Hypertrophic pyloric stenosis in twins; genetic or environmental factors.

The etiology of infantile hypertrophic pyloric stenosis (IHPS) remains obscure. Over 120 years after the condition has become a clinical entity the debate whether the cause of IHPS is genetic, environmental or both, has not yet reached a final conclusion. Herein, we present a pair of monozygotic male twins with IHPS together with a review of the literature. We aimed to support genetic pre-disposition in the epidemiology of IHPS, adding a twin data to the literature and to review the associated articles about the pathogenesis and inheritance patterns.

Subcutaneous emphysema and pneumomediastinum due to foreign body aspiration.

Pneumomediastinum is a result of perforation of the tracheobronchial tree. When air enters the interstitial space, air diffuses along the perivascular sheaths toward the mediastinum. If an air leak persists, subcutaneous emphysema occurs owing to the continuous leakage of air. Foreign body aspiration rarely causes pneumomediastinum and subcutaneous emphysema. Removal of the foreign body results in a resorption of air and the resolution of symptoms. Here, we present a rare case of foreign body aspiration in a 2-year-old patient who presented with pneumomediastinum and subcutaneous emphysema.

Preliminary experience with tracheal stent application in children with tracheal stenosis.

Tracheobronchial stenosis in infants and small children is a frustrating lesion for both the pediatric surgeon and the patient and his or her family. Different surgical methods have been described to treat stenoses. Recently, tracheal stents have been introduced to relieve the airway obstruction in these patients. Here, we present 6 patients (2 newborns, 3 infants, and 1 three-year-old) with tracheal or bronchial obstructions treated with age-specific Palmaz balloon-expandable tracheal stents. One patient died due to sepsis. One patient's stent was removed successfully. No other problems occurred in the other 4 patients during 4 to 12 months of follow-up.

Outcome of aggressive fibromatosis treated with radiation therapy.

INTRODUCTION: The purpose of this study is to report the clinical course and outcome in 7 patients with aggressive fibromatosis. MATERIAL AND METHODS: Between the years 2000 and 2003, 7 patients who were treated with combined modalities were evaluated retrospectively. Patients' demographic information, including age and gender, tumour characteristics, surgical resection, and the use of radiotherapy were recorded and evaluated. RESULTS: The mean patient age was 34 years. The median time to follow-up was 15.5 months. Resection was performed with positive surgical margins in three cases. Three patients were evaluated as inoperable and one patient was treated with debulking surgery. All patients received radiation therapy with a median dose of 51 Gy. At follow-up, three patients had no evidence of disease, three patients were alive with disease, and one patient died 15 days after radiotherapy. CONCLUSION: Local control is the primary problem in aggressive fibromatosis. There is no appropriate treatment for aggressive fibromatosis and the type of treatment depends on tumour characteristics and location as well as patient characteristics.

Poorly understood and often miscategorized congenital umbilical cord hernia: an alternative repair method.

PURPOSE: Umbilical cord hernia is poorly understood and often miscategorized as "omphalocele minor". Careless clamping of the cord leads to iatrogenic gut injury in the situation of umbilical cord hernia. This study aimed to determine the characteristics and outcomes of umbilical cord hernias. We also highlight an alternative repair method for umbilical cord hernias. METHODS: We recorded 15 cases of umbilical cord hernias over 10 years. The patients' data were retrospectively reviewed, and preoperative preparation of the newborn, gestational age, birth weight, other associated malformations, surgical technique used, enteral nutrition, and length of hospitalization were recorded. RESULTS: This study included 15 neonates with umbilical cord hernias. The mean gestational age at the time of referral was 38.2 ± 2.1

Spontaneous rupture of extrahepatic choledochal cyst: two pediatric cases and literature review.

Spontaneous rupture of extrahepatic choledochal cysts is very rare. Neonatal cases generally present with biliary ascites, and older children with acute abdomen. Although the cause is unclear, accumulation of protein plugs in an anomalous pancreaticobiliary junction, irritation of the cyst wall due to reflux of pancreatic juice, and weakness due to a developmental error such as common channel syndrome have all been suggested to play contributing roles. The treatment of choice is complete excision of the cyst and hepatico-jejunostomy. In newborns who are in a poor condition, the primary treatment should be simple peritoneal drainage.

A very unusual presentation of Niemann-Pick disease type B in an infant: similar findings to congenital lobar emphysema.

The main features of Niemann-Pick disease type B (NPD-B) are enlargement of the liver and spleen, and mild pulmonary involvement. Recurrent respiratory tract infection and progressive decline in pulmonary function are major contributors to morbidity and mortality in this patient group. Massive pulmonary involvement in early life is extremely rare. The most common finding on chest X-rays of NPD-B patients is reticular or nodular infiltration of the lungs. This article describes a very rare presentation of NPD-B in an infant who had suffered recurrent respiratory tract infections. Massive emphysema and marked infiltrative parenchymal changes (infiltration of the parenchyma) were initially attributed to congenital lobar emphysema and its compressive effects. However, NPD was suspected when a lung biopsy showed foamy cells and sea-blue histiocytes were detected in a bone marrow biopsy. The definitive diagnosis was established with an enzyme study for sphingomyelinase.

Ipsilateral and contralateral testicular biochemical acute changes after unilateral testicular torsion and detorsion.

OBJECTIVES: An experimental study has been conducted to investigate testicular blood flow alterations through acute biochemical changes during unilateral testicular torsion and detorsion. METHODS: One hundred twenty male albino rats were divided into 12 groups, each containing 10 rats. One group served to determine basal values of biochemical parameters, 4 groups were subjected to varying periods of unilateral testicular torsion (3, 6, 12, and 24 hours, respectively), 3 groups were subjected to detorsion following varying periods of torsion (6, 12, and 24 hours, respectively), and 4 groups underwent sham operations as controls. Levels of lactic acid, hypoxanthine, and lipid peroxidation products were determined in testicular tissues. RESULTS: Values of these 3 parameters obtained from sham operation control groups did not differ significantly from basal values (p > 0.05). All 3 parameters were increased significantly in both ipsilateral torted and contralateral nontorted testes after unilateral testicular torsion when compared with basal values (p < 0.05). Detorsion did not cause significant changes in levels of lipid peroxidation products in both ipsilateral torted and contralateral nontorted testes when compared with values obtained after torsion (p > 0.05). CONCLUSIONS: Ipsilateral testicular torsion causes a decrease not only in the ipsilateral torted but also in the contralateral nontorted testicular perfusion. The clues of reperfusion injury do not become evident following detorsion of testicular torsion lasting more than 6 hours.

Three-dimensional conformal breast irradiation in the prone position.

The prone position can be used for the planning of adjuvant radiotherapy after conservative breast surgery in order to deliver less irradiation to lung and cardiac tissue. In the present study, we compared the results of three-dimensional conformal radiotherapy planning for five patients irradiated in the supine and prone position. Tumor stage was T1N0M0 in four patients and T1N1M0 in one. All patients had been previously submitted to conservative breast surgery. Breast size was large in three patients and moderate in the other two. Irradiation in the prone position was performed using an immobilization foam pad with a hole cut into it to accommodate the breast so that it would hang down away from the chest wall. Dose-volume histograms showed that mean irradiation doses reaching the ipsilateral lung were 8.3+/-3.6 Gy with the patient in the supine position and 1.4+/-1.0 Gy with the patient in the prone position (P = 0.043). The values for the contralateral lung were 1.3+/-0.7 and 0.3+/-0.1 Gy (P = 0.043) and the values for cardiac tissue were 4.6+/-1.6 and 3.0+/-1.7 Gy (P = 0.079), respectively. Thus, the dose-volume histograms demonstrated that lung tissue irradiation was significantly lower with the patient in the prone position than in the supine position. Large-breasted women appeared to benefit most from irradiation in the prone position. Prone position breast irradiation appears to be a simple and effective alternative to the conventional supine position for patients with large breasts, since they are subjected to lower pulmonary doses which may cause less pulmonary side effects in the future.

The use of eccentric circummeatal-based flap with combined limited urethral mobilization technique for distal hypospadias repair.

We report a suitable approach to the correction of subcoronal meatus for any variants and report our experience with this method. An eccentric circummeatal-based flap was used to treat 42 patients with hypospadias during a 4-year period. The position of the meatus was coronal in 18 patients (43 percent) and subcoronal and distal penile in 16 (38 percent) and 8 (19 percent) patients, respectively. In addition, 19 patients had marked ventriflexion. The mean length of the urethroplasty was 1.1 cm (ranging from 0.6 to 1.7 cm). In no instance was residual ventral curvature noted nor did the neourethra restrict the phallus during erection. The functional and cosmetic results were excellent, and in many cases the glans penis assumed a nearly normal shape and appearance. No patient required a second procedure to improve cosmesis. This is a convenient procedure for correction of distal hypospadias in which the urethral gap is no longer than 2 to 2.5 cm. The surgical repair does not depend on glanular or meatal variations or even if the patient had a previous operation. It is particularly suitable in cases that have marked fibrous chordee.