Intracranial ependymomas in childhood--a retrospective review of sixty-two children

Of the 818 tumours of the central nervous system diagnosed between 1972 and 1991, 62 patients (35 males and 27 females) with histopathologically confirmed ependymomas were treated and followed-up at the Children's Hospital of Hacettepe University during that period. The median age was 6 years (range 1-17 years). Headache, nausea and vomiting were the most frequent symptoms; papilledema was the most common sign in our patients. Tumour sites were in the posterior fossa in 47 patients and supratentorial in 15 patients. All patients underwent surgery. Gross- total resection was performed in 27 patients, subtotal resection in 32 patients and biopsy in the remaining 3 patients. Initially, 53 patients were given postoperative radiotherapy. Four patients did not receive radiotherapy because of their young age, whereas five patients died prior to starting radiotherapy. Two slightly different types of chemotherapy protocols were applied for an average of one year in 47 patients. Event-free and overall survival rates at 10 years were 36% and 50%, respectively. Twenty children suffered relapse 4 to 55 months after diagnosis (median 16 months). Relapses were distant in 3 cases and local in 17. Age was the only statistically significant prognostic factor, patients younger than 5 years of age having a poorer outcome. Sex, histopathologic type, localization of the tumour, extent of surgery, and chemotherapy did not influence the prognosis in our study. Because the majority of recurrences were local, better local tumour control is required. New treatment strategies should be developed in order to improve local control.     

Caroli's disease in children: Is it commonly misdiagnosed?

Aim: Caroli's disease is a simple form of intrahepatic bile duct ectasia. It can be complicated with the involvement of liver parenchyma and portal hypertension. Herein, the difficult management of delayed presentation of Caroli's disease is reported. Methods and results: We report on four different forms of clinical presentation of Caroli's disease: an infant with fulminant liver failure, a teenager with persistent biliary fistula, a boy with hypersplenism in the face of portal hypertension and a girl with variceal bleeding.

Conclusions: Caroli's disease must be included in the differential diagnosis of cystic lesions in the liver. Delayed diagnosis of Caroli's disease is difficult to manage and appropriate investigations are warranted before planning a surgical approach.     

Coexistence of Hodgkin lymphoma and cyst hydatic disease of the liver

Although both Hodgkin lymphoma and cyst hydatic disease in children have been seen with an increased frequency, there is no previously reported case of Hodgkin lymphoma associated with cyst hydatic disease from Turkey. The authors report such a case of Hodgkin lymphoma. Intrahepatic cystic masses were diagnosed during ultrasound examination for clinical staging on admission. The diagnosis of cyst hydatic of the liver was confirmed by surgery. Although there was no residual and/or new cyst formation on radiologic follow-up, elevated antibody titers (indirect hemagglutination test) persisted following surgical excision at least for 2 years of follow-up.     

Intraoperative and intermediate-term angiographic results of coronary artery bypass surgery with Symmetry proximal anastomotic device

OBJECTIVE: The objective of this study was to investigate the patency in saphenous vein coronary bypass grafts in which the proximal anastomoses were performed with automatic connector devices or with a traditional suture technique. METHODS: Forty-six patients underwent coronary artery bypass grafting without cardiopulmonary bypass by using one thoracic graft and one or more saphenous vein grafts. Grafts were attached to the aorta with a Symmetry connector (St Jude Medical, Inc, St Paul, Minn) in 23 patients, and partial occlusion of the aorta and sutured anastomoses were used in 23 other patients. Grafts were studied intraoperatively with transit time flowmetry and angiography and revised if necessary. Angiography was repeated after 3 to 5 months. RESULTS: Intraoperative graft patency did not differ between the 2 groups. Follow-up angiography demonstrated excellent thoracic graft patency. Vein graft patency decreased to 50% in the Symmetry group, whereas it was 90% in the suture group ( P = .01). Twenty-five percent of the Symmetry grafts had significant stenosis in the connector. CONCLUSION: Saphenous vein grafts anastomosed to aorta with the Symmetry proximal connector have low intermediate patency compared with those with traditionally sutured anastomoses. We do not recommend the routine use of this device in coronary artery bypass operations.     

Immunogenicity of Borrelia burgdorferi arthritis-related protein

Immunization against arthritis-related protein (Arp) elicits antibody in mice that resolves arthritis but is not protective against challenge with Borrelia burgdorferi. In mice immunized against Arp, an unrelated 37-kDa protein (P37-42), outer surface protein A (OspA), or glutathione S-transferase (GT) and then challenged by syringe or tick, only OspA conferred protection. Passive transfer of Arp antiserum into infected SCID mice induced arthritis resolution, but antisera to P37-42, OspA, GT, or six overlapping Arp peptide fragments did not. Results suggest that the arthritis-resolving immunogenicity is specific to Arp, but the relevant epitopes may be conformational.     

Cloning and expression of a Helicobacter bilis immunoreactive protein

In an effort to identify immunoreactive Helicobacter bilis antigens with potential for serodiagnosis, sera from mice experimentally infected with H. bilis were used to screen an H. bilis genomic DNA expression library. Among 17 immunoreactive clones, several contained sequences that encoded a predicted 167-kDa protein (P167). Five overlapping P167 peptides (P167A to P167E) of approximately 40 kDa each were generated and tested. Immune sera reacted with fragments P167C and P167D at dilutions of 1:1,600 and 1:6,400, respectively, and reacted with an H. bilis membrane extract at a dilution of 1:800 in an enzyme-linked immunosorbent assay. Sera from mice experimentally infected with H. hepaticus did not react with P167C and P167D. Sera from mice naturally infected with H. bilis but not sera from mice naturally infected with H. hepaticus reacted with P167C and P167D. Hyperimmune sera against P167C peptide reacted with recombinant P167C and with a 120-kDa band in H. bilis lysates but did not react with a protein of the same size on immunoblots prepared from H. hepaticus, H. muridarum, or unrelated Borrelia burgdorferi and Campylobacter jejuni whole-cell lysates. Nevertheless, the P167A, P167B, P167C, and P167D primers, but not the P167E primers, amplified DNA from H. hepaticus, and all five primer sets amplified DNA from H. muridarum. These results suggest that P167 is an immunodominant, H. bilis-specific antigen that may have potential for use in serodiagnosis.     

Is it possible to create a slit-like meatus without incising the urethral plate?

A series of hypospadias repairs using the Mathieu and V-incision-sutured (MAVIS) technique, aiming to achieve a normal-looking,vertical, slit-like meatus in 32 patients was reviewed. A Mathieu procedure was performed in 26 patients as a primary repair and in 3 as a rescue operation. In another 3 patients who had severe proximal hypospadias with failed repair, techniques other than Mathieu's were used. Good cosmetic and functional results were achieved in all patients.There were no complications such as fistula or stricture formation. The MAVIS meatoplasty creates a natural-looking, slit-like glanular meatus in both distal and proximal hypospadias, even in complicated cases.     

Mathieu urethroplasty as a salvage procedure: 20-year experience

PURPOSE: We assessed the results of the Mathieu procedure in a series of patients who had undergone multiple prior hypospadias repairs. MATERIALS AND METHODS: Medical records of 55 boys who underwent repeat surgery with the Mathieu procedure were retrospectively reviewed. Prior surgeries, level of hypospadias defect, number of prior surgeries and complications were recorded. Statistical analyses were performed on factors that would impact the outcome. RESULTS: Mean patient age at the time of the salvage Mathieu procedure was 10.33 years (range 2 to 30). In 14 of the 55 patients (25.4%) fistula occurred following the salvage repair. Complications were split glans in 1 case, skin dehiscence in 1 and complete breakdown of the repair in 1. Fistula formation did not differ in groups categorized according to the level of the urethral meatus. Fistula occurred in 6 of 37 (16.2%) and 8 of 18 (44.4%) boys who underwent 1 and more than 1 prior surgery, respectively (p = 0.04). CONCLUSIONS: Although the Mathieu operation had a favorable outcome (74.6% cure) as a salvage procedure in 1 stage, fistula occurred more frequently as the number of prior surgeries increased. Therefore, if primary surgery fails, repeat operation should be performed by an experienced "hypospadiologist."     

CT fluoroscopy-guided percutaneous needle biopsies in thoracic mass lesions

OBJECTIVE: We aimed to evaluate the usefulness of computed tomographic (CT) fluoroscopy guidance for transthoracic needle biopsies. METHODS AND MATERIAL: CT fluoroscopy-guided biopsies were performed in 81 patients with thoracic mass lesions. Interrupted CT fluoroscopy technique was used with 50-130 mA at 120 kV exposure parameters and slice thickness of 10 mm. We used aspirating needle in 41 patients, cutting needle in 28 patients, and both in 12 patients. We obtained adequate biopsy material in 69 patients at first attempt. Mean fluoroscopy time was 15.17 s and maximum procedure time was 18 min. RESULTS: Adequate samples for pathological diagnosis were obtained in all lesions. Pathological diagnoses were malignant in 41 patients, benign in 27 patients, and suspiciously malignant in 13 patients. There was no significant difference between diagnostic accuracy of the needles in malignant and benign lesions. Complications were observed in 11 patients (13.5%). DISCUSSION AND CONCLUSION: CT fluoroscopy-guided technique provides effective real-time needle biopsy in patients with small tumor size and with tumor located near blood vessels, and in non-compliant patients for diagnosing thoracic lesions.