New phenotype with generalized platyspondyly, large mandible, hypoplastic teeth, strabismus, hyperopia and low cholesterol levels.

A sporadic, adult male patient with generalized platyspondyly, large mandible, hypoplastic teeth, strabismus, and low serum cholesterol levels is presented. Some of the patient's features resemble brachyolmia, Spondylo-epiphyseal dysplasia tarda, Kenny-Caffey and Stickler syndromes. Based on literature review, possible diagnoses are discussed. In conclusion, this patient can have a variant of brachyolmia or Spondylo-epiphyseal dysplasia tarda. However, we cannot exclude that this constellation of clinical features may represent a new syndrome.

The effect of gained experience on postoperative complications and mortality in cadaveric liver transplantation: a single-center experience.

AIM: Liver transplantation (OLT) has become the treatment of choice for end-stage liver failure, as well as for selected cases of malignancies and metabolic disorders. Decreased postoperative complications and mortality rates are expected to correlate with improvements in the interdisciplinary team approach, the perioperative anesthesiologic and intensive care management, and careful follow-up after transplantation. In this study, we have evaluated the effect of gained experience on postoperative complications and mortality in cadaveric OLT performed in our institution. MATERIALS AND METHODS: Data from cadaveric OLT patients (n = 34) since 2003 were retrospectively evaluated. Patients were divided into 2 groups: Early (2003-2006; n = 15) and late (2007-2010; n = 19). Age, gender, cold and warm ischemia times, intraoperative transfusion rates, infectious complications, biliary and vascular complication rates, and early and late postoperative mortality rates were compared in the 2 groups. RESULTS: The age and gender distribution was similar among both groups. Mean cold and warm ischemia times, intraoperative transfusion rates, and operative times were significantly lower in the late period group (P = .004, .012, and 0.008, respectively; CI=%95). T-tube usage was also significantly lower in the late period group (P < .001). There was no significant change for postoperative intensive care period (P = .404), but the overall length of stay in hospital was shorter for the patients in the late period group (P = .019). The nonsurgical early postoperative complication rate was lower (P = .001) and early postoperative mortality was nearly significant (P = .06) in patients who comprised the late period group. There was no difference in terms of biliary and vascular complication rates and overall survival rates between patients in the early and late groups (P = .664, .264, and .107, respectively). CONCLUSION: Our results indicate that the institutional improvements toward an interdisciplinary team approach in cadaveric OLT correlate with better results in ischemia and operative times and lower intraoperative transfusion rates and hospital stays. Early postoperative complication and mortality rates were found to decline in parallel to the team experience.

Craniospinal involvement in a patient with isolated bone marrow mastocytosis. A case report.

Central nervous system involvement in systemic mastocytosis (SM) is very rare. This case report describes the computed tomography and magnetic resonance (MR) imaging findings of central nervous system involvement in a patient with isolated bone marrow mastocystosis. Bone marrow infiltration in SM caused cranial nerve dysfunction and meningeal irritation secondary to narrowing of cranial apertures and meningeal involvement, respectively. MR imaging is the modality of choice in both detection and follow-up of SM and also useful for differential diagnosis and detection of complications.

Comparison of contrast-enhanced T1-weighted and 3D constructive interference in steady state images for predicting outcome after hearing-preservation surgery for vestibular schwannoma.

We compared contrast-enhanced T1-weighted and 3D constructive interference in steady state (CISS) sequences for demonstrating possible prognostic factors in hearing-preservation surgery for vestibular schwannoma. We studied 22 patients with vestibular schwannomas having hearing-preservation surgery. Postoperatively six (27%) had a facial palsy and eight (36%) had hearing loss. There was a significant correlation between the size of the tumour and facial palsy (r=-0.72). Both techniques adequately demonstrated all tumours. Involvement of the fundus of the internal auditory canal (IAC) and a small distance between the lateral border of the tumour and the fundus were correlated significantly with hearing loss (r=-0.81 and -0.75, respectively). The 3D-CISS sequence, by virtue of its high contrast resolution was superior to T1-weighted images ( P<0.05) for detection of the fundal involvement. The direction of displacement of the facial nerve did not correlate with facial palsy or hearing loss. We think that 3D-CISS images better show the features influencing surgical outcome, but that contrast-enhanced T1-weighted images are required for diagnosis.

Massive lower gastrointestinal hemorrhage from the surgical anastomosis in patients with multiorgan trauma: treatment by subselective embolization with polyvinyl alcohol particles.

PURPOSE: To evaluate the efficacy and safety of subselective arterial embolization with polyvinyl alcohol (PVA) particles with or without microcoil augmentation to control postoperative lower gastrointestinal (GI) bleeding. METHODS: Ten patients with clinical, scintigraphic, and angiographic evidence of postoperative lower GI bleeding were considered for subselective embolization. Subselective embolizations were performed through coaxial microcatheters with 355-500 micron PVA particles with or without additional coil embolization. RESULTS: Embolization was technically successful in 9 of 10 (90%) patients. In one patient, subselective embolization was not possible; consequently no embolization was performed. Clinical success was achieved after a single embolization in 6 of 10 (60%) patients and after a second embolization in an additional 3 of the 10 (30%) patients. While there was no rebleeding in patients with normal coagulation parameters, all three patients (100%) with coagulopathy rebled, two of them from another source. Although no acute ischemic effects developed, no long-term sequela such as ischemic stricture were specifically looked for. Seven patients developed abdominal discomfort and/or fever within 24-48 hr. Four of 10 patients died of complications other than hemorrhage or ischemia. CONCLUSION: Subselective PVA embolization with or without a microcoil embolization is an effective and safe means of managing postoperative lower GI hemorrhage in patients with multiorgan trauma.

Percutaneous treatment of hepatic hydatid cyst in pregnancy.

A 20 cm hepatic hydatid cyst with daughter cysts, was diagnosed in a primigravida in the fifteenth week of pregnancy and was managed percutaneously. No complications occurred and the patient subsequently gave birth to a healthy baby.

Percutaneous treatment of hydatid cysts of the liver: long-term results.

OBJECTIVE: The purpose of the study was to present the long-term results of percutaneous treatment of liver hydatid cysts. SUBJECTS AND METHODS: Seventy-two patients (44 male and 28 female, ranging in age between 10 and 69 years; mean age, 35 years) with 106 liver hydatid cysts underwent percutaneous treatment with albendazole prophylaxis. Puncture, aspiration, injection, and reaspiration (PAIR) were used for hydatid cysts smaller than 6 cm (n = 66). Larger cysts (n = 40) were treated by catheterization. Hypertonic saline solution and absolute alcohol were used as the cytotoxic and sclerosing agents. Sonographic guidance with or without fluoroscopy was used in all patients. Follow-up was mainly by sonography every third month of the first year, every sixth month of the second year, and once a year thereafter. The mean follow-up time was 37 months. RESULTS: The mean reduction in volume at the time of the first follow-up was 87.0% and 73.5% in catheterization and PAIR patients, respectively. The immediate sonographic changes after treatment were detachment of the endocyst and disappearance of the regular endocyst, with a reduction in the fluid component. The solid appearance of the cyst remnant indicated complete cure as the cyst wall became irregular and thicker. The average time for development of a solid appearance was 19 months in PAIR patients and 26 months in catheterization patients. Seventy of 72 patients were cured, whereas two recurrences (2.8%) were observed. No mortality, abdominal dissemination, or tract seeding occurred. Minor complications were urticaria and fever in eight patients (11.1%). Major complications were infection of the cyst cavity in two patients (2.8%) and development of biliary fistula in four patients (5.6%). Mean hospitalization times were 17 days for complicated cases and I day for uncomplicated cases. CONCLUSION: The long-term results of percutaneous liver hydatid cyst treatment accord with short-term results, indicating that the procedure is efficient and safe and offers complete cure in selected patients with a short hospitalization.

Percutaneous renal hydatid cyst treatment: long-term results.

BACKGROUND: To evaluate the effectiveness of percutaneous treatment of renal hydatid cysts. METHODS: Four male and one female (14-52 years old, mean = 37 years) patients with five renal cysts were treated percutaneously. All five cysts from the patients were pure fluid collections, which were consistent with type I hydatid cysts according to Gharbi's classification. After entering the cystic cavity under sonographic guidance, cystic fluid was aspirated, and the cavity was filled with hypertonic saline (15% NaCl). In three patients with cysts larger than 6 cm in diameter, catheterization was performed under fluoroscopic guidance, and the cavity was filled with 95% absolute alcohol to sclerotize the cyst walls. In two patients with cysts smaller than 6 cm in diameter, the procedure was carried out by a technique in which the cyst was puncture aspirated, hypertonic saline solution was injected, and the cyst was reaspirated. The patients were followed by ultrasonography and computed tomography. Follow up was 5-62 months (mean = 33.8 months). RESULTS: Sonographic follow-up examinations indicated a gradual decrease in cyst size and volume. The size reduction was significant (p < 0.05). The volume reduction rate was 55-95% (mean = 81%). During follow up, fluid components of all five cysts reduced gradually and finally disappeared, leaving a remnant that is called a "pseudotumor appearance." Neither mortality nor any dissemination was encountered during follow up. The only complication seen in this series was an abscess that was successfully treated with percutaneous drainage. CONCLUSION: According to the results of our study, percutaneous treatment of renal hydatid cysts avoids the morbidity of open surgery and preserves the residual function of the kidney.

Deep vein thrombosis in spinal cord injured patients.

In this study to determine the incidence of deep vein thrombosis (DVT) in spinal cord injury (SCI) patients, we evaluated 30 instances of bilateral ascending venography obtained in 31 patients. Every patient was on prophylactic low dose heparin anticoagulant therapy. The incidence of DVT was found to be 53.3%. Adverse effects due to venography were seen in 10% of patients. No major complications including postvenographic phlebitis and allergic reactions were observed.