep mountain climbing. In his words, he felt that the onset of left calf pain was like "being hit by a 100-ton train". Under the impression of the possible rupture of the left medial head of the gastrocnemius muscle, US examination was prescribed.
With the patient in the prone position, US examination was performed by a clinician who was well trained in using soft tissue ultrasound. The SONOS 4500 (Philips Medical Systems, Andover, MA, USA) US machine and S12 5-12 MHz real-time linear-array transducer (Philips Medical Systems) were used to examine the patient. After careful examination, bilateral symmetrical sonographic findings of the calf muscles were noted without evidence of muscle ruptures. Roentgenogram of the left lower limb did not reveal any evidence of bony fractures.
The patient returned to the clinic one week later complaining that the pain in his left calf area persisted and could be further aggravated by tiptoeing and weight bearing maneuvers. Again, US examination in the prone position did not reveal any abnormal sonographic findings.
After two normal sonographic findings in the prone position, the examiner tried a different approach. The patient was placed in the supine position with his knees flexed at 90 degrees (Figure ). The transducer was then placed pointing upwards to examine the muscles. An area of well-defined anechoic fluid collection with hypoechoic surroundings was noted (see Figure ). Under US guidance, a 21-gauge needle was inserted into the fluid collection area and 15 ml of serosanguinous fluid was aspirated (Figure ). Dramatic pain relief was noted after aspiration. An elastic stocking was applied to his left calf area after aspiration and follow-up two weeks later did not reveal further fluid accumulations.


A 62-year-old Caucasian woman presented with a two-month history of intermittent fever. Her past medical history included hypertension, dyslipidema, coronary artery disease and mild chronic kidney disease. She was found to have Enterococcus faecalis endocarditis of her native, bicuspid, aortic valve. She was treated with vancomycin, gentamicin and penicillin and subsequently underwent aortic valve replacement (AVR) with a Saint Jude valve in October of 2004. Her postoperative course was complicated by evacuation of a mediastinal hematoma, splenectomy and a partial colectomy with diverting colostomy for hemorrhagic colitis. The patient had been discharged and re-admitted multiple times and was ultimately transferred to the Oklahoma University Medical Center in May 2005.
Upon transfer, the patient complained of dyspnea, orthopnea and lower-extremity swelling. She was afebrile and hemodynamically stable. Her physical examination revealed jugular venous distention, a III/VI systolic murmur in the right second intercostal space, and bilateral lower-extremity edema to the knees. No peripheral stigmata of endocarditis were identified.
Nine blood cultures obtained over a five-day period were positive for Candida lusitaniae. Susceptibility testing was performed on the initial isolate only and revealed sensitivity to amphotericin B, caspofungin and fluconazole (Table ). Transesophageal echocardiography revealed multiple vegetations on a partially dehiscent mechanical aortic valve (Figure ) with severe aortic valvular regurgitation and a left ventricular ejection fraction of 50% (Figure ). Therapy with caspofungin was initiated prior to obtaining results of susceptibility testing on the initial isolate and was continued based on the minimal inhibitory concentration (MIC). The patient eventually underwent repeat AVR with coronary artery bypass grafting. Vegetations were noted on her removed prosthetic valve (Figures and ). Her post-operative course was complicated by a chest hematoma which was evacuated. Despite eventual clearance of her fungemia, the patient died from multi-organ failure in June 2005.


Our patient, an 81-year-old man who had hypertension with peripheral vascular disease, initially underwent a total knee replacement (TKR) for osteoarthritis in 1991. He later had two further revision procedures in the following three years, first for presumed infection (although all culture results were negative) and then for component failure. In 1999, he underwent complex revision surgery in an adjacent hospital, requiring a massive distal allograft of the distal femur and impaction grafting of the tibial component. This was performed via a tibial tubercle osteotomy and a constrained condylar total knee replacement was implanted. Unfortunately, the allograft did not fuse to the host bone, although the joint was mobile.
In late 1996, our patient was admitted as an emergency case with an infected knee replacement. He was febrile (39.5°C) and blood tests revealed a raised C reactive protein of 184 mg/L and peripheral white cell count of 14×109/L. Blood cultures were negative. Joint fluid and multiple tissue specimens taken intra-operatively from the joint and capsule were sent for microscopy, culture and sensitivity. Gram staining identified Gram-positive bacilli in all specimens, which on culture was later confirmed as Lactobacillus paracasei through phenotypic characterisation using the API CH50 biochemical identification kit (BioMérieux). He was treated with intravenous amoxicillin and then oral clindamycin. Initially, he responded well and his inflammatory markers improved. However, a couple of weeks later, his symptoms recurred and the decision was taken to proceed with an above knee amputation. There were no postoperative problems.


We present a 6-month-old African American male child with poor interval growth. His mother has noticed that though he is thought to be breastfeeding appropriately, as defined by feeding 4 ounces of pumped breast milk every 2-3 hours, and has been meeting developmental milestones, his weight and height are not as expected. He has been exclusively breastfed and his mother has not introduced solid foods as of yet to his diet.
He was a full term, spontaneous vaginal delivery without complications during the pregnancy or labor. He was in the 50th percentile for both height and weight at his 2 month visit, but has fallen to below the 3rd percentile for weight and is at the 3rd percentile for height. He is on no medications, there are no other siblings with failure to thrive and his mother has no post-partum depression or substance abuse issues. There is no family history of malabsorptive conditions. His mother and father are of normal stature.
His review of systems is negative for emesis, diarrhea, fever, appetite changes, swallowing abnormalities, respiratory symptoms, apnea, repeated acute illnesses, or frequent injuries.
His weight at the four month well child visit was 6477 grams with a length of 63.5 cm increasing to only 6761 grams and a length of 66 cm by his six month well child visit. His vitals signs are otherwise stable. His physical exam is significant for an alert, playful, developmentally appropriate child, small for his age. His head/neck, cardiac, respiratory, gastrointestinal, genitourinary, musculoskeletal and neurological exams were within normal limits.
He is appropriately diagnosed with failure to thrive at this visit based on deviation across two major percentiles on standardized growth curves. His estimated weight needs were calculated to 0.33 kg/month and a follow up visit was established in one month with addition of solid foods and continued breastfeeding with the addition of formula to pumped breast milk for increased caloric intake. His mother was also instructed to keep a strict food diary.
Interval weight gain was not maintained with a weight of 7045 grams at follow up despite adequate caloric intake estimated based on his food diary and formula supplementation. Laboratory studies ordered were complete metabolic profile, thyroid stimulating hormone (TSH), lead level, and complete blood count (CBC). Electrolytes, kidney function, bilirubin, AST, ALT, protein, albumin, TSH, CBC, and lead were all normal. Alkaline phosphatase was elevated at 4280 (on repeat 6310). Normal should be less than 500 IU/L in neonates and 1000 IU/L in children up to age 9. Follow-up labs including gamma glutamyl transferase (which was normal, suggestive of boney resorption) [], C reactive protein, T3, free T4, phosphate, parathyroid hormone, and 25-OH D were ordered. Phosphate was low at 2.9 (normal 3.0-4.5) and the vitamin D level was 11 (45-50 ng/mL).
A skeletal survey was ordered showing metaphyseal fraying and cupping of bilateral distal femurs, bilateral proximal and distal tibiae and fibulae, bilateral proximal and distal humeri, bilateral distal radii and ulni and the distal aspects of 2nd through the 5th metacarpals most consistent with rickets of the extremities, see radiograph 1 and 2. These classic findings may be paired with a separation of the periosteum from the diaphysis secondary to unmineralized osteoid when evaluating radiographic evidence of rickets [,]. Radiographic improvement should manifest within 3 months of appropriate treatment. Underlying malabsorptive conditions or noncompliance should be considered if this is not observed [].
The diagnosis of rickets was made and the patient was started on 2000 IU of vitamin D and calcium carbonate 1000 mg daily. He was also started on iron sulfate 22 mg daily and Zinc 20 mg daily as recommended by pediatric endocrinology and nutrition staff. His catch-up growth has lead to a current weight (at 2 years of age) in the 45th percentile and height in the 30th percentile (). Follow up labs (calcium, phosphorous, alkaline phosphatase) should be performed one month after therapy is initiated and again at 3 months along with magnesium, PTH, and 25-OH D [,]. Follow up labs in this patient indicated an improvement in vitamin D up to 29 ng/mL with ongoing supplementation continuing.


The 41-year-old African-American man presented to the Emergency Department with severe shortness of breath, as well as severe, sharp, intermittent right-sided chest pain for 2-days duration without radiation. He had a history of asthma and HIV with progression to AIDS in the past 3 years. He also had a history of chronic alcohol and illicit drug use, and 20-pack years of cigarette smoking. Over the past 30-years, he worked in housekeeping at various hotels and occasionally as a shoe shiner. He did not have any occupational history of mining/milling, construction, ship-yard work or plumbing. The patient did have a brief history of assisting to demolish a house over an 8-hour period approximately 1 year previously; however it was unclear whether there was asbestos exposure during that time.
Physical examination revealed an anxious man in severe respiratory distress. His blood pressure was 157/86 mmHg; pulse rate 120/min; respiratory rate 40/min; temperature 97°F and oxygen saturation 92% on room air. He had reduced breath sounds throughout the left lung fields, with crackles in the right lower lung fields. The trachea was midline. He was tachycardic with normal heart sounds and a soft systolic murmur at the left lower sternal border. His abdomen was soft and without distention, tenderness or organomegaly. Capillary refill was normal, and Homan’s sign was negative. There was clubbing of his fingers. Neurologic examination was normal.
After sedating and intubating the patient, empiric antibiotics were initiated, and 1.6 L of serosanguinous pleuritic fluid was aspirated. Arterial blood gases on 100% oxygen via facemask revealed a blood pH of 7.42; PaCO2 30 mmHg; PaO2 80 mmHg; HCO3 20 mEq/L and SaO2 97%. Urine toxicology was positive for cocaine and opiates, while serum electrolytes, blood counts and liver function tests were normal. Cardiac enzymes were unremarkable, and electrocardiography showed sinus tachycardia with left ventricular hypertrophy and left atrial enlargement.
Radiography of the chest revealed left basilar infiltrates and pleural density that could represent pleural effusion. Also, there were chronic right upper lung scarring and cystic changes. Computed Tomography of the chest and abdomen showed extensive left basilar pleural disease with loss of lung volume, nodular left pleural thickening and a nodular pericardium. Nodules in the right lung were consistent with metastatic disease (see ). The right adrenal gland contained a necrotic mass (not shown). Positron Emission Tomography-Computed Tomography (PET-CT) revealed extensive left pleural thickening with associated hypermetabolism. A greater degree of hypermetabolism was located in the posterior lung base, with a density that was interpreted as pleural fluid (see ). The right adrenal mass demonstrated hypermetabolic activity (see ).
H&E stain of the left posteroinferior pleura and right adrenal mass showed a malignant neoplasm consisting of papillary structures, branching tubules, and gland-like acinar and cystic spaces. These structures were lined by uniform cuboidal and flattened epithelial-like cells with large, round-to-oval, pleomorphic, vesicular nuclei, prominent macronucleoli, occasional mitotic figures and abundant eosinophilic cytoplasms with distinct cytoplasmic borders (see ). Occasional mucin droplets were present. The surrounding matrix varied from myxoid to densely fibrous with hyalinized material with rare mitotic figures (1/10 hpf). Based on microscopic features, it was essential to distinguish malignant mesothelioma from pulmonary adenocarcinoma. This was achieved by immunohistochemical analysis.
Immunohistochemical (IHC) analysis of the left posteroinferior pleural and right adrenal masses showed similar immunoreactivity. Neoplastic cells were strongly positive for cytokeratin 7, focally positive for calretinin and cytokeratin 5/6, and negative for thyroid transcription factor-1 (TTF-1) (see ). This profile was consistent with primary malignant mesothelioma of the left pleural mass and metastatic mesothelioma of the right adrenal mass. IHC analysis is typically used for diagnosing and differentiating mesotheliomas from other lung cancers. This requires the utilization of a panel of immunostains such as TTF-1, calretinin, CK5/CK6, MOC31, B72.3, and BG8. Anti-calretinin antibody is the most specific marker of epithelial mesothelioma, since it exhibits both cytoplasmic and nuclear distribution patterns. TTF-1 is found only in thyroid-derived tissue and lung carcinomas. CK5 and CK6 are strongly positive in epithelial mesotheliomas, while pulmonary adenocarcinomas are predominantly negative. Conversely, both lung carcinomas and mesotheliomas are strongly immunoreactive with CK7.


A levator claviculae muscle () was found unilaterally, in the right side of a 65-year old male cadaver, during a routine dissection in our Laboratory. It was a flat and longitudinal muscular slip originating from the transverse processes of the 3rd - 5th cervical vertebrae. Then, it pursued an oblique course, inferiorly, anteriorly and slightly laterally, crossing over the brachial plexus and omohyoid muscle to insert into the acromial extremity of the clavicle. As far as the neurovascular supply of the levator claviculae muscle is concerned, a motor branch of the C4 nerve and the ascending cervical artery supplied this supernumerary muscle.


A 59-year-old, Caucasian (Greek origin), multiparous (Gravida 3, Para2, two spontaneous vaginal deliveries, housewife) was hospitalised in the medical ward complaining of dyspnoea and abdominal distension progressing during the last eight months. She was 1.62 m high, and she was weighing 73 kg at admission. She had no significant medical and surgical history, and she did not smoke or drink. Initial abdominal imaging with computed tomography and abdominal ultrasound showed huge ascites and suggested no signs of ovarian or other intra-abdominal pathology (). Biochemistry was normal except for liver function tests that appeared moderately elevated. Tumor markers were normal except CA-125 which was 300 U/ml. Serum-ascites albumin gradient was 1.30. There was a history of hepatitis B infection, but at that time the patient was receiving no treatment for the liver. The patient underwent serial drainage of approximately 35 litres of fluid to relieve symptoms. Cytology of the aspirated fluid was normal. The patient was discharged on spironolactone and furosemide and was examined on an outpatient basis. Six months later she was admitted again for recurrence of symptoms. Further drainage removed another 14 litres. Repeat CA-125 was within normal limits. A new sonographic approach (ALOKA, SSD-1700, transabdominal transducer 3.5-5.5 MHz) after paracentesis revealed normal uterus and left ovary, an increased vascularisation of the right adnexa on the colour flow mode, and the liver characteristically repelled on the back wall of peritoneal cavity, findings compatible with those of a giant ovarian cyst (-). A new computed tomography confirmed the sonographic diagnosis. At subsequent laparotomy the patient underwent excision of the giant cyst and a total abdominal hysterectomy with bilateral salpingo-oophorectomy. Pathology examination showed that the lesion was a mucosal-serosal ovarian cystadenoma of borderline malignant potential. The patient had an uneventful postoperative course and after 3 years of follow up she is doing well.


A 55-year-old Caucasian man from Germany presented in our tinnitus clinic with complaints of severe disabling right-sided tinnitus. The tinnitus resulted from sudden right-sided deafness, which occurred about one year before. In spite of different therapies such as intravenous steroids, infusion of rheological agents and tympanoscopy with grafting of the round window, the affected ear remained deaf. Subsequently, a pre-exisiting mild bilateral tinnitus exacerbated in the right ear (narrow band noise, 5 kHz). Due to the tinnitus the patient complained of insomnia and concentration problems resulting in severe disability of leading a normal life. Pure tone audiometry testing revealed right-sided deafness and moderate hearing impairment on the contralateral side (pure tone average of 500, 1000 and 2000 Hz: 37 dB HL). Electrical promontory stimulation via needle electrode demonstrated normal function of the auditory nerve. Tinnitus masking was impossible due to the right-sided deafness even when sounds were presented to the left ear. Tinnitus severity has been assessed with questionnaires [,] and visual analogue scales (VAS) (). Using a ten-point VAS the tinnitus was rated by the patient according to loudness and annoyance. Analysis of the scores showed severe impairment in consequence of the unilateral tinnitus. Magnetic resonance imaging (MRI) resulted in regular anatomical structures of the cochlea and the cranial nerves. No pathologic processes affecting the brain, the brainstem or the cerebellum were detected.
The deaf right ear was successfully implanted with a MedEL SONATAti 100 Cochlear Implant (MedEL, Innsbruck, Austria). Post-operative X-Ray revealed a correct position of the electrode in relation to the basal turn of the cochlea. Shortly after activation of the cochlear implant in the context of a standard fitting procedure the patient reported a reduction of his tinnitus, which completely disappeared during electrical stimulation within the following 3 months. With a deactivated implant the tinnitus only reoccurred after presentation to loud noise. The clinical improvement was also reflected by a distinct decrease in the scores of the questionnaires and the VAS (). Speech reception of monosyllables at 65 dB SPL improved from 0% preoperatively to 60% 3 months after first fitting and the patient reported no conflict of hearing between the implanted and the contralateral ear.


A 61-year-old Asian Taiwanese woman having pancreatic cancer with liver and left adrenal metastases presented with progressive dyspnea for one month. Chest examination revealed crackles at the bases. Cardiovascular examination showed no jugular venous distension and normal heart sounds. An echocardiogram showed normal left ventricular function. A chest radiograph () revealed diffuse reticular opacities and bilateral small amount pleural effusion. HRCT of the lung () showed thickening of peribronchovascular interstitium, interlobular septa, and centrilobular regions. Cytological examination of the pleural fluid revealed adenocarcinoma, most suggestive of a primary origin in the pancreas. However, there was no response to chemotherapy with gemcitabine and she passed away one month later.


A 47 year-old Asian female was transferred to the authors’ institution from another hospital with a subacute history of shortness of breath, bilateral lower extremity swelling, pleuritic chest pain, cyanosis, episodic loss of consciousness and hypotension. Two years prior to admission she had underwent an abdominal hysterectomy for uterine fibroids, which were complicated by symptomatic anemia from associated menorrhagia. Two months prior to admission, she sustained a brief loss of consciousness, then two weeks before admission she developed gradual onset of pleuritic chest pain and lower extremity edema. One day prior to admission, she complained of some shortness of breath and a non-productive cough. There were no known risk factors or family history of thromboembolism. She had no other significant past medical, surgical or obstetric history. She was a non-smoker, non-alcoholic and denied use of illicit substances. She had no history of drug allergy.
On the day of admission, she developed dizziness at home and fell to the ground. Her family helped her to bed wherein she had a witnessed four-minute period of loss of consciousness. She was taken by ambulance to an outside hospital where she appeared cyanotic and hypotensive with a weak pulse, was immediately sedated, endotracheally intubated and started on intravenous norepinephrine. Physical examination, prior to intubation, revealed a dusky blue skin suggestive of cyanosis. She had a tachycardia with pulse rate of 110 per minute, hypotension with systolic blood pressure of 70 mmHg and tachypnea with respiratory rate of 34 breaths per minute. There was teeth clenching and hypersalivation. An electrocardiogram and serum troponin levels were normal, her initial arterial blood pH was 7.25 with a PCO2 of 2.93 kPa (22 mmHg) and a PO2 of 67.46 kPa (506 mmHg) on an inspired fraction of oxygen of 1.0. The leukocyte count was 12.9 x 103 /μL with 81% neutrophils and 16.2% lymphocytes. Other hematologic and coagulation parameters were within the normal range. The serum bicarbonate was 14.6 mmol/L while other serum chemistries, renal and liver function tests were unremarkable. A computed tomography (CT) scan of the head was normal. A venous duplex ultrasound scan showed the presence of a right lower extremity proximal deep venous thrombosis (DVT) extending to the groin and she was commenced on intravenous standard unfractionated heparin.
Later that day the patient was transferred to our hospital where a pulmonary embolism protocol computed tomography (CT) scan of the thorax and pelvis (, , ) and a repeat transthoracic echocardiogram were performed (). Because of the positive venous duplex ultrasound finding of a DVT, a D-Dimer was not requested.
The patient was seen by a pulmonologist who made a differential diagnosis of an intracardiac tumor thrombus versus a thromboembolism-in-transit, and advised a surgical opinion. Because of delays in obtaining a concensus on appropriate surgical management from the cardiothoracic, vascular and gynecologic surgeons together with the need for further imaging studies, ongoing hypotension, presumed transient complete occlusion of the tricuspid valve and a confirmed deep venous thrombosis, the patient was initially thrombolysed intravenously with 100 mg of tissue plasminogen activator to treat any life-threatening component of venous thrombosis. Following this, the patient’s hemodynamic indices improved only slightly. The next day, the patient underwent a joint procedure with single stage median sternotomy and exploratory laparotomy, extraction of an inferior vena cava, common iliac vein and right atrial tumor thrombus, and included bilateral salpingo-oophorectomy. The histopathology of the tumor was consistent with leiomyoma ().
Post-operative transoesophageal echocardiogram and CT showed complete removal of the intravascular lesion with minimal residual pelvic mass. There were no complications and the patient was subsequently taken off pressors, extubated and discharged to follow-up on oral anticoagulation.


This 14-year-old Turkish male patient from Azerbaijan had a history of right focal seizures that began when he was 3 years of age. His previous examination and therapy was significant in that he experienced episodes of focal seizure until 6 years of age. He was treated with antiepileptic medicine (Phenobarbital), and a cranial MR image showed a left frontal mass. He underwent a craniotomy, and a biopsy was done during the first operation. Histopathological examination revealed a Dysembryoplastic Neuroepithelial Tumor (DNET). His seizures decreased over one year (Phenobarbital was continued for one year), then were lost without treatment of antiepileptic medicine until 12 years of age. He had experienced medically refractory complex partial seizures to sodium valproate with loss of consciousness. His neurological examination found no significant deficits. Magnetic resonance (MR) imaging of the brain showed a cystic mass in the left frontal lobe, directly adjacent to the pre motor area gyrus without gadolinium enhancement (). An electroencephalography (EEG) showed an epileptic wave focus on the left frontal lobe. Meanwhile, the patient's coagulation parameters, such as prothrombin time, activated partial thromboplastin time, international normalized ratio and thrombosit count, were within normal limits. The patient underwent a craniotomy for a left frontal tumor resection. Perioperative dehydration was achieved by a diuretic agent (Furosemide, 20 mg/2ml) and the infusion of 20% mannitol (250 ml). The brain was shrunken by the administration of the above agent. The aggressive anti-edema procedure caused the brain to become very slack.
Surgery was completed without complications. An epidural or a subgaleal drainage tube was not used. During surgery, the patient's mean arterial pressure ranged between 60 and 105 mm Hg. Histopathological examination of the surgical specimen yielded findings consistent with Dysembryoplastic neuroepithelial tumor.
Postoperative Course: The patient was transferred directly to ICU after surgery. He was recovered in ICU for six hours postoperatively. First hours post recovery period (7 hours postoperatively), the patient was somnolent and on neurologic examination, had right hemiparesis. The mean arterial pressure was between 55 and 100 mmHg.
Immediate postoperative brain computed tomography showed massive edema that caused a disappearance of the resected mass cavity and a suspected linear hemorrhage in the right cerebellar hemisphere in the maximal diameter 15 × 20 mm ().
Anti edema treatment was achieved with 20% mannitol (100 ml infusion per six hours for two days). He had an episode of focal seizure, four times with a mean duration of two minutes on the first and second postoperative days. Postoperative first day MRI confirmed the cerebellar hemorrhage ().
A CT scan of the patient's head was obtained on postoperative day five and revealed a significant decrease in the cerebral and cerebellar edema, the previous surgery cavity reappeared and the cerebellar hematoma resembled the immediate postoperative brain CT. He required two antiepileptic combination treatments (Lamostrigine and phenytoin sodium). The patient had no seizure activity after starting the antiepileptic combinations. He had no significant neurological symptoms, and he was transferred to the ward on postoperative day five. He was discharged at day eight postoperatively, his postoperative follow-up history revealed that the patient had no seizure activity, and a neurologic examination was conducted on 12th postoperative month.


This case describes a Caucasian 13-year-old boy who presented motor difficulties from birth. He came to our attention after an episode of hypertonia, fixed gaze lasting 1-2 minutes and loss of consciousness. Biochemical analysis, performed during hospitalization in the nearest hospital, showed low calcium concentration (1.1 mmol/l = 4.4 mg/dl) for which he was treated with calcium gluconate 10% ev. He also had elevated PTH concentration (177 pg/ml, reference value 17-73) and hyperphosphataemia (3.16-3.26 mmol/l = 9.5-9.8 mg/dl). The ECG showed a long Q-T interval.
On his admission to our paediatric unit he was in a good general condition, alert and collaborating, orientated in space and time. He did not show signs of tetany. Clinically he appeared tall, with tapering fingers, with neither teeth diastasis nor enamel alterations. He showed mild dysmorphic facial traits, bull neck, kyphosys, barrel-shaped thorax, pseudogynecomasty, but neither rounded face nor brachydactyly, or brachymetacarpia. The biochemical analyses confirmed the hypocalcaemia, hyperphosphataemia and the elevated concentration of PTH with low-normal vitamin D metabolites serum concentration (25OHD: 17.1 ng/ml, reference value 10-68; 1,25(OH)2D: 25.1 pg/ml, reference value 15-50) and renal function. There were no other endocrine defects (IGF-I, gonadotropin, testosterone, prolactin, cortisol, adrenocorticotropic hormone were all within the reference range). During hospitalization the calcium gluconate infusion was continued and then converted to oral calcium carbonate (2 g/day) associated with oral calcitriol (1.25 mcg/day).
During the following months several therapeutic adjustments were needed to maintain an adequate calcium-phosphate balance. Currently the patient has achieved a satisfactory calcium-phosphate homeostasis with 1.5 mcg/day of calcitriol.


A Caucasian 10-year-old boy initially came to our attention for rapidly progressing early puberty. After clinical examination and biochemical hormonal analysis the diagnosis was compatible with a heterozygous deficit of 21-hydroxylase.
Six months later he presented an episode of tonic-clonic seizures associated with loss of consciousness, overflow of saliva, fixed gaze to the right, resolved spontaneously after few seconds. For this reason a computed tomography (CT) scan and a Magnetic Resonance Imaging (MRI) scan of the brain were performed together with an Electroencephalogram (EEG) (basal and after sleep deprivation): all these investigations were normal.
At the age of 11 he referred to the emergency room of our hospital with a lipothymic episode that occurred while playing. Several biochemical examinations were performed: a severe hypocalcaemia (1.75 mmol/l = 7 mg/dl) was detected. Considering the previous convulsive episodes, the boy was hospitalized for further analyses. The calcium-phosphate metabolism revealed a low-normal calcium serum concentration (calcium 2.05 mmol/l = 8.2 mg/dl) and a phosphataemia at the upper normal limits (2.14 mmol/l = 6.42 mg/dl) with a moderately elevated PTH (77 pg/ml, reference value: 17.3-72.9). The thyroid ultrasound scan was normal. At the clinical examination the patient showed a brachymetacarpia of the fourth finger in both hands (this trait was shared by his mother and his grandfather). He did not have any other dysmorphic trait. He began an oral therapy with calcium carbonate (0.5 g/day) and calcitriol (0.25 mcg/day).
A month later his calcium-phosphate metabolism was partially normalized with a phosphataemia still at the upper limits (2.3 mmol/l = 7 mg/dl). In the following months the phosphataemia progressively lowered to the normal values and the patient did not present other convulsive episodes.


A Caucasian 12-year-old boy came to our attention with a history of two syncopal episodes. The first one had occurred four months before while cycling (the physical stress was associated with an emotional stress because of the bursting of a firecracker). During the following hospitalization the electrocardiogram (ECG) showed a long Q-T interval and the biochemical analyses revealed a mild hypocalcaemia. The echocardiogram and all the ergometric tests were normal. He began a therapy with Nadolol 40 mg/day and the molecular analyses for the long QT syndrome (LQTS) were performed. The cerebral CT scan and the neurological examination were normal. The EEG showed minimal non specific dysfunctional notes.
Persistent hypocalcaemia (2.1 mmol/l = 8.4 mg/dl) with high phosphate serum concentration (2.6 mmol/l = 7.8 mg/dl) was still present a month later.
A second syncopal episode occurred. This time it was preceded by dizziness and coincided with head rotation to the left and hand twitch contractions. During hospitalization the biochemical analyses showed alterations typical of pseudohypoparathyroidism: hypocalcaemia (1.9 mmol/l = 7.6 mg/dl), hyperphosphataemia (3.16 mmol/l = 9.5 mg/dl) and elevated PTH levels (1157 pg/ml, reference values 12-72). The EEG confirmed the non specific disfunction pathway. He started an oral calcium supplementation with calcium carbonate 1 g/day.
This patient finally came to our Institute after a month of calcium treatment. At the clinical examination, signs of latent tetany (Trousseau and Chvostek signs) were present and he had a mild kyphosis attitude. The boy did not show the peculiar clinical features of Albright hereditary osteodistrophy. He began a therapy with calcitriol 0.25 mcg twice a day and calcium carbonate (1 g/day divided in four doses). Ten days later the calcium was slightly higher while the phosphate value did not change. With several therapeutic adjustments the phosphataemia progressively normalized in the following three months and the latest biochemical analyses, performed 6 months after the beginning of the combined therapy (Calcitriol + Calcium Carbonate), showed a normal calcium, a slightly high phosphate, but reduced if compared to the previous value, and a PTH that decreased from 1088 to 162 pg/ml.


A Caucasian 8-year-old girl came to our outpatient clinics presenting as markedly overweight and with the presence of premature thelarche. Her height was 121.2 cm (3rd-10th centile) and her weight 39 kg (>90th centile) with a BMI of 26.7 (>97th centile). She had stretch marks at her hips, acanthosis nigricans on her neck and chest and cellulite. The mammary glands had a diameter of approximately 1 cm. This precocious puberty associated with skeletal abnormalities (she had a marked hypoplasia of the third, fourth and fifth metacarpals shortening and a dysmorphia of the carpal nucleus with a reduced carpal space) found in a radiograph of the left hand and wrist had raised the suspicion of an Albright hereditary osteodistrophy. Several biochemical examinations were performed: all results were within the normal range, except for an elevated PTH (127 pg/ml, reference value 17.3-72.9). The calcium phosphate metabolism, the phenotypic aspect (rounded face, short stature, bull neck, squat hands and feet, epicanthus) and the brachymetacarpia strengthened the diagnostic hypothesis of an Albright hereditary osteodistrophy. For this reason a genetic analysis was planned (still in course).
During the following months she repeated the biochemical analyses, which confirmed normal calcium and phosphate levels, normal 25OHvitamin D and elevated PTH. Two tests (arginine and dexamethasone) were compatible with a growth hormone deficit. The phosphate renal transport (TRP) and the phosphate maximum transport (TmPO4) were elevated.
summarizes the biochemical values showed by the four patients at arrival to our Clinic.