An infantile splenic hemangiopericytoma case treated with partial splenectomy.

Splenic hemangiopericytoma is a very rare tumor. So far only 10 patients (9 adults, 1 child) have been reported in the literature and all of them were treated with total splenectomy. Herein, we report the first infant case of the splenic hemangiopericytoma in a 10-month-old girl and the first case that was treated with partial splenectomy for splenic hemangiopericytoma.

Associated malformations in Morgagni hernia.

Morgagni hernia (MH) is a congenital herniation of the abdominal organs through the subcostosternal portion of the diaphragm. It is a rare type of diaphragmatic hernia that has been associated with other congenital anomalies. The purpose of this clinical review was to determine the incidence of associated anomalies in patients with MH at a major pediatric referral center. The medical records of all patients with the diagnosis of MH were reviewed retrospectively between 1983 and 2006. The age, sex, side of herniation, presenting symptoms and signs, associated anomalies, radiologic features, intraoperative findings, postoperative course, complications, and mortality were recorded. There were 11 males and 5 females. The average age of the patients was 34.5 months (range 2 months-14 years). Twelve patients (75%) had at least one associated congenital malformation and eight (66.6%) of them had multiple anomalies. Six (37.5%) patients had a chromosomal anomaly, Down's syndrome in five and Turner syndrome in one. MH has a high incidence of associated congenital malformations.

Late presentation of bochdalek-type congenital diaphragmatic hernia in children: a 23-year experience at a single center.

PURPOSE: The purpose of this article is to report on our 23-year experience of treating children with late-presenting congenital diaphragmatic hernia (CDH), focusing on diagnostic difficulties, associated anomalies, and morbidity. METHOD: We reviewed 19 children in whom Bochdalek-type CDH was diagnosed after the neonatal period, between 1983 and 2005. RESULTS: There were 14 boys and 5 girls, with a mean age of 18.3 months (range, 5 weeks-14 years). Ten (52.6%) of the patients presented with respiratory symptoms and five (26.3%) with gastrointestinal symptoms. The diagnosis of CDH was based on the findings of chest X-rays, gastrointestinal tract contrast radiographs, and computerized tomography findings. The CDH was on the left side in 16 patients and on the right side in three patients. A hernia sac was present in seven (36.8%) patients. The only postoperative complications were intestinal obstruction caused by adhesions, incisional hernia, and eventration of the diaphragm in one patient each. None of the patients died within this study period. CONCLUSION: The possibility of a delayed presentation of CDH should be considered in the differential diagnosis of recurrent non-specific respiratory or gastrointestinal tract symptoms in a child. A plain roentgenogram with a swallowed nasogastric tube might assist in the diagnosis. Early surgical correction of the diaphragmatic defect is crucial for preventing life-threatening complications.

PERSISTENT Müllerian duct syndrome associated with transverse testicular ectopia: report of two cases.

Persistent Mullerian duct syndrome (PMDS) with transverse testicular ectopia (TTS) is a very rare pathological association, often discovered during repair for inguinal hernia or cryptorchidism. We present 2 cases with PMDS associated with TTS and discuss the diagnosis and management. A hysterectomy was performed, with resection of the underdeveloped fallopian tubes. Both gonads wereplaced into subdartos pouches in each scrotum by the transseptal approach. The diagnosis of PMDS was confirmed postoperatively by genetic and hormonal investigations. Chromosome analysis revealed 46,XY. Pathological findings demonstrated the immature testes and the immature uterus.

Effects of gabexate mesilate on ischemia-reperfusion-induced testicular injury in rats.

The aim of this study was to determine the effects of a synthetic serine protease inhibitor, gabexate mesilate (GM), in rats with ischemia-reperfusion (I-R) damage due to unilateral testicular torsion. Thirty male Sprague-Dawley rats were separated into three groups, each containing ten rats. A sham operation was performed in group 1 (control). In group 2 (I-R/untreated), 1 h detorsion of the testis was performed after 6 h of unilateral testicular torsion. In group 3 (I-R/GM), after performing the same surgical procedures as in group II, gabexate mesilate was given intravenously. In all experimental rats, ipsilateral orchiectomies were performed for histological examination and measuring the tissue levels of malondialdehyde (MDA), superoxide dismutase (SOD), catalase (CAT) and glutathione peroxidase (GSH-Px). MDA values and the testicular injury score decreased and SOD, CAT and GSH-Px values increased in the GM-treated group compared to the I-R/untreated group. The Tc-99m pertechnetate uptake ratio and the perfusion index were significantly decreased in the group 2 compared to the group 1 and 3 rats. In group 3, these values were significantly increased compared to group 2. Most of the specimens in the GM-treated group showed grade-I testicular injury. However, the injuries in the I-R/untreated rats varied between grade-III and grade-IV. The results of this study show that GM may play a role in reducing the injury caused by I-R.