Almost two years ago, a 64-year-old Caucasian man was referred to our Cardiology Department for evaluation of back pain and shortness of breath on minimal physical activity. He had a long medical history, including surgical repair of aortic coarctation by Dacron patch plasty at the age of 26, with residual systemic hypertension. He also underwent bi-leaflet prosthetic valve (Sorin Biocarbon 27) implantation for severe degenerative stenosis of the bicuspid aortic valve at the age of 54. Twelve years previously he had been referred to hospital for ischemic stroke with left hemiparesis. On that occasion he was diagnosed as having a 5cm diameter asymptomatic aneurysmal dilatation of the thoracic aorta at the site of the first surgical procedure. Given the lack of symptoms at that time, the decision was made to follow him with regular reassessments.
Twelve years later, given the onset of symptoms, he was hospitalized in our department. At admission, physical examination revealed stable vital signs, blood pressure at 160/80mmHg, normal heart rate at 64 beats per minute, stony dullness to percussion of the left posterior thorax, and no evidence of left ventricular dysfunction at rest. An electrocardiogram showed sinus rhythm, first-degree atrioventricular block with a PQ interval of 280 milliseconds, left bundle branch block and rare ventricular premature beats. Conduction abnormalities had been already documented during previous evaluations.
Initial evaluation consisted of a plain chest radiograph, which showed a well-contoured opacity with a diameter of approximately 10cm, next to the left border of the aorta (FigureA,B). The chest contrast-enhanced computed tomography (CT) scan confirmed the presence of a large dilatation of the thoracic aorta (FigureA,B). Transthoracic echocardiography found good left ventricular systolic function, normofunctional aortic prosthesis, no abnormalities of the native valves, absence of pulmonary hypertension, absence of pericardial effusion, and a dilated (49mm) ascending aorta and aneurysmal descending thoracic aorta. Coronary angiogram and aortography performed in the same session showed no significant lesions of the epicardial vessels and location of the aortic aneurysm immediately below the left subclavian artery.
A multidisciplinary team, including a cardiologist, cardiovascular surgeon, angiographist and radiologist, decided on a hybrid resolution of the aneurysm.
One year after the last evaluation successful exclusion of the aneurysm was carried out by percutaneous stent-graft implantation (Proximal Free Flow Straight Valiant Thoracic® TF3232C200X, Medtronic Inc., Minneapolis, MN, USA) using a right femoral approach (FigureA,B). The stent-graft partially covered the ostium of the left subclavian artery and a paraprosthetic leakage was observed (FigureC). The percutaneous intervention was continued with the surgical transposition of the left subclavian artery to the left common carotid artery within the same session. The post-procedural debranching had to be done because of the occlusion of the left subclavian artery during the procedure. Pre-procedural evaluation had not suggested this, but at the end of the interventional procedure the final angiogram revealed a partially covered ostium of the left subclavian artery by the stent-graft. The surgical possibilities for debranching in this situation were carotid-subclavian bypass or transposition of the left subclavian artery to the left common carotid artery. We chose the second procedure because of the presence of a paraprosthetic leakage originating from the left subclavian artery. The closure of the subclavian artery with sutures resolved both problems: the leakage and the left upper arm and vertebral artery vascularization. Both procedures were successful. Post-procedural evolution was uneventful, except for a fever of a few days duration of up to 38°C, which was related to post-implantation syndrome, with no evidence of infection and mild residual dysphonia. Three months after the procedure, our patient was clinically well without any clinical signs of left lower limb ischemia or new onset neurological abnormalities. Contrast-enhanced CT revealed complete thrombosis of the excluded aneurysm (FigureA,B).


A 20-year-old Japanese woman presented to a local hospital with lower abdominal pain. She had mental retardation and epilepsy, and was unable to indicate her intentions. She was found to have a multicystic tumor in her pelvic cavity and was referred to our gynecologic department. Her lower abdomen was soft and showed slight swelling without tenderness. Magnetic resonance imaging revealed that a multicystic tumor occupied her abdominal cavity. Because the tumor did not have a solid component or enhancement with gadolinium enhancement and tumor markers were normal (carbohydrate antigens [CA] 125, CA19-9 and carcinoembryonic antigen), a benign ovarian tumor was suspected. A laparotomy was performed, unexpectedly revealing swelling of her bilateral ovaries. The right ovarian tumor was the size of an adult head and the left ovarian tumor was the size of a fist. A right oophorectomy and left ovarian cystectomy were performed. Gross findings were that the bilateral ovarian tumors seemed benign without a solid or papillary component. The pathological diagnosis of the right ovarian tumor was mucinous cystadenoma; however, the pathological diagnosis of the left ovarian tumor was an intestinal type mucinous borderline tumor. Our patient did not receive any adjuvant therapy and we planned to follow up every three months.
Two years and nine months after the primary surgery, a multilocular tumor, approximately 10 cm in diameter, was detected in her pelvic cavity on follow-up ultrasonography. A computed tomography scan revealed a multicystic tumor in her pelvic cavity, without contrast by a radiopaque agent (Figure ). Magnetic resonance imaging revealed round cystic lesions in her pelvis, with low to intermediate intensity on T1-weighted images and high intensity on T2-weighted images (Figure ). A small part of the cyst wall was slightly enhanced with the administration of gadolinium. The preserved left ovary was not separately identified. Therefore, a recurrent ovarian borderline tumor was suspected.
Our patient could not give her own opinion regarding her disease so her parents made all decisions regarding her treatment. Our patient had suffered from dysmenorrhea for a long time and her parents therefore requested removal of her uterus and remaining ovary.
Our patient then underwent a second surgery and a multicystic tumor measuring 10 × 10 cm was found in her pelvic cavity. The tumor consisted of many non-isolated cysts of diverse sizes (Figure ), which adhered to the surface of her ovary, mesentery, mesocolon and pelvic wall. Slight transparent ascites was present. Her uterus was normal and her left ovary was thumb-sized without swelling. A hysterectomy, left oophorectomy, omentectomy, pelvic lymph node biopsy, appendectomy and tumorectomy of her abdomen were performed. Complete resection of the lesion was achieved.
On histology, epithelium with a mucinous component was not seen in the cyst or cyst wall (Figure ). In the removed left ovary, a tiny component of mucinous cystadenoma was present though neither a mucinous borderline tumor nor a mucinous adenocarcinoma was present. Immunohistochemical staining of the lining cells of the cyst wall were positive for calretinin and Wilms’ tumor protein and negative for D2-40. The removed uterus, lymph nodes and omentum were normal. Our patient was finally diagnosed with BMPM. She recovered uneventfully and was discharged from the hospital on the seventh day after the operation. Our patient received estrogen replacement therapy and remains free from recurrence nine months after the second operation.


A 57-year-old male patient was admitted to our clinic with a five-month history of multiple bilateral nodules in the neck. He had cough, expectoration, and low fever in the previous one month. A thoracic-computed tomographic (CT) examination revealed a 4.5 × 3.0 cm mass in the right lower lobe, with enlargement of multiple mediastinal lymph nodes ().
Subsequently, he underwent CT-guided fine needle biopsy of the right lower lobe and biopsy of the right cervical lymph node. Microscopic examination revealed poorly differentiated squamous cell carcinoma of the right lung and mantle cell lymphoma of the right cervical lymph node. The patient received 2 cycles of preoperative chemotherapy. Subsequently, he underwent a lower lobectomy of the right lung and received 4 cycles of postoperative chemotherapy.


A 7-year-old boy was referred to our department for the evaluation of a mass in the left upper cervical region. The child described a painless tumor that had been progressively increased in size for the last six months without any associated symptoms. The child was known to be healthy, vaccinated without any specific medical problems. Physical examination showed a soft, deep, and immobile, noninflammatory, 3.0 × 2.5 × 2.0 cm left parotid mass associated with left facial paresis grade III (House-Brackmann classification). Multiple left upper cervical enlarged lymph nodes were noticed. The largest one reaching 2.0 × 2.0 × 2.0 cm was hard and mobile.
The remaining otolaryngology exam was within normal limits. Serologies for Epstein-Barr virus, cytomegalovirus, Bartonella henselae as well as tuberculin PPD test (purified protein derivative) were all negative. Cervical magnetic resonance imaging (MRI) () showed a large tumor with heterogeneous signals of left parotid gland with multiple necrotic areas, associated with multiple jugulodigastric and retrocervical lymph nodes. A fine-needle aspiration (FNA) revealed atypical cells without a definite diagnosis; open biopsy of the cervical lymph nodes showed a metastatic carcinoma consistent with myoepithelial carcinoma of salivary gland origin. Additional workup including chest X-ray, brain MRI, and a computerized tomography (CT) scan of the abdomen were within normal limits. Subsequently the patient was planned for surgery.
Left total parotidectomy with modified neck dissection of the cervical areas I, II, III, and IV was performed. The tumor was of 3.0 × 2.5 × 2.0 cm lobulated, dark red color invading the superficial and deep lobes of the parotid gland. The main trunk of the facial nerve and its subdivisions were totally invaded by the tumor. Thus sacrificing the facial nerve was necessary for a one-block excision of the tumor. A left sural nervous graft of 8 cm, with two branches (Y shape), was grafted between the main trunk proximally and the mandibular as well as the zygomatic branch distally. The accessory nerve, internal jugular vein, and the sternocleidomastoid muscle were preserved. A frozen section of the parapharyngeal fat was negative for malignancy.
The patient had an uneventful postoperative course and was discharged home on the fourth postoperative day with a total left facial nerve paralysis.
Histopathology showed a 3.0 × 2.5 × 3.0 cm high-grade myoepithelial carcinoma () replacing most of the gland, extending to the extraglandular tissues, invading the facial nerve with lymphatic vascular invasion. The margins were free of tumor. Metastatic carcinoma was present in 7 of 14 periglandular nodes and in 6 of 47 left cervical nodes.
The tumor was composed of nests and sheets of spindled and epithelioid cells with areas of necrosis. Immunostains showed that the tumor cells express cytokeratin, EMA, cytokeratin 7, smooth muscle actin, P63, CEA, and S100 and are negative for desmin, LCA, CD34, and CD20. This pattern of immunostaining is consistent with the diagnosis of myoepithelial carcinoma.
Because of the aggressiveness of this tumor, the patient underwent postoperative radiotherapy, despite the lack of clear guidelines in the literature. MRI and whole body positron emission tomography (PET) scan performed six months later were normal. Unfortunately, on the postoperative tenth month, the patient presented a pulmonary and lumbar vertebra metastasis.


A thirty-five year old, 34-weeks pregnant woman suffering from hypertrophic obstructive cardiomyopathy diagnosed when she was thirty presented to the emergency room reporting a dyspnea NYHA class IV, chest pain, and heart palpitations. Notably, the family history did not include cardiological disorders or unexpected death due to cardiac dysfunction. The patient was followed for many years. The patient was never severely symptomatic and had a normal tolerance of effort and of controlled physical activity. In particular, she never presented with heart palpitations or dyspnea. She has been treated chronically with metoprolol. The course of the pregnancy remained asymptomatic. Her pulse was 95 bpm. Humeral arterial pressure was about 105/70. An elective systolic murmur mesocardiac point, accentuated by the valsalva manoeuvre, was auscultated. There were no signs of jugular turgor. Her respiratory rate was 18 breaths per minute with 98% oxygen saturation on room air; there were no pulmonary pathological noises. Electrocardiography revealed sinus tachycardia, left ventricular hypertrophy by voltage criteria, and ST segment depressions with T wave inversions in the lateral leads. A chest radiograph was normal contrasting with the severity of dyspnea. Arterial blood gas values were PH = 7.51, PaO2 = 89 mmHg, and PaCO2 = 27 mmHg. She was anemic at the time of admission (hemoglobin = 8.9 g/dL). Other laboratory values were white blood cell count 12.48 ∗ 103 mL, platelet count 135 ∗ 103/mL, calcium 9.6 mg/dL, potassium 4.0 mEq/L, and creatinine 0.63 mg/dL. Two-dimensional echocardiography demonstrated an aortic root of normal diameter (30 mm), an aortic tricuspid valve with normal systolic spread, a left atrium with slightly increased dimension (30 cm2), a left ventricle with normal dimension and preserved systolic function (the ejection fraction was 65%), and a marked concentric hypertrophy of the left ventricule with midventricular systolic obliteration (intraventricular septum 20 mm, posterior wall 17 mm). The right atrium and right ventricle had normal dimensions, with normal right ventricular function. An elevated right heart pressure was observed. Moderate tricuspid regurgitation was recorded (Figures and ). Right ventricular systolic pressure was calculated to be 60 mmHg (). Continuous Doppler showed the presence of a mean midventricular gradient of 53 mm Hg and a peak gradient of 125 mmHg, a peak velocity of 5.5 m/s, and the presence of relaxation diastolic dysfunction (). In summary, the echocardiographic exam showed severe pulmonary hypertension, hypertrophic obstructive cardiomyopathy with a very hypertrophic left ventricle with normal internal dimension and global systolic function. Pulmonary embolism was suspected because of very high pulmonary hypertension and lungperfusionscintigraphywasperformed andshowedadefect of infusionof4 and 8segmentsof the right lung. Venous echo Doppler of lower limb was normal. Therapy with heparin was started and we decided to perform a caesarian under general anesthesia to give better haemodynamic control was recommended because of the elevated risk of heart failure and arrhythmias: Therapy with heparin was started and we decided to perform a caesarian under general anesthesia because of the elevated risk of heart failure and arrhythmias. At the anaesthesiological examination the patient was classified as anaesthesiological risk ASA IV and informed of the elevated anaesthesiological risk. The postoperative course remained stable. The systolic arterial pressure was about 100–115 mmHg and SpO2 remained stable about 99%. The new-born child was male, 2725 g in weight, 46 cm in length, and presented with Apgar indices of 6 and 9 at 1 and 5 min from birth, respectively. The postoperative course of the patient was regular, asymptomatic, and haemodynamically stable for the whole period of stay in the intensive care unit. The electrocardiographic monitoring showed periods of monomorphic ventricular tachycardia, with a maximal cardiac frequency of 150 heart beats. The patient was kept under observation in the postintensive care unit for 2 days before being discharged and retransferred to cardiology, where she continued her therapy with enoxaparin (6000 UI twice a day) and Bisoprolol (10 mg/day). The patient was discharged from the hospital a week after delivery. At followup of 12 months after birth, the child exhibited normal growth and development for his age and did not show any clinical abnormalities and the patient did not suffer from any symptoms. Midventricular peak gradient decreased from 125 mmHg to 54 mmHg during 4 months followup.


An 11-year-old girl (UPN1) was referred for evaluation for matched sibling bone marrow transplantation (BMT) for treatment of MDS. She was the youngest of three children born to unrelated parents. The first male born to her parents was small for gestational age, weighing 1.87 kg at term. He had severe chest deformity (babygram shown in Figure ) and died of respiratory distress at four hours of age. A diagnosis of probable ATD was made although no genetic testing could be performed, as causative genes were unknown at that time. The second was a well, unaffected male (who subsequently became the bone marrow donor for UPN1).
UPN1 was born at 40 + 6 weeks gestation by a normal vaginal delivery. Her Apgar scores were 6 at one minute and 8 at 5 minutes and she required suction and oxygen by mask. Her birth weight was 2.59 kg, length 47 cm and head circumference 33 cm (all approximately on the 3rd percentile). From 12 to 36 hours of postnatal life she developed tachypnoea, reaching a maximum of 120 breaths per minute. There was no cyanosis. On examination she had an obvious narrow chest, poor chest expansion, hepatomegaly of 2 cm and significant tachypnoea but no other obvious abnormalities. Chest auscultation was clear and cardiac examination was normal. A chest radiograph (not available) showed a narrow chest with no evidence of cardiac disease and the lung fields were consistent with transient tachypnoea of the neonate. UPN1 was admitted to the neonatal unit and was discharged 48-hour later with mild tachypnoea only. A geneticist had reviewed her and a diagnosis of probable ATD was reached. At this time her haemoglobin and platelet counts were normal and her total white cell count was 5.2 x 109/l (no differential performed). Shortly afterwards her respiratory symptoms resolved completely.
Through the first two years of life head circumference stayed at the third percentile and her length percentile dropped. By two years she was 10 cm below the 3rd percentile for height and 2 kg below the 3rd percentile for weight. At 22 months of age she was admitted with sepsis with a short history of vomiting, pyrexia and a rapidly progressive purpuric rash. Meningococcal septicaemia was the presumed diagnosis and she was fully treated for this but meningococcus was not cultured and coliforms were grown subsequently from urine. Haemoglobin on admission was 4.6 g/dl, white cell count was 7.8 x 109/l (neutrophils 2.34 x 109/l) and the platelet count was 18 x 109/l. These abnormalities were presumed to be secondary to severe sepsis and she made a rapid and full response to intravenous antibiotics. Ten days later her neutrophils had dropped to 1.14 x 109/l but platelets had recovered to 53 x 109/l. There is no documented full blood examination until 5 years of age when she developed haemolytic uraemic syndrome (HUS) and required several weeks of dialysis; this was followed by intermittent, self-limiting episodes of haematuria with mild renal impairment. At the time of admission with HUS she was noted to have learning difficulties and short stature. Renal imaging at the age of 7 years with ultrasound and DMSA showed no renal scarring, malformation, dilatation or reflux. At this time and for the following 4 years her blood counts were normal (including neutrophils) apart from a platelet count ranging from 43-161 x 109/l but mostly in the range 50–100 x 109/l.
Between the ages of 9 and 10 years UPN1 progressed to being consistently pancytopenic (haemoglobin 6.8 g/dl, white cell count 1.8 x 109/l, neutrophils 0.47 x 109/l, lymphocytes 1.09 x 109/l and platelets of 33 x 109/l). She had no evidence of hepatosplenomegaly or lymphadenopathy. A bone marrow aspirate and trephine showed normal cellularity with trilineage dysplasia and no fibrosis. There was no excess of blasts initially, although repeat examination after referral showed 10% blasts and the marrow had become hypocellular. Cytogenetics revealed a deletion of 7p in a clone on the initial bone marrow aspirate but serial testing eventually revealed three different clones, one with an isochromosome of 7q, an abnormality frequently described in SDS and rarely seen outside this syndrome []. Lymphocytes showed no increase in chromosome breakage compared to control in response to mitomycin C challenge, excluding Fanconi anaemia.
UPN1 became red cell and platelet transfusion dependent in the year leading up to transplant referral. During this time she suffered epiglottitis requiring intensive care, a prolonged episode of fever with a perianal abscess and periorbital cellulitis of one eye. Perianal soreness and infection became a recurring theme.
Specific questioning to exclude well-recognised causes of bone marrow failure revealed that UPN1 had passed 1-2 stools per day throughout her life, which were yellow, offensive and difficult to flush. Microbiological examination had been consistently negative. Subsequent examination revealed the presence of fat globules and a reduced faecal chymotrypsin (0.7U/g, normal range 6-99). Fat-soluble vitamin levels were reduced (vitamin A 0.6 μmol/l [reference range 1.1-3.5] and vitamin E 9.7 μmol/l [reference range 10.2-39.0]). UPN1 was growing but was still small for age; at eleven years and two months she weighed 22.9 kg (5 kg below 3rd percentile) and was 113 cm tall (approximately 20 cm below the 3rd percentile). Skeletal survey (See Figures , and ) revealed abnormalities in the metaphyseal regions, most prominent in the upper femoral neck and in the lower femoral and upper tibial metaphyses. These showed areas of lucency and sclerosis and irregularity. Coxa valga was present on the right and some broadening and coxa vara on the left. The ribs were a little shortened.
The radiographic changes on skeletal survey in combination with the evidence of pancreatic insufficiency, confirmed a clinical diagnosis of SDS as a cause of her chondroplasia and bone marrow failure. A DNA sample from UPN1 was later tested for conversion mutations of the SBDS gene. The restriction endonuclease digest assay confirmed mutations c.183_184TA>CT and c.258+2T>C. These account for 74% of SBDS mutations []. Testing of parental DNA also confirmed that one mutated allele was inherited from each parent.
UPN1 had significant learning difficulties and was in a special school. MRI brain was unremarkable. She was commenced on fat-soluble vitamins and pancreatic enzyme replacement with rapid improvement in stool frequency and consistency. An MRI scan of the abdomen showed that the pancreas was bulky and infiltrated with fat. At referral she was receiving packed cell transfusions every three weeks and platelets approximately weekly, but blood counts improved rapidly following pancreatic and vitamin supplementation. During the following 16 months, she required only one further packed cell transfusion and became platelet independent, although she did require G-CSF in order to increase her neutrophil count. She had minimal infectious complications. Due to this improvement, plans for transplantation were postponed.
When UPN1 was 12.5 years old, her blood count fell further, she developed pyrexia of unknown origin (PUO) lasting four weeks and was admitted for intravenous antibiotics. During this time her spleen enlarged to 14 cm below the costal margin, causing significant abdominal discomfort. The PUO settled and she was discharged but returned 4 months later with recurrence of fever and further progression of splenomegaly. Extensive imaging was performed during these two admissions. Chest radiographs and CT scans initially showed no focus of infection. There was hepatosplenomegaly and, of note, the pancreas was enlarged with markedly reduced attenuation consistent with being almost completely replaced by fat (See Figure ). There was also mural thickening of the sigmoid colon and rectum, suggesting neutropenic colitis/proctitis as the cause of PUO (See Figure ). Lung function studies revealed both FVC and FEV1 in the low normal range.
Progressive splenomegaly was thought to be related to progression of MDS and chronic G-CSF exposure as imaging showed no evidence of portal hypertension. UPN1 continued to have fevers resistant to intravenous antibiotics with no evidence of invasive fungal disease. Splenectomy was performed due to concerns that splenic sequestration was contributing to decreasing blood counts. Pathology revealed a large spleen (737 grams compared with mean weight for age being 89 grams) with several areas of infarction. The white pulp was atrophic and the red pulp congested. Microscopy showed areas of infarction with diffuse expansion of the red pulp, resulting from extensive infiltration of left shifted myeloid cells and clusters of atypical blasts. These features were thought to be attributable to MDS. Rectal biopsy was also performed, revealing features consistent with neutropenic colitis.
Persistent neutropenia, abdominal pain and recurrent fevers necessitated matched sibling BMT after conditioning therapy comprising fludarabine (125 mg/m2), melphalan (140 mg/m2) and alemtuzumab (0.9 mg/kg). This procedure was complicated by primary graft rejection, necessitating re-transplantation following administration of fludarabine (100 mg/m2), high dose methylprednisolone (10 mg/kg twice daily for four doses, then 2 mg/kg twice daily for four doses followed by slow weaning) and OKT3 (0.2 mg/kg for eight doses). This was followed by rapid and uncomplicated donor engraftment. Mixed T-cell chimerism led to donor lymphocyte infusions to reduce the risk of relapse of MDS. UPN1 is now over 5 years post-transplant and is well with normal blood counts. Whole blood and T-cell chimerism levels are 98% and 89% donor respectively. Hepatomegaly resolved rapidly following successful engraftment.


It was a 12 year-old girl, who had frequent cough and wheezing which required hospital visits and intravenous anti-asthmatic medication every year, since she was two years old. However, after she was ten her symptoms worsened when her wheezing became daily episodes accompanied with violent cough which could last for one or two hours before remission, making her difficult to lie down at midnight. She also reported sneezing, eye-itching and tearing before the onset of cough and wheezing, but no notable heartburn or regurgitation was recalled. During the years, despite her longing to improve grades in school, she often had to cut school due to hospital visits or fatigue, Her family had consulted five major hospitals in different provinces, in addition to almost all the local hospitals. The diagnosis was always the same: allergic asthma. Her spirometry test showed 16% improvement in FEV1 after inhaling albuterol and the only one positive skin prick test result was house-dust mites four years ago. Despite maximum dose of oral corticosteroid and Beta-agonists inhaling, or traditional Chinese medicine, her symptoms seemed to be uncontrollable. At last, she came to us for gastroesophageal reflux (GER) evaluation to find out if the asthmatic symptom is second to GER. The routine 24-hour pH monitoring showed pathological acid reflux (DeMeester score: 25.45), which was more severe in supine position. And the longest reflux (18.1 min) occurred at one midnight when she had an asthma attack. According to the data we concluded that her asthma was GER related. We conducted Stretta Frequency (SRF) procedure on the patient after having approval from the hospital’s ethics research committee and obtaining parental consent from the patient’s family. Since the day of the anti-reflux treatment, the patient can sleep well and until now her handicapping symptoms have disappeared for 31 months without medication.


A 73-year-old man was referred to the ENT Department of the University Hospital of Larissa complaining of persistent hoarseness and slowly progressive dyspnea during the past year. The patient had undergone microlaryngoscopy with biopsies for a left vocal cord tumour nine years ago and his histology examination revealed hyperplasia of the surface epithelium, focal keratosis, slight chronic inflammation, and subepithelial scarring. He was a smoker with medical history of chronic renal failure, coronary artery disease, and diabetes. Endoscopic examination revealed a large nodular lesion along the left true vocal cord with impaired laryngeal mobility ipsilaterally. Chest X-ray was normal. MRI scan showed soft tissue fullness in the area of the left vocal cord (). No lymph node enlargement was found in the neck, while the rest ENT examination was unremarkable. Microlaryngoscopy under general anaesthesia confirmed the existence of the lesion on the left true vocal cord and the extent of it in the immediate subglottic area. Biopsy specimens were taken and on histological examination the patient diagnosed as having IPT; abundant myofibroblasts with scattered inflammatory cells were seen, cellular pleomorphism was demonstrated, and mitoses were absent. Immunohistochemically stromal cells were positive for vimentin, SMA, and calponine; CD34 and S100 were focally positive, while ALK1 staining and desmine resulted in negative reactions ( and ). The patient subsequently underwent a median vertical thyrotomy under general anaesthesia, after the airway was secured by a tracheotomy at the beginning of the procedure. The lesion was excised including some surrounding muscle fibres, which appeared to be involved. Grossly, the tumour was firmly attached to the left true vocal cord and presented to be nodular, measuring in diameter 1.7 × 1.1 × 0.7 cm (). Prophylactic perioperative antibiotics were administered preoperatively and continued for five days postoperatively. The patient was decanulated on the third postoperative day and had an uneventful recovery. Two months postoperatively, the left true vocal cord had regained mobility and his voice had improved enough. No recurrence has been noticed eight months postoperatively.


A 34-year-old woman presented with occipital headache of sudden onset, episodic vomiting, and imbalance while walking. Neurologic examination revealed cerebellar syndrome, no sensory or motor deficit. CT and MR of the brain showed a large well-circumscribed, lobulated, heterogeneous space-occupying lesion in the right cerebellar hemisphere measuring 4.1 × 2.5 cm with a broad tentorial attachment (). It was predominantly hyperdense on plain CT and had heterogeneous low signal intensity on T1-weighted images, and nonhomogeneous central bright signal areas on T2-weighted images with fairly heterogeneous postcontrast enhancement. The left cerebellar hemisphere and vermis were compressed with a sharp interface between the lesion and brain. Because of its apparent extraaxial location and broad attachment to the tentorium, a working diagnosis of meningioma or medulloblastoma was entertained.
The patient underwent posterior fossa surgery. Intraoperatively the tumor was soft, easily aspirable with suction and could be clearly separated from the cerebellum. The tentorium was free of tumor, suggestive of an intraaxial lesion. Frozen section revealed malignant small round cells. Histopathological analysis showed nodules of small round cells with occasional neurocytic differentiation and surrounding collagen-rich tissue suggestive of desmoplastic medulloblastoma. Postoperatively, the patient had been given both radiotherapy and chemotherapy. She did well for 3 years with no evidence of recurrence on follow-up CT scans of the brain. She had no other complaints until she developed low back pain and sciatica. Plain radiographs of the lumbar spine showed mixed lytic and sclerotic lesions of the vertebral bodies of L5 and S1 (). The described findings presumed spondylodiscitis, and a biopsy of the vertebrae was performed under CT scan guidance. Histopathological examination was reported as primitive neuroectodermal tumor characterized by small, round, and anaplastic cells compatible with medulloblastoma. The patient was hospitalized and underwent a chemotherapy treatment protocol with good outcome for two years.


We report the MRI findings of an incidental focal liver lesion, which was detected by ultrasound (US) in a 75-year-old Albanian man treated for benign hypertrophy of the prostate. His physical examination and laboratory test results were unremarkable.
Multi-detector chest and abdominal computed tomography (MDCT), with intravenous administration of 100mL iodinated contrast material was performed using a Emotion 6 MDCT device (Siemens Medical Systems, Erlangen, Germany). MDCT scans showed a focal liver lesion in the right lobe, and a mild enlargement of the prostate gland. No other abnormalities were identified in the rest of his chest and abdomen. Subsequently, an MRI scan of the upper abdomen was performed for better characterization of this lesion.
An MRI scan was performed using a 1.5T magnetic resonance unit (Avanto; Siemens Medical Systems, Erlangen, Germany), using a set protocol including unenhanced sequences and enhanced sequences, obtained in early phase at 25 seconds after administration of intravenous contrast gadolinium (Magnevist, Bayer HealthCare) at 2mL/second, 45 and 90 seconds (late phase).
Two-dimensional gradient echo in axial plane was performed using these parameters: TR/TE 140 to 200/2.2 to 2.4ms, flip angle 80, slice thickness 6mm, image matrix 128 × 256 and acquisition time 20 seconds.
A three-dimensional gradient echo sequence (VIBE) with a fat saturation was performed before and after administration of intravenous contrast using the following parameters: 5.58/2.38ms, flip angle 10, matrix 320 × 256, slice thickness 3.3mm and acquisition time 20 seconds.
A dual T1-weighted spoiled gradient echo (SGE) sequences in phase/out-of-phase was obtained in the axial plane using the following parameters: 113/4.92/2.22ms, flip angle 70, slice thickness 8mm and acquisition time 22 seconds for each sequence.
T2-weighted half-Fourier acquisition single-shot turbo spin echo (HASTE) with and without fat saturation was obtained using the following parameters: 1000/94ms, flip angle 15, slice thickness 6mm, matrix 320 × 246 and acquisition time 22 seconds.


A 31-year-old African man presented to our emergency unit with a four-hour history of penile swelling and voiding difficulty. He had earlier tried to suppress an erection before his routine morning religious rituals by kneading on his erect penis. He heard a popping sound with associated minimal pain and sudden detumescence. He noticed no bleeding per urethra but subsequently had difficulty with voiding and urinary frequency. On physical examination, he had a grossly swollen and deformed penis with deviation to the right side (Figure ). A diagnosis of penile fracture with probable associated urethral injury was made. Bedside urine analysis results were negative for blood and he was taken for penile ultrasound and sonourethrography about an hour after presentation to the emergency unit. This was performed using a high-frequency 7.5MHz transducer with our patient in a supine position and the penis supported with towels between the thighs. A generous amount of sonographic acoustic gel was applied on the dorsum of the penis and images were obtained both in longitudinal and transverse planes beginning at the glans and proceeding proximally to the root of the penis. This was performed initially without urethral distension. A 16Fr Foley catheter was then placed at the urethral meatus with its balloon inflated with 2mL of water at the fossa navicularis. Retrograde urethral distension using normal saline was achieved via the catheter and sonographic images were obtained as above. The penile ultrasound showed a tear of the TA covering the ventrolateral aspect of left CC with associated hematoma in the proximal third of the penile shaft, and the real-time sonourethrography additionally revealed an intact urethra (Figures and A,B). He also had retrograde urethrography performed, which also showed an intact urethra (Additional file 1). Three hours after presenting to the hospital he had penile surgery via a subcoronal circumferential degloving incision. A Foley urethral catheter was placed pre-operatively to identify and avoid injury to the urethra. A 1.5cm tear of the TA in the ventrolateral aspect of the proximal third of the left CC just adjacent to the urethra was seen (Additional file 2). The hematoma was evacuated and the tear repaired with 2/0 silk with burying of the knots. An artificial erection created with intra-cavernosal injection of normal saline revealed an intact repair with no leakages. The wound was closed with 3/0 vicryl (Additional file 3). He was discharged 24 hours after surgery and advised to abstain from sexual activity for six weeks. At one-week follow-up, he reported return of good nocturnal erections and normal voiding.


A 42-year-old male patient was admitted to a peripheral hospital for headache and fever (39°C) 15 days after returning from Cameroon, where he had spent two months under suboptimal anti-malarial prophylaxis with doxycycline. Microbiological explorations, including thin and thick blood smears, were initially negative. However, the fever persisted and the haemoglobin level fell to 92g/L. The patient was referred to a university hospital with suspicion of a macrophage activation syndrome. A venous blood smear was performed on admission (i e, 10 days after onset of symptoms) and came back positive for P. falciparum (0.6% parasitaemia). A three-day course of oral atovaquone plus proguanil was effective, as assessed by fever clearance and disappearance of asexual parasite stages from subsequent blood smears. Few mature stage V gametocytes were observed on thick blood smears at day 3 and day 7. A bone marrow biopsy was performed after two days of treatment to exclude other causes of fever and anaemia.
Stained histological sections of the bone marrow showed gametocytes at different maturation stages and numerous macrophages containing blood cells or malaria pigment. Gametocytes were readily identified and staged based on their oval or elongated shape and finely distributed pigment (Figure ). Only 29% (4/14) of stage V gametocytes were in extravascular spaces (Table ), the majority being in intravascular spaces (Figure C). By contrast, 89% (87/98) of immature stage II-IV gametocytes were observed in extravascular spaces (Table , Figure ) in immediate contact with erythroblasts (Figure B), adipocytes (Figure A) or other nucleated cells (Figure B). Several gametocytes did not co-localize with CD34-positive vascular structures (Figure D) or CD68-positive cells (Figure E).


A 25-year-old medically fit and well male patient presented twice within a two-week period at a dental emergency clinic. First presentation was due to a 7-day history of constant aching pain affecting the left side of his face, and the patient could isolate the pain to the left mandible. Extraoral and intraoral soft tissue examination was unremarkable. Periodontally there were no pathological pockets, and oral hygiene was good. Dentally the patient had an unrestored adult dentition with absent third molars and no detectable caries. The lower left first premolar tooth was markedly tender to percussion, and a long-cone periapical radiograph (LCPA) revealed a significant periapical radiolucency (). A diagnosis of acute apical periodontitis was confirmed although the cause of loss of vitality was unexplained, without apparent coronal or periodontal pathology being identifiable. The patient was offered extraction or pulp extirpation, and the patient opted to save the tooth. The tooth was successfully accessed and dressed with ledermix, cotton wool, and glass ionomer cement (GIC). The patient was discharged to find a general dental practitioner (GDP) to complete root canal treatment.
Two weeks later, the patient reattended the same dental emergency clinic complaining of constant aching pain affecting the lower right mandible. Once again, no decay or periodontal issues were found clinically, but LCPA examination of the lower right quadrant showed periapical pathology associated with the mandibular right first premolar tooth (). A diagnosis of acute apical periodontitis was confirmed, and the patient again had pulp extirpation completed.


A 48-year-old woman, with no family history of neoplastic diseases, underwent in 2005 to right pulmonary resection for a bronchial carcinoids and was in oncological follow-up for this reason.
In 2007, during a normal physical examination, a 2 cm sized nodule in her left thyroid lobe was palpated. The thyroid function tests were normal. The ultrasonography report revealed a solid hypoechoic nodule measuring 2.5 × 1 cm in the left lobe. A fine needle aspiration was attempted under ultrasound guidance and the final cytodiagnosis was papillary thyroid carcinoma.
Following cytodiagnosis, a total thyroidectomy was performed, but at the time of surgery, during the neck exploration, the patient was found to have bilateral enlargement of parathyroid glands with nodular aspect and all glands were resected. Histology confirmed the diagnosis of thyroid papillary cancer and revealed bilateral parathyroid adenoma.
In September 2008, a biochemical screening revealed high plasma levels of CgA of 2230 (normal 20–150) and serum gastrin (648 pg/mL). She was then referred to our hospital.
A 68Ga-DOTANOC-PET was performed and revealed 3 small areas of hyperaccumulation in pancreatic region and one in the liver.
For this reason, an EUS was performed and identified 2 nodular hypoechoic lesions of 5 mm with hypervascular Doppler pattern located in the body and in the tail of the pancreas. During the exploration, also a peripancreatic nodal involvement was found and the suspected metastasis (15 mm) of left liver was confirmed. We performed EUS-guided FNA with a 22G and a 25G needle, with on-site cytopathologist, and a Pan-NET (T) tumor () with (N) lymph node () and (M) liver () involvement was diagnosed.
A mutation of the MEN-1 gene was identified and, due to the high level of serum gastrin, a diagnosis of gastrinoma was reached.
She started therapy with octreotide LAR followed by receptor radiometabolic therapy with radiolabelled somatostatin analogues (177 Lu-DOTATATE) and she is presently alive.


A healthy, 47-year-old male presented with testicular discomfort and a palpable nodule in the upper scrotum just medial to the thigh. He reported that the nodule had been present for nearly seven years and had been slowly enlarging over the last few years. The mass had been initially asymptomatic, until recently when he began to experience some discomfort during exercise along with other activities. He denied any history of trauma or any prior surgeries to the area.
On physical exam, a palpable 3 cm soft structure was present in the right scrotum as shown in . It was minimally tender and mobile within the subcutaneous tissue, not adherent to the skin or spermatic cord. A scrotal ultrasound revealed a uniform, hypoechoic right extra-testicular lesion. The lesion appeared to be avascular. The mass did not originate from the scrotal wall, but was abutted by the lateral aspect of the right testis as shown in .
Given the low malignant potential, we did not send testicular markers and proceeded with excision of the mass. The cyst was found to be superior and lateral to the right testicle, but not originating from it. It was excised without difficulty. Intraoperative findings revealed the mass to be covered with a thin sac that contained necrotic, proteinaceous material. Pathological findings revealed benign epidermal inclusion cyst as shown in . The patient was seen in 1 month followup and recovered with no postoperative complications.


Revision laparotomy due to septic condition one day after radical right hemicolectomy showed an extensive infarction of the small bowel in a fifty-one year old woman as seen in . She had mesenteric venous thrombosis involving the ileum and the jejunum probably due to compromised blood flow in the superior mesenteric vein. The proximal part of the jejunum of about 80 cm (segment I), was slightly congested but appeared to be vital. The other part of the smaller intestine up to the ileocolic anastomosis (segments II and III) was congested and viability was highly questionable. The colon (segment IV) looked normal. Aiming to avoid short bowel syndrome, we decided to use LDF and spectrometry to save as much bowel as possible (O2C device, LF-2 probe, LEA Medizintechnik GmbH, Germany).
In this unknown situation we used general threshold values recommended by the manufacturer (microvascular haemoglobin concentration <90 units, microvascular flow >10 units, microvascular haemoglobin oxygenation >10%). At the time of measurement positive end-expiratory pressure was 5 mmHg, the patient was eucapnic and without hypoxia, her mean arterial blood pressure was 80 mmHg, the haematocrit was 34 percent, 30 micrograms norepinephrine per minute was administered and she had received pantoprazole, metamizole, midazolam, fentanyl, propofol, atracurium, metronidazole, cefuroxime, and imipenem.
Starting our measurements at the proximal part of the jejunum and proceeding stepwise towards the terminal ileum, we defined the cut margin just before the recommended threshold values were reached as seen in . The viable colon was used for reference measurement. Instead of 80 cm we could preserve 190 cm of small bowel. A split stoma was constructed to avoid primary anastomosis.
After operation lactate ion levels decreased to normal values, the stoma remained vital and no further surgical intervention was required. No long-term parenteral nutrition was needed.
The histological examination showed the haemorrhagic ischemic necrosis reaching to the cut margin as near as 5 mm ().


A 13 year-old boy presented to our paediatric gastroenterology outpatient clinic with a one month history of blood-stained diarrhoea, associated with urgency, weight loss of 1.5kg and asthenia. He had no complaint of abdominal pain, fever or anorexia. The onset of symptoms coincided with the end of a 4-day history of acute gastroenteritis.
His past medical history revealed a recurrent aphthous stomatitis, and his family history was positive for celiac disease (one maternal cousin), irritable bowel syndrome (one paternal uncle) and bowel cancer (paternal grandfather). His growth parameters were normal and the physical examination revealed palpable stool masses in the left lower abdomen. Based on the clinical presentation, the differential diagnosis included inflammatory bowel disease, celiac disease, infectious colitis and intestinal polyp. Laboratory investigations showed no biological markers for inflammation (normal full blood count, ESR: 5mm/h and CRP: 0.28mg/l) or celiac disease. Fecal calprotectin was not measured. Stool cultures for conventional enteric pathogens (Salmonella, Shigella Campylobacter) were negative. Upper and lower endoscopy revealed mucosal oedema in the sigmoid and rectum, which was probably due to the required bowel preparation for colonoscopy. Histological examination on conventional hematoxylin and eosin stain of the colonic biopsy specimens revealed a fuzzy band on the surface of the enterocytes. This finding was highly suggestive of intestinal spirochetosis (Figure
). Interestingly, no specific inflammatory reaction or lesions were observed. The diagnosis of intestinal spirochetosis was confirmed by Fluorescence in situ hybridization (FISH), confirming the presence of Brachyspira aalborgi (Figure
).
On the assumption that most intestinal spirochetes (obtained from veterinary specimens) are susceptible to amoxicillin, the patient was initially treated with this antibiotic (30mg/kg/day, divided into 3 doses) during 14 days, with subsequent cessation of rectal bleeding but persistent mucous diarrhoea. Additional treatment with metronidazole (30mg/kg/day, divided into 3 doses) for 10 days resulted in complete resolution of symptoms, therefore, no follow-up endoscopy was performed.


A 10-year-old girl underwent application of a humeral lengthening frame of her right humerus to correct severe humeral shortening gradually. The shortening was secondary to osteomyelitis of the right proximal humerus aged 10 weeks. This had left her with a 10-cm discrepancy between the lengths of the right and left humeri, resulting in significant functional difficulties. The lengthening frame was applied with the elbow extended without any intra-operative complications. In recovery, her right hand was warm and well perfused with full sensation and movement of the wrist and small hand joints. On the first post-operative day, however, she complained of severe burning pain in her right arm and hand requiring significant opiates for relief and a pain team review. Examination revealed evidence of a partial right radial nerve palsy; weakness of right wrist extension, an inability to extend the fingers and diminished sensation on the dorsum of the first webspace. She displayed evidence of hyperalgesia with an exaggerated response to pin-prick on the dorsum of the first webspace. There was no mechanical allodynia. All other hand and wrist movements and sensation were intact.
An ultrasound scan of the right arm was performed. This revealed no haematoma or collection. The radial nerve was not visualised.
On the second post-operative day, her symptoms decreased slightly. However, on the third post-operative day, her symptoms did not settle further and the patient was taken back to theatre for exploration of the radial nerve due to ongoing severe pain. The radial nerve was found to be in continuity without any bruising or contusion. Distally, it was found to pass tightly against the most distal external fixator pin such that, with the elbow flexed the nerve became tightly pressed against the pin. The distal pin was removed and re-sited slightly proximal.
Post-operatively, there was complete resolution of the right arm pain and hyperalgesia. Sensation to the first webspace recovered on the second post-operative day with motor function returning more gradually. There was full recovery of radial nerve motor function at 4 months post-operatively.


A 42-year-old woman was admitted to our intensive cardiac care unit with symptoms of acute left heart failure. Three months prior to admission, she underwent the implantation of a mechanical bileaflet mitral valve (25 mm SJM Master; St. Paul, MN, USA) due to severe rheumatic valve stenosis. No native valve- or chordal-sparing procedures were performed during the replacement. The postoperative course went smoothly and transthoracic echocardiography (TTE) showed proper functioning of the prosthesis. The patient then followed adequate anticoagulation treatment with wafarin and the international normalized ratio maintained above 2.0. No episode of atrial fibrillation and other risk factors for thrombus formation were identified. One week before this admission, she had noticed a progressive physical deterioration. After the treatment at local hospital, the patient showed no signs of recovery and finally presented with severe shortness of breath and coughing up pink, foamy mucus on the arrival of our hospital.
On admission, the patient was conscious but presented with marked respiratory distress. Initial vital signs showed blood pressure 80/40 mmHg, sinus rhythm, a regular pulse of 140 beats/min and a respiratory rate of 40 breaths/min. Auscultation of the chest revealed diffuse rhonchi and expiratory wheezes without any distinct heart murmurs. No signs of jugular venous distention and extremities edema were observed. The patient was intubated due to the deteriorated respiratory status. After intubation, bedside TTE demonstrated that the prosthetic bileaflet was immobilized and only a small transvalvular jet was observed. However, no paravalvular leakage, and vegetations or periannular abscesses that indicating endocarditis were identified (Figure A).
In view of the patient’s unstable hemodynamic conditions and echocardiographic findings, an emergent surgical intervention was performed. Intraoperative transesophageal echocardiography (TEE) demonstrated the immobilization of the prosthetic leaflets, which might be attributed to the acoustic shadowing around the valve ring (Figure B and Figure C). During surgical inspection, a noncircular fibrotic tissue ingrowth from the atrioventricular side was detected. The invaded pannus strictly adhered to the valve pivots and arrested both prosthetic leaflets (Figure ). The failed prosthesis was explanted and a new 27 mm SJM Master mechanical valve was implanted. The postoperative course was regular and in-hospital TTE showed normal functioning of the newly implanted mitral prosthesis. The patient had an uneventful recovery and was discharged home at two weeks after surgery.
On microscopic examination (Figure ), the resected pannus tissue was found to be mainly constituted with infiltrated leukocytes (neutrophils, macrophages, lymphocytes and plasma cells), pleomorphic spindle cells such as myofibroblasts, and interspersed capillary vessels.


A 66-year-old woman underwent mastectomy and axillary lymph node dissection for breast cancer. The histopathological findings were papillo-tubular carcinoma with metastases in two axillary lymph nodes. IHC staining showed that estrogen receptor (ER) and progestin receptor (PR) were positive, and Human Epidermal Growth Factor Receptor 2 (HER-2) oncoprotein was negative. As adjuvant therapy, doxorubicin (60 mg/m2) and cyclophosphamide (600 mg/m2) were administered four times and aromatase inhibitor was prescribed for five years. There was no post-mastectomy radiotherapy (PMRT). Nine years post-operatively, local metastases developed in the left axillary area. Chemotherapy (weekly paclitaxel: 80 mg/m2) and radiotherapy (total 30 Gy) were attempted, but the lesions continued to enlarge. On examination, a tumor measuring 5 cm in diameter was found in the left axillar area (Figure
A). Therefore, we added hyperthermia combined with radiotherapy and chemotherapy. Hyperthermia was performed using a Thermotron RF-8 capacitive heating device (Yamamoto VINITA Co., Ltd., Osaka, Japan at a surface temperature of 38°C to 41°C, once a week for 50 minutes
[]. The output power ranged from 1,300 to 1,400 W. After four weeks, the surface of the tumor began to dissolve, then became gradually necrotic (Figure
B). Along with a reduction of the tumor size, the foul odor disappeared. Eight weeks later, the tumor became nearly flat and the response was evaluated as a complete response (CR), according to the Response Evaluation Criteria in Solid Tumors Group criteria, and the patient noted improved activity in her daily life (Figure
C).


A 76-year-old man with lumbar spinal stenosis was admitted to our hospital with low back pain and bilateral sciatica. For his low back and bilateral leg pain, he had undergone L4/L5 non-instrumented intertransverse fusion with granules of HA for lumbar spinal stenosis in another hospital seven years before. After surgery, his complaint disappeared completely, however, after two years, he once again felt low back pain and increasing bilateral sciatica. On admission, he showed intermittent claudication after 20 meters, and lateral roentgenograms showed instability, suggesting failure of the attempted posterolateral intertransverse fusion. Computed tomography showed that hardly any bone had formed around the HA and no bony intertransverse bridge existed between the transverse process and HA (black arrow heads; Figure A). Magnetic resonance imaging (MRI) of the T2 wedged axial plane revealed a high intensity area between the mass of hydroxyapatite and the transverse process. Enhancement with gadolinium was observed around the transplanted HA, suggesting failure of the intertransverse fusion (Figure B). Decompression and instrumented intertransverse fusion with autologous iliac bone for L4/L5 were performed after removal of the HA granulation. Both sides of the granules of HA that formed an en bloc mass were resected obtusely and evaluated histologically.


46-year-old female patient whose blood group was “A1 Positive” underwent deceased donor liver transplantation (DDLT) from identical blood group donor for end-stage liver disease due to Hepatitis C with MELD score of 26. Pre-operatively, she had comorbidities in the form of diabetes mellitus and recurrent hepatic hydrothorax requiring frequent therapeutic aspiration.
Past history revealed that she had three children underwent Caesarean section during third delivery. She received two units of blood transfusion during Caesarean section, and there was no history suggestive of transfusion reaction following blood transfusion. In 2005, she was admitted in outside center for Hepatitis C-related liver disease. She received several units of fresh frozen plasma (FFP) and one unit of blood transfusion, which was followed by hemolytic transfusion reaction. Later, she did not receive any blood transfusions in other centers as cross-match was found to be incompatible.
She was worked up for liver transplantation in our center in February 2006. During preoperative evaluation, Indirect Antiglobulin Test (IAT) was positive and Direct Antiglobulin Test (DAT) was negative. She was found to have significant alloantibodies (1 : 256), specificity could not be typed as there were multiple antibodies. There were no cold and warm autoantibodies. DAT, IAT, and compatibility test were done by column agglutination technique (CAT).
She was given erythropoietin regularly during waiting period for liver transplantation and maintained hemoglobin level of 10-11 gm%. Preoperative autologous deposit and plasma exchange (to lower titers of alloantibodies) were not done as she was waitlisted for DDLT.
She underwent DDLT in February 2007. We had limited inventory of cross-match compatible A Positive red cell units at the time of her surgery. Stringent measures were taken by the transplant team during surgery to minimize blood loss and allogenic blood transfusions. It was decided to have lower transfusion triggers; Hb < 6 gms% instead of <8 gm% for red cell transfusion, to go for intraoperative red cell salvage, and to give antifibrinolytics during surgery.
Intraoperatively, Tranexamic Acid (TA) was given in a dose of 10 mg/kg as bolus, followed by TA infusion (5 mg/kg/hr) throughout the surgery. She received 6 units of Cytomegalo virus (CMV-) reduced risk and leuco-reduced Antiglobulin cross-matched compatible red cell units, 6 units of FFP, 10 units of pooled cryoprecipitate, and 1 unit of CMV-reduced risk and leuco-reduced apheresed platelets during surgery, apart from 460 mL of salvaged blood which was reinfused. Intraoperatively, there was no evidence of hemolysis. During surgery, she received Ceftizoxime 2 gm IV bolus followed by 1 gm IV tid which was continued in immediate postoperative period. Apart from this, she also received Levofloxacin and Flucanazole. She was given intravenous Heparin infusion intraoperatively for organized portal vein thrombosis. On day 1 of postoperative period, patient presented with hemolysis, which manifested as extra vascular hemolysis, positive DAT, clinically significant warm autoantibodies in a titer of >1 : 1024, increased reticulocyte count (2.2%), indirect bilirubin (3.3 mg/dL), and LDH (1278). After Ceftizoxime was withdrawn, clinically there was no evidence of hemolysis, reticulocyte count (0.8%), and LDH (320) dropped, but DAT remained positive, and titers of warm autoantibodies remained 1 : 1024 throughout her hospital stay. Alloantibodies titer dropped to 1 : 8 during postoperative period, which was probably due to the immunosuppressive drugs (Mycophenolate and Tacrolimus) which she received during postoperative period. She underwent reexploration on day 6, clot evacuated and hemostasis achieved. Later, she developed fungal sepsis due to mucormycosis and she was on liposomal Amphotericin B. She received 22 units of Antiglobulin cross-match compatible red cell units during postoperative period, which was uneventful.


A 51-year-old woman who had received conservative treatment (nonsteroidal anti-inflammatory drugs, physical therapy, and subacromial steroid injections) for 2 years for right shoulder pain was referred to our hospital because the shoulder pain was refractory to treatment. At the initial visit, on palpation, tenderness was noted over the lesser and greater tuberosities. Clinical evaluation revealed positive impingement signs [, ]. Test of the subscapularis musculotendinous unit, including the belly-press test and the lift-off test, was positive [, ]. The patient had nearly full active range -of -motion, but had a painful arc between 60° and 120°. The pain was increased on passive external rotation. Blood tests did not reveal any abnormal findings. Plain radiography showed calcified deposits in the upper and anterior parts of the humerus (Figures and ). Three-dimensional computed tomography (3D-CT) showed calcific deposits at the lesser tuberosity and the middle facet of the greater tuberosity (Figures and ). On the basis of these findings, we made a diagnosis of calcific tendonitis of the subscapularis and infraspinatus tendons and suggested arthroscopic removal of the calcific deposits.
The patient was placed in the beach-chair position under general anesthesia. Standard diagnostic arthroscopy was performed through a posterior portal. Despite careful exploration of the glenohumeral joint and subacromial bursa by using probes, it was difficult to locate the calcific deposits. Therefore, to locate and completely remove the calcific deposits, we decided to detach the subscapularis and infraspinatus tendons from their insertion. The distance between the bicipital groove and the calcifications was initially predicted on the basis of the aforementioned 3D-CT findings. Then, 2 incisions were made at the lesser and greater tuberosities by using the tendon of the long head of the biceps brachii as a landmark. Consequently, complete rotator cuff tears were made at 2 sites. After thorough removal of the calcific deposits, the cuff tear was firmly repaired using the suture-anchor technique.
The patient wore a sling for 6 weeks. Pain-free passive range of motion exercise started immediately from day 1 after surgery. After 6 weeks, active range of motion exercise was permitted, and a muscle-strengthening program was initiated 9 weeks after surgery.
Radiography performed 2 years after surgery revealed no calcifications, and the patient showed significant improvement with resolution of pain and improvement in the range of motion (Figures and ).


A 41-year-old woman with unknown native kidney disease received a renal transplant from deceased donor in January 2009. She was on treatment with immunosuppressive therapy based on tacrolimus, steroid, and mycophenolate mofetil. She presented 19 months after transplantation a wart on right index finger () which obtained partial clearance after 6-month treatment with cryotherapy and elettrocoagulation but it regrew rapidly. We attempted treatment with simple local application of activated vitamin D (gauze wet with calcitriol 0.5 μg solution) at least two times a day (during the morning and the next night). The patient was advised to reapply a gauze wet with calcitriol 0.5 solution after each handwashing. Three months later, the wart disappeared without pain or other side effects (), and it has not recurred within the 9 months since the disappeared. The medication was well tolerated. No adverse effects or abnormal serum test results, including elevated serum calcium level, were observed.


A healthy 57-year-old, male, Chinese with a good past medical health, right hand dominant worked as maintenance worker in a company. While working, a lamp metal bar cover fell on the right middle finger which causes laceration. Upon examination at the Accident and Emergency Department, bleeding wound further had examination under digital block anaesthesia with lacerated wound 3 cm obliquely at the posteroradial aspect of the proximal phalanx and 80% cut of the extensor tendon and no bony involvement. Radiograph findings revealed no bony involvement ().
Emergency surgical exploration at the operating theatre under digital anaesthesia with tourniquet was applied. The operative findings oblique laceration skin wound over posteroradial aspect of the proximal phalanx middle finger right, slight macerated of the skin edge 80% cut of the extensor tendon over the radial aspect (Zone IV), and branch of radial digital nerve was cut. No bony involvement was seen during exploration thus confirmed the plain radiographic findings. The procedures thorough debridement and irrigation, tendon repaired with 2-strands of 4/0 prolene using modified Kessler and 6/0 prolene continuous epitendinous stitch, digital nerve repaired with 4X 8/0 nylon epineurial stitches, and wound closured with 5/0 Nylon.
Following trauma and surgical procedure, extensive physiotherapy rehabilitation programme and occupational therapy treatment protocol was implemented. After the removal of skin sutures, a palpable mass was noted that begins as a painful over the scar which gradually becomes nontender and gradually increases, as well as firmer to palpation in progressive increase in size at the 19th day after the surgery with continuous extensor programme rehabilitation, and it develops gradual decrease of the range of motion on the metacarpal phalangeal joint as well as the proximal interphalangeal joint even with standard rehabilitation programme; later on, it develop into significant block of flexion and extension (). Radiographic imaging taken with calcifications was noted ().
An MRI + contrast performed after three months showed a finding that there is a heterogenous soft tissue lesion in the radial and extensor aspect of the shaft of the proximal finger, measuring 1 × 0.7 × 1.8 cm (cross section × long) in size. This lesion was T1W hypointense, T2W heterogeneously hyperintense with some enhancement. The MRI finding was a nonspecific local lesion underneath the extensor tendon of the middle finger at site previous injury. The extensor tendon is intact and elevated by the mass ().
This partly calcified bony tissue mass of size up to 18 mm × 10 mm × 8 mm mass was treated by a complete excision and approached the original surgical site at the posteroradial aspect of the proximal middle finger. The extensor tendon healed completely. A bony mass underneath the radial border of the extensor tendon had reached the surface of the proximal phalanx at the radial border (). A bony-calcified tissue was removed up to 18 mm × 10 mm × 8 mm in measurement (). The underlying proximal phalanx was intact, and there was no evidence of previous fracture.
Histological examination section showed a nubbin of bone surrounded by fibrovascular connective tissue. The marrow is predominantly fatty with prominent ectatic vessels and a patchy mild lymphocytic infiltrate. The patient recovered uneventfully after the operation. He regained full range of active and passive movements, and the neurovascular status was intact.


A 45-year-old woman presented to our heart transplant outpatient clinic complaining of anorexia, asthenia, and lethargy. She denied shortness of breath, fever, chills, cough, or any other symptom.
She had undergone orthotopic heart transplantation 5 weeks earlier for end-stage heart failure due to primary systemic amyloidosis (amyloid light-chain, AL) with severe cardiac involvement with restriction diagnosed only few weeks before. Her past medical history was only significant for pulmonary tuberculosis treated 20 years before. Before the transplant, she was assisted with an Extracorporeal Membrane Oxygenator (ECMO) device during 35 hours due to unstable clinical conditions, and she was then transplanted on an urgent basis. The donor was a 50-year-old woman died of cerebral haemorrhage. There was a mismatch between donor and recipient for Epstein-Barr virus and Toxoplasma but not for Cytomegalovirus. The postoperative course after the transplant was uneventful, and the patient was discharged home on day 15 postoperatively. The first two endomyocardial biopsies were normal.
On physical examination, the patient had a temperature of 38.5°C, heart rate of 110, blood pressure of 120/80 mmHg, respiratory rate of 20, grossly normal neurological status without any deficit, a normal cardiac auscultation, some bilateral pulmonary basal crackles, an unremarkable abdomen, and a mild peripheral oedema.
She was on tacrolimus 2 mg/day, mycophenolate mofetil 2000 mg/day, prednisone 7,5 mg/day, and aspirin 100 mg/day. She was not receiving any antibiotic prophylaxis. She had had tolerance induction therapy with two doses of daclizumab (1 mg for kg of body weight) on day 1 and 14 postoperatively. The patient was waiting for bone marrow autotransplantation as definitive treatment of her primary systemic amyloidosis.
Blood chemistry revealed a haemoglobin of 10 g/dL (normal: 12–14), WBC: 17000/uL and 83% neutrophils (normal WBC: 6000–9000, neutrophils 40–60%), sodium: 126 mEq/L (normal 135–145 mEq/L), normal renal function, a slightly increase in hepatic enzymes (GOT and GPT 76 and 75 resp.). Tacrolimus and MMF through levels were 31 ng/mL and 1,6 ug/mL, respectively. C-reactive protein was 6.3 mg/dL. Chest X-ray revealed a small left pulmonary basal consolidation, which was suspected of pneumonia. CT-scan of the thorax showed scars and bronchiectasis in the left pulmonary base but did not confirm pneumonia.
On admission, suspension of tacrolimus, correction of hyponatremia with saline solution, and antimicrobial treatment with levofloxacin 500 mg iv/day, ceftriaxon 2 g iv/day, and amphotericin B 250 mg iv/day were started. Blood and sputum cultures were negative for bacteria and viruses. Serology for Toxoplasma as well as Legionella pneumophila persisted negative.
After an initial mild improvement of her symptoms, on the third day of admission the patient complained of shortness of breath at rest and a decreased level of conscience. Physical examination revealed increased Respiratory Rate, blood pressure of 100/60 mmHg, heart rate of 120, reduced air entry on both lungs, and peripheral hypoperfusion. Arterial blood gas revealed a pO2 of 57 mmHg, pCO2 of 24 mmHg, Sodium of 125 mEq/L, and a lactate concentration of 2.6 mmol/L. Vancomycin, trimethoprim/sulfamethoxazole, and acyclovir intravenously were added to the treatment under the suspicion of atypical pneumonia. Repeated serology for Toxoplasma and Legionella still resulted negative.
The patient's clinical condition rapidly worsened with respiratory failure that required mechanical ventilation, liver and renal failure that required replacement therapy. The patient finally died of multiorgan failure the same day. Postmortem examination revealed a disseminated toxoplasmosis with involvement of the heart, lungs, and liver. Microscopic analysis of pulmonary and cardiac sections () stained with hematoxylin/eosin revealed interstitial and intra-alveolar infiltrates of lymphocytes and monocytes/macrophages containing Toxoplasma trophozoites. Culture of tissue sample from lungs was negative for bacterial and CMV infection.


An 82-year-old woman presented with a two-month history of fatigue, anorexia, and pruritic skin lesions nonresponding to oral antihistamines. On admission, physical examination was unremarkable except for multiple polycyclic-annular, erythematous papules, and plaques mainly affecting her extremities, some with signs of central clearing and scales (Figures –). Many lesions initially simulated wheals, yet they grew slowly over 1-2 weeks to fade subsequently leaving behind grayish-colored maculas (). Her medical history included coronary heart disease and hyperuricaemia on furosemide, metoprolol, isosorbide mononitrate, captopril, aspirin, and allopurinol at the time of admission. No new medications were instituted over the last year, even on per needed basis.
Laboratory tests revealed elevated erythrocyte sedimentation rate and increased C-reactive protein. Rest of laboratory evaluation was within the normal range or negative ().
A lesional skin biopsy showed a moderately dense perivascular and interstitial inflammatory cell infiltrate in the upper dermis (), consisting of lymphocytes, monocytes, and plentiful eosinophils. Endothelial swelling, intraluminal fibrin, eosinophils within vessel walls and eosinophilic dust around them were featured, consistent with the diagnosis “eosinophilic vasculitis” (). Flame figures were not seen. Direct immunofluorescence studies were negative. Based on the clinical-pathologic correlation analysis and the differential diagnosis considerations (discussed in the next paragraph), a “recurrent cutaneous eosinophilic vasculitis” (RCEV) was diagnosed.
A series of conditions was included in the differential diagnosis of the present case. Because of the presence of annular lesions, some of them also with a “collaret-like” scale, an erythema annulare centrifugum (EAC) was initially considered in the differential diagnosis of the skin lesions of this patient. However, in the present case, the lesions are much infiltrated, oedematous, and quite numerous to be diagnosed as EAC, evolve too quickly for EAC, and also heal with signs of vasculitis. At tissue level, EAC is usually characterized by the presence of a moderately dense mixed cells infiltrate around vessels of the superficial vascular plexus of the dermis. Also eosinophils, sometimes plentiful, may be present. However, in contrast to the present case in EAC the infiltrate is typically well demarcated (“coat sleeve”) and morphologic findings of vasculitis fail. Wells' syndrome and hypereosinophilic syndrome were excluded in the present case because of the histopathological findings of skin inflammation confined to the perivascular area of the upper dermis and lack of blood eosinophilia. Moreover, microscopic signs of massive eosinophil degranulation failed. Also the diagnosis of sarcoidosis, at first considered in the differential diagnosis of this recurrent annular eruption with findings of systemic inflammation, was subsequently disregarded: radiological (chest X-ray) and laboratory findings (normal ACE and calcium levels in serum) as well as the histopathological picture of skin lesions without any granulomatous inflammation sufficiently permit exclusion of sarcoidosis. Finally, fully developed lesions of “classic” urticarial vasculitis present histopathologically as leukocytoclastic cutaneous vasculitis, not seen in the present case on the occasion of two lesional skin biopsies. On the other hand, the presence of (eosinophilic) vasculitis sufficientlydistinguishes the skin lesions of this patient from persistent urticaria [, ]. In conclusion, based on clinical, laboratory and biopsy findings, Wells' syndrome, hypereosinophilic syndrome, erythema annulare centrifugum (EAC), sarcoidosis and urticarial vasculitis were all excluded and the differential diagnosis of the eruption of this patient was narrowed down to “recurrent cutaneous eosinophilic vasculitis” (RCEV).
A subsequent abdominal computed tomography, prompted by persistently elevated ESR, revealed the presence of a soft-tissue mass enveloping the aorta (), from the renal arteries down to the aortic bifurcation, that caused right mild hydronephrosis and dilatation of the ipsilateral ureter, findings consistent with CP (retroperitoneal fibrosis).
Oral methylprednisolone (0.5 mg/kg/d) in combination with colchicine (1 mg/d) was initiated. Over the next month, the patient had gradual resolution and she decided on her own to discontinue the treatment. One month later, the eruption relapsed; a second skin biopsy was taken in order to exclude early mycosis fungoides with RCVE findings as the first. Oral corticosteroids were restarted at the same initial dose and tapered slowly over a three-month period. At six-month followup, the patient was free of skin lesions and symptoms and remained at the 12-month follow-up visit.


A 68-year-old gentleman was admitted to hospital through the emergency department with increasing breathlessness, productive cough, and a four-week history of recurrent left-sided loin pains.
Past medical history was of adenocarcinoma of the sigmoid colon 10 years previously for which he had undergone a curative surgical resection. Intraoperative urethral injury required him to intermittently self-catheterise his bladder and over the subsequent decade he had suffered multiple urological problems including recurrent urinary tract infections, nephrolithiasis, and hydronephrosis, particularly of the left renal collecting system. Five months prior to the index admission a nephrostogram had shown multiple calculi on the left-hand side with a multilayered calcified stone causing compression of the pelvicalyceal system and a separate stone at the level of the vesicoureteric junction, and therefore both an antegrade stent (24 cm JJ stent) and a nephrostomy tube (capped 8 French) were inserted. Despite this intervention he continued to suffer upper urinary tract infections that required treatment with intravenous antibiotics. An isotope renogram (99Tcm MAG-3 with furosemide) performed 2 weeks prior to admission had revealed a drainage curve suggestive of ongoing obstruction and a split renal function of 31% on the left and 69% on the right.
On initial assessment he was tachycardic, tachypnoeic, and febrile with reduced breath sounds over the left hemithorax. He was noted to be expectorating malodorous creamy yellow sputum and purulent urine drained on insertion of a Foley catheter. Inflammatory markers were raised (leucocyte count 25.0 × 109/L, C-reactive protein 250 mg/dL), and serum urea and creatinine were within the local normal reference ranges. A moderate-sized left-sided pleural effusion was seen on chest radiograph. Given his extensive urological history a computed tomographic (CT) scan of the renal tract was organised showing a left-sided perinephric collection and a large pleural effusion that had gas-containing areas consistent with an empyema. A respiratory consult was arranged, and bedside ultrasound examination confirmed a complex loculated pleural effusion (see ).
Further discussion with a consultant radiologist about the radiographic appearance of the empyema determined that there was in fact a fistulous tract extending between the retroperitoneal collection and the pleural space (see Figures and ). Over the following day he exhibited signs of respiratory distress and an urgent chest drain was inserted (24 Fr Thal-Quick, Cook inserted by Seldinger technique under direct ultrasound guidance). Over 1 L of frankly purulent material drained and although his observations initially improved, he continued to require supplementary oxygen therapy and developed progressive respiratory failure. He was therefore transferred to the intensive care unit where he required endotracheal intubation and mechanical ventilation. He was commenced on intravenous piperacillin/tazobactam, and a percutaneous nephrostomy (8 fr locking drain) was performed to drain the left perinephric abscess. Microbiology specimens from both the pleural fluid and urine grew mixed facultative anaerobes (Escherichia coli and Enterococcus faecalis) with identical resistance patterns.
Successful extubation was performed after 24 hours of ventilatory support, and he continued to improve, being stepped down from the high dependency unit with good resolution of the empyema radiologically. Prior to discharge to the urological ward and after discussion with the microbiology team, it was decided to follow on from his intravenous antibiotics with an extended course of targeted oral antimicrobial therapy with metronidazole, amoxicillin, and ciprofloxacin. A left nephrectomy is now planned, after a period of convalescence, to deal definitively with the source of the recurrent infection.


A 42-year-old man presented with a six-month history of nonbloody diarrhea associated with the clinical triad of fever, splenomegaly, and pancytopenia. Anti-Leishmania antibodies serological tests (indirect immunofluorescence title 1 : 640 and rapid test with the recombinant k39) were positive. The diagnosis of visceral leishmaniasis was firmed by the detection of amastigotes in a bone marrow aspirate smears. Because of upper gastrointestinal symptoms (mild dysphagia and vomiting), an esophagogastroduodenoscopy was performed, revealing in duodenum the presence of erosions coated with fibrin, plates of enanthema, and thickened pleats with small whitish spots distributed diffusely. In a tissue fragment obtained by biopsy, the duodenal mucosa showed villous enlarged and filled with numerous histiocytes packed with intracytoplasmic round structures with morphology suggestive of amastigotes of Leishmania spp. The patient received liposomal amphotericin B, 4 mg/kg of body weight during 5 days with complete improvement of visceromegaly, fever and recovery of white blood cell and platelets counts. After treatment and hospital discharge, diarrhea and anemia persisted, and his condition continued to deteriorate. Four months later the patient was admitted once again to hospital with worsening diarrhea, dehydration, metabolic acidosis, and electrolyte imbalance. Blood tests revealed a normochromic normocytic anemia, profound hipoalbuminemia (1.9 g/dL), and a high erythrocyte sedimentation rate (120 mm/1 hour). Laboratory examinations also revealed low serum folic acid and iron levels. Both vitamins were replaced and nutritional support was beginning. This time, an extensive search for parasites in stool microscopy was done and eggs of S. mansoni were identified on stool microscopy. The patient was treated with praziquantel 60 mg/kg of body weight. After four weeks, diarrhea remained unchanged, and his condition continued to deteriorate with anorexia and weight loss. Abdominal computer tomography showed mild splenomegaly, no lymphadenopathy, and a normal liver size. There was mild dilatation of intestinal loops of the colon. A bone marrow aspiration was also performed showing only reactive changes and no Leishmania amastigotes. Real-time quantitative PCR (qPCR) assessment on whole blood and bone marrow samples showed positive results using the ribosomal RNA small subunit gene (SSU rRNA) from Leishmania spp. as DNA target. Patient's lymphocyte counts were TCD4+: 539/mm3, TCD8+: 503/mm3 e TCD3+: 1,094/mm3. A colonoscopy was normal. Despite the absence of symptoms of upper gastrointestinal system, an upper gastrointestinal endoscopy was performed, and it showed the presence of diffuse pallor in stomach and duodenum with swollen appearance without ulceration. Duodenal mucosa histopathology showed the persistence of amastigotes, as four months before (). The immunohistochemical study [] confirmed the presence of numerous immunolabeled amastigotes forms of Leishmania (). Tests to detect anti-Trypanosoma cruzi antibodies, a protozoan also endemic in Brazil and with tropism for the gastrointestinal tract, and the anti-HIV testing were negative. The patient was treated with amphotericin B deoxycholate, 0.8 mg/kg of body weight for 30 days, and diarrhea resolved completely within two weeks. Two months after the end of treatment the patient had reached its normal weight and normalized erythrocyte levels. The plasmatic levels of IFN-γ and IL-10 were measured before and after treatment by sandwich-enzyme-linked immuonosorbent assay, as previously described []. The results were expressed as picograms per milliliter (pg/mL) based on a standard curve. IFN-γ was undetectable before and after treatment, while IL-10 levels decreased from 36 pg/mL at initial evaluation to 16.3 pg/mL (55% reduction) after amphotericin treatment and clinical improvement.


A 78-year-old male presented to the hospital with the history of fever and breathlessness. After initial assessment and investigations, the diagnosis of chest infection was made, and the patient was admitted to short stay unit for further management. The patient was a known case of myasthenia gravis and had history of asbestosis in the past. Whilst being on ward, the patient suddenly collapsed and required resuscitation. The anaesthetist was called to intubate the patient and was then transferred to the Intensive care Unit (ICU) for further management. After initial stabilisation, the patient was investigated to find the cause of sudden collapse. The ECG showed the right bundle branch block. The patient had raised Troponin T level, and the echocardiogram revealed well-preserved left ventricular function with reduced right ventricular function and bright mass in pulmonary artery, thus confirming the diagnosis of massive pulmonary embolism.
The patient was thrombolysed using 100 mg of tissue plasminogen activator (tPA) over two hrs and Heparin 1000 units per mL at 1 mL/hr given in separate lines. The nor-adrenaline was used to maintain mean arterial pressure of around 85. The patient had femoral arterial line after numerous failed attempts to have a right radial artery line. The patient responded very well to the thrombolytic treatment leading to stable haemodynamic condition. Eight hours following the initiation of thrombolytic therapy, the right forearm of the patient was noted to be very swollen and tight. The orthopaedic team was called immediately to assess the forearm in view of compartment syndrome. As patient being intubated, it was difficult to diagnose a compartment syndrome on just clinical ground, and hence a universally accepted, calibrated handheld device (Stryker, Kalamzoo, Michigan) was used to measure the compartment pressure in the involved forearm compartments (). The pressure was measured to be 45 mmHg. The decision of an emergency fasciotomy of the involved forearm was taken following this measurement and high index of suspicion.
The fasciotomy of the forearm was performed by extensile Henry's approach along with decompression of carpel tunnel and abductor compartment of the hand (). The muscles within both superficial and deep compartment were bulging and bloodstained. During this procedure, the massive blood clot was found in the volar compartment of the forearm, close to the radial artery puncture mark and was carefully evacuated. Flexor digitorum profundus was found to be partially necrotic and was therefore debrided till bleeding muscle identified. The wounds of carpel tunnel and adductor compartment release were closed primarily, and volar forearm wound was left open. After 72 hrs, the fasciotomy wound was then evaluated again and closed satisfactorily without any tension on the suture lines (). The patient made remarkable recovery postoperatively and was then commenced on vigorous physiotherapy.
At the end of 3-month followup, the patient had full range of movements in elbow, terminal restriction of movement in wrist and hand. He had functional muscle power in his intrinsic muscles of the hand. Apart from mild tingling in the hand, there was no sensory deficit.


A 4-year-old left-handed boy presented to our outpatient clinic with a small, solitary, bluish, and slightly elevated skin lesion on the tip of his left index finger. The lesion was present since birth and did not enlarge with age. His parents had never sought any medical attention for their son's skin lesion. However, recently the boy was complaining of pain while playing and holding things in his left hand.
Clinical examination revealed a 0.3 cm, slightly elevated, bluish lesion tender on light pressure. There was no history of significant trauma. No axillary lymphadenopathy was noted. The lesion was shaved off with the clinical differential diagnosis of a blue naevus or a myxoid cyst.
Macroscopically, the specimen consisted of a 0.3 cm fragment of skin. Microscopically, the section revealed a small fragment of acral skin. The epidermis was unremarkable. Within the dermal papillae there were regularly distributed Meissner's (tactile) corpuscles. In the papillary/reticular dermis was a poorly defined proliferation of haphazardly arranged, small nerve twigs admixed with somewhat increased number of capillary-like, thin-walled vessels some of which displayed variable size and shape (). Some nerve twigs were in intimate contact with the vessels forming a kind of abortive neurovascular structure (). In places, nerve twigs appeared to be protruding within the lumens of dilated vessels. There was no evidence of old or recent trauma and no fibrosis. No skin adnexae were present.
Immunoperoxidase studies were performed. The nerve twigs were positive for S100 (). Epithelial membrane antigen decorated the perineurium around the nerve twigs. Smooth muscle actin and CD34 highlighted muscular and endothelial components of the vessels.


About 3 years ago, a 53-year-old man was referred to our hospital with the chief complaint of a repetitive painful nodule over the left scrotum. He had visited a local medical clinic and had been treated with empirical antibiotics with the diagnosis of epididymitis. The patient was sexually active and reported no lower urinary tract symptoms. He denied any history of exposure to asbestos or scrotal trauma. First, scrotal ultrasonography was arranged to rule out an abscess. The ultrasonographic pictures (Figure
A) showed a left scrotal mild hydrocele and an increased thickness of the scrotal wall as seen in epididymitis. Following modification of antibiotic treatment, the symptoms subsided for about one year.
One year later the patient returned with the problem of several episodes of scrotal pain and recurrent epididymitis. This time, antibiotic therapy had a limited therapeutic effect, and the inflammatory symptoms fluctuated, but never subsided. Thus, we decided to perform partial epididymectomy plus a preventive vasectomy. Prior to surgery, follow-up scrotal ultrasonography demonstrated a hypoechoic lesion of about 0.5 cm in size over the left epididymal head (Figure
B), and inflammatory granuloma was suspected. During the operation, an ill-defined nodule (measuring 2 × 1 × 1 cm) was found lying on the tunica vaginalis near the epididymal head. The nodule was excised from the tunica vaginalis and a partial epididymectomy plus a preventive vasectomy were performed.
The pathological report showed unusual neoplastic change of the tunica vaginalis. Under microscopic examination, the nodule showed a picture of malignant mesothelioma, well-differentiated, accompanied by tubulopapillary morphological features. These tumor cells were rounded or a cuboidal pattern with nuclear atypia, prominent nucleoli and a diffuse infiltrating pattern (Figure
A). By immunohistochemical staining, the cells were found to be consistently positive for cytokeratin stain and calretenin stain but negative for CEA stain (Figure
B-D), and the diagnosis of malignant mesothelioma of the tunica vaginalis testis was confirmed.
Once the pathologic diagnosis was established, complete workup of the tumor staging was arranged. By computerized tomography, no evidence of local invasion or metastasis in the adjacent lymph nodes was demonstrated. Subsequently, radical orchiectomy with wide excision of the hemi-scrotal wall was performed. The patient was discharged and so far (after over 3 years of follow-up) there is no evidence of local or distant recurrence.


An 88-year-old male patient arrived at our emergency department with a 3-hour history of acute crushing retrosternal chest pain. The chest pain began while he was asleep. He had no history of spontaneous bleeding, smoking, hyperlipidemia, diabetes mellitus, atrial fibrillation, or family history of coronary artery disease. However, he had hypertension and underwent tracheotomy because of laryngocarcinoma 7 years previously.
The physical examination showed that his respiratory rate was 22/min, heart rate was 46/min, and blood pressure was 110/70 mmHg. His neck veins were not distended, and his breath sounds were clear. His heart rhythm was regular and heart sounds were clear with no murmurs. Peripheral pulses were intact with no edema.
His initial electrocardiogram (ECG) showed ST-segment elevation in the inferior (II, III, and aVF) and anterior (V3–V6) leads (Figure ), which was considered to indicate acute inferior and anterior myocardial infarction. However, his second ECG in the cardiac care unit had changed to show ST-segment elevation in the inferior leads but ST-segment depression in the anterior leads (Figure ).
The patient underwent primary angioplasty after receiving aspirin (100 mg) and clopidogrel (300 mg). Coronary angiography revealed that the right coronary artery (RCA) was acutely and totally occluded at the midportion (Figure ) and that the proximal and midportion of the left anterior descending coronary artery (LAD) had a hazy filling defect, which suggested an acute thrombus (Figure ). We inferred that the RCA and LAD had first totally occluded simultaneously, followed by spontaneous partial lysis of the LAD thrombus. Intervention of the RCA was performed first (Figure ), and only because of TIMI III flow in the LAD was glycoprotein IIB-IIIA inhibitor subsequently injected into the LAD. The patient’s symptoms and postoperative ECG results were significantly improved (Figure ). After the operation, the patient received antiplatelet and antithrombotic therapy, aspirin, clopidogrel, low-molecular-weight heparin, and glycoprotein IIB-IIIA inhibitor.
ECG after the procedure showed an ejection fraction of 54% with mild hypokinesia of the inferior walls. Creatine kinase-MB fraction levels peaked 12 hours later at 310 ng/mL (normal, 0–4.3 ng/mL). After the procedure, the patient had an uneventful course with no evidence of pulmonary congestion or recurrence of chest pain. The patient was discharged home 10 days later on aspirin, clopidogrel, a statin, a beta blocker, and an angiotensin converting enzyme inhibitor.


A 79 year old male with history of sick sinus syndrome associated with frequent syncopal events underwent dual lead pacemaker implantation with atrial and ventricular active fixation leads. Six months post implant he experienced acute inferolateral ST-segment elevation myocardial infarction complicated by cardiogenic shock and underwent successful primary percutaneous revascularization. During diagnostic coronary angiography the angiographic catheters were identified to engage the previously implanted pacemaker leads. Inadvertent positioning of the atrial and ventricular leads via the left subclavian artery to the right coronary cusp and left ventricular apex, respectively, was confirmed with detailed fluoroscopy and selective subclavian angiography. Pacemaker interrogation confirmed lack of atrial capture and normal ventricular sensing and capture thresholds. Following revascularization, the patient had an uneventful hospital course and was discharged on appropriate medical therapy including antithrombotic therapy with aspirin and thienopyridine. Warfarin was not instituted due to persistent anemia and requirement of dual antiplatelet therapy.
Given the high risk position of the atrial lead tip in close proximity to the right coronary artery ostium, the patient was electively admitted one month later for transarterial lead removal. Under general anesthesia and in a hybrid operating theater and catheterization laboratory, vascular access was obtained in the right and left femoral arteries and right femoral vein. Prior to systemic anticoagulation, the left subpectoral pocket was opened under sterile conditions, and the subcutaneous tissue was carefully dissected until the leads were visible and freed. Venipuncture was performed using the Seldinger technique, and the left axillary vein was accessed. Two active fixation pacemaker leads were implanted in the right ventricular outflow tract and the right atrial appendage, respectively. Fluoroscopy and aortography were performed demonstrating the dual pacemaker system (). Aortic arch angiography demonstrated a type 1 arch and no significant disease in the origin of the great vessels. Using 5-French diameter, 90 cm length guiding sheaths, the right and left common carotid arterieswere selectively engaged over a guidewire. Carotid angiography revealed nonobstructive disease in the intracranial and extracranial portions bilaterally. Unfractionated heparin was administered to achieve an activated clotting time of approximately 350 seconds. A distal embolic filter wire (Spider, EV3 Inc., Plymouth, MN)was then deployed in the distal segment of the both internal carotid arteries. Selective left subclavian angiography was then performed, confirming the pacemaker leads entry point in the subclavian artery, but no critical subclavian arterial stenosis. Unfractionated heparin was administered to achieve an activated clotting time of approximately 350 seconds.
Stylets were then advanced through the chronic transarterial leads, and after retracting the active fixation mechanism, gentle traction on the ventricular lead enabled withdrawal without requirement of laser extraction. Similarly, the atrial lead was manually withdrawn from the aortic root, and both leads were carefully pulled back until the tips were in close proximity to the insertion site of the subclavian artery. Over an antegradeguidewire, intravascular ultrasound was performed to ensure appropriate sizing for an 8mm x 60 mm self-expanding nitinol covered stent (Viabahn, W.L. Gore, Flagstaff, Arizona) placement. The stent was delivered and positioned between the proximal and distal insertion site margins and deployed immediately as the leads were withdrawn (). Angiography confirmed adequate stent expansion and no contrast extravasation. The distal embolic protection devices were subsequently withdrawn. Following the procedure, the patient remained hemodynamically stable, and recovery was uneventful without neurological or vascular complications.