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.

The effect of C.P.A.P. upon pulmonary reserve and cardiac output under increased abdominal pressure.

The cardiopulmonary effects of CPAP under conditions of increased intra-abdominal pressure were studied using the rabbit as an experimental model. Central venous pressure and cardiac output were reduced when the intra-abdominal pressure was increased. Addition of CPAP caused a further reduction in C.V.P. and C.O. Despite these reductions CPAP significantly improved arterial PaO2 without significant effects on pH and PaCO2. The use of CPAP in conditions associated with pulmonary dysfunction secondary to increased intraabdominal pressure may be a definite therapeutic modality.

Epigastric cleft: a previously unreported entity.

A newborn who is believed to have an anterior midline abdominal wall fusion defect located in the epigastric region is presented, with a proposal of new nomenclature as "epigastric cleft."

Gastric diverticulum: an uncommon cause of abdominal pain in a 12 year old.

A 12-year-old girl presented with long-lasting intractable abdominal pain associated with a gastric diverticulum. The clinicopathologic features of this rare entity are discussed with emphasis on pathogenesis, diagnosis, and treatment. The authors emphasize that congenital diverticulum of the stomach should be considered in the differential diagnosis of abdominal pain in childhood. Surgical treatment consisting of simple excision of the diverticulum is warranted in cases presenting with long-lasting symptoms after failure of medical treatment.

Congenital urethrocutaneous fistula: a case report and literature review, with a nomenclature proposal.

The authors report the case of a 4-year-old boy who had a congenital urethrocutaneous fistula (CUF) on the penile urethra. The previous reports of patients with fistula from the urethra to the skin are evaluated, and the spectrum of CUF is discussed.

Vaginal atresia and Bardet-Biedl syndrome association: a component or a distinct entity?

Bardet-Biedl syndrome is an autosomal recessive disorder. It is characterized by cardinal anomalies including retinal dystrophy, digital malformations, mental retardation, obesity, and hypogonadism. Recently, renal anomalies also are mentioned among the cardinal signs. Although association of genital anomalies among affected boys are well known, the association of vaginal atresia and other structural genital anomalies are not mentioned among the less-common manifestations of Bardet-Biedl syndrome in girls. Two girls with Bardet-Biedl syndrome presented with hematometrocolpos in the preadolescent period and vaginal atresia was diagnosed. After surgical treatment and extended hospitalization, uncontrolled sepsis resulted in progressive renal failure and death of both patients. Vaginal atresia is often delayed or missed in the early childhood period. In girls with Bardet-Biedl syndrome, vaginal atresia or other structural genital anomalies should be evaluated more systematically during the initial diagnosis of the syndrome. In infancy, the evaluation of a child with vaginal atresia also should include the differential diagnosis of Bardet-Biedl syndrome. Vaginal atresia may either form a component of the syndrome, or girls who present with vaginal atresia in addition to other components of Bardet-Biedl syndrome might form a distinct entity.

Complications of typhoid fever requiring laparotomy during childhood.

All the complications requiring laparotomy among 680 children who were diagnosed as having typhoid fever between 1981 and 1990, at Hacettepe University Children's Hospital, were retrospectively analyzed. Four patients developed intestinal perforation, two gastrointestinal bleeding, and two splenic abscess, with frequencies of 0.58%, 0.29%, and 0.29%, respectively. The overall frequency was 1.17%. While the risk was 1.58% among males, it was 0.42% among females with no statistically significant difference (P > .05). The risk was higher among patients above 6 years of age (P < .05). While perforation and hemorrhagic complications developed within 10 days of initial symptoms and splenic abscess required 10 and 30 days for development, there were no clinical signs and laboratory findings predicting the occurrence of complications in the course of typhoid fever. Two patients, one with intestinal perforation and the other with gastrointestinal hemorrhage, died within 2 days following the septic postoperative courses. On the other hand, 12 patients, among those without complications requiring laparotomy, died of unremitting septic course of the disease. The mortality rate rose from 1.78% to 25% when complications occurred. The findings in this series demonstrate that typhoid fever presents a challenge to the pediatric surgeons not only because of the complications requiring laparotomy with high mortality rates, but also the absence of criteria predicting the occurrence of complications in the course of typhoid fever.

The long-term results of diaphragmatic plication.

Twelve patients who had undergone diaphragmatic plication with the diagnosis of congenital diaphragmatic eventration between 1975 to 1989 were evaluated for the long-term results of plication. Assessment of the long-term functions of the plicated diaphragms 1.5 to 11 years postoperatively was achieved by fluoroscopic, ultrasonographic, and spirometric studies. The absence of paradoxical motion with normal localization of the diaphragms in all patients, and satisfactory motions of diaphragms in 9 patients were documented by fluoroscopy. Measurements of diaphragmatic thicknesses showed that plicated diaphragms of all patients maintained their growths in proportion to the contralateral sides. Additionally, normal values of pulmonary function tests in five of six patients of suitable age for spirometry were obtained. All the clinical studies demonstrated that diaphragmatic plication did not interfere with further development of diaphragms, and late functional results of the plication were acceptable. This supports the choice of surgery in the treatment of diaphragmatic eventration.

In utero defecation of the nondistressed fetus: a roentgen study in the goat.

An experimental study was performed to investigate gastrointestinal motility and meconium passage, with simultaneous blood gas measurements, in the fetuses of eight pregnant goats at 110 to 114 days' gestation (full term, 147 to 155 days). With the goats under halothane anesthesia, a nasogastric tube and a heparinized central venous catheter were inserted into the fetuses. 24 hours after surgery, 10 mL of gastric juice from the fetus was replaced with a nonhydrosoluble contrast medium, and serial roentgenograms and blood samples (for pH, PO2, and PCO2 measurement) were taken every 4 hours. All fetuses began to pass the contrast medium into the amniotic cavity within 16 to 22 hours, and central venous blood gas values were normal. The results of this study suggest that the fetus, which urinates routinely, also defecates routinely into the amniotic cavity, even in the absence of distress. The increased incidence of meconium staining in fetal distress conditions may be indicative of impaired clearance of amniotic fluid.

Adhesive small bowel obstruction in children: the place and predictors of success for conservative treatment.

The records of 230 adhesive small bowel obstruction (ASBO) episodes in 181 patients have been reviewed to observe the place of conservative treatment and to establish criteria to predict the success of conservative trial. Immediate operation has been reserved for 81 episodes that have presented with fever and leucocytosis and/or localized abdominal tenderness, or complete obstruction. The remaining 149 episodes have initially undergone conservative trial. Although 110 episodes (73.8%) have been cured with conservative trial, 39 (26.2%) subsequently necessitated surgical intervention. No adverse occurrences have been observed during or after delayed operations. There was no strangulated bowel nor mortality both in delayed operation and conservatively treated groups. Recurrence has occurred with rate of 18.75% and 36.47% after surgery and conservative treatment, respectively, being significantly different (P less than .01), but the treatment method of the previous episode has been without influence on the method used in the recurrent obstruction (P less than .05). Among the assumed predictive criteria, age at recent laparotomy (P less than .02), time elapsed between recent laparotomy and obstructive episode (P less than .01), the primary condition necessitating laparotomy (P less than .01), the incision of previous laparotomy (P less than .05), and duration of conservative trial (P less than .01) correlated significantly with the success of conservative trial. The number of previous laparotomies and obstructive episodes (P less than .05) have not showed correlation. By the conservative approach used in selected patients with ASBO, 40% overall have been spared operation, without any adverse occurrences. Using the proposed criteria, the success rate of conservative treatment can be predicted.

Transpubic urethroplasty in children: report of 10 cases with review of the literature.

Posttraumatic prostatomembranous urethral strictures (PUS) in children have special features that warrant consideration with respect to management. Transpubic urethroplasty (TPUP) was used to treat 10 children who presented with posttraumatic PUS. All patients previously had unsuccessful repair attempts with other techniques. Urethral stricture was noted in three patients who were cured within a few months by urethral dilatations. Appropriate antibiotic therapy was begun for prolonged urinary infection in four patients. Complete urinary continence was achieved in seven patients (70%), and three (30%) are still incontinent. In the latter, the bladder neck and posterior urethra were reported to have been damaged seriously, owing to extensive dissections at the time of initial treatment. Severe retropubic tissue loss and fibrosis were noted in these patients. Thus, the authors emphasize that avoiding further injury to any of the continence mechanisms that have survived the injury is as important as performing a patent urethral anastomosis. There were no significant complications or morbidity associated with TPUP. TPUP provided excellent exposure and visualization of the involved anatomy and facilitated accurate suture placement and tissue realignment in all patients.