The use of pH monitoring and esophageal manometry in the evaluation of results of surgical therapy for gastroesophageal reflux disease.

BACKGROUND/PURPOSE: Surgery is indicated for the treatment of gastroesophageal reflux disease (GERD) when medical treatment fails or complications are encountered in children. However, it has not been fully established how the results after surgery can be evaluated. A prospective study was performed to evaluate the results of surgical therapy for GERD by pH monitoring (PM) and esophageal manometry (EM) in children. METHODS: Patients who were candidates for anti-reflux surgery between 2003 and 2004 were evaluated for symptoms, growth and results of PM and EM both in the pre- and postoperative periods. RESULTS: Thirteen patients were included (mean age = 6.65 +/- 3.28 years, male/female ratio = 10/3). Most frequently occurring symptoms were recurrent respiratory infections (RRI) (n = 11) and vomiting (n = 8). Nissen fundoplication was performed because of unresponsiveness to treatment (n = 10), RRI (n = 9), failure to thrive (n = 7) and esophagitis (n = 2) after medical treatment (2 - 36 months). Symptoms were resolved in 83.9 % of patients and were not changed in 16.1 % following surgery. Weight percentiles had significantly improved (pre: 12.38, post: 25.4, p < 0.05) during a short follow-up period (1 - 4 months). Mean reflux index (pre: 24.73 +/- 21.07 %, post: 0.93 %, min: 0 - max: 3.6, p < 0.05), reflux time (pre: 368 +/- 313 min, post: 17.1 +/- 15.9 min, p < 0.05), number of episodes (pre: 344.7 +/- 343.6, post: 19.53 +/- 11.13, p < 0.05) and number of reflux episodes longer than 5 minutes (pre: 4.3, min: 0 - max: 58, post: 0.61, min: 0 - max: 3, p < 0.05) were found to be reduced after surgery by PM. Lower esophageal sphincter pressure (pre: 55 +/- 27 cmH (2)O, post: 64.46 +/- 30.85 cmH (2)O), contraction amplitude (pre: 141.92 +/- 69.11 cmH (2)O, post: 130.69 +/- 45 cmH (2)O) and contraction velocity (pre: 1.94 cm/s, min: 0.1 - max: 7.5, post: 4.29 cm/s, min: 0.2 - max: 10) did not differ postoperatively (p > 0.05). However, contraction times were decreased postoperatively (pre: 73.6 +/- 52.9 s, post: 27.67 +/- 20.1 s, p < 0.05) and were found to be correlated with reflux time and the number of reflux episodes longer than 5 minutes. CONCLUSION: Nissen fundoplication is effective for the treatment of GERD. It supports the anti-reflux mechanism without affecting esophageal motility except for contraction times. The decrease in contraction time after surgery can be explained by the decreases in reflux time and in the number of reflux episodes longer than 5 minutes. PM and EM confirmed the clinical improvement and can be used for the evaluation of results of NF.

Bannayan-riley-ruvalcaba syndrome from the point of view of the pediatric surgeon.

Bannayan-Riley-Ruvalcaba syndrome (BRRS) is an overgrowth syndrome and polyposis condition, which carries an increased risk for development of benign and malignant tumors of various tissues and organs. A 9-year-old boy with BRRS who had multiple subcutaneous mesenchymal tumors and follicular adenoma of the thyroid and a 14-year-old boy with multiple subcutaneous mesenchymal tumors and gastrointestinal polyposis are presented, with special emphasis on the surgical management of these patients.

Sigmoid colon vaginoplasty in children.

BACKGROUND: Vaginal construction is necessary for the patients with aplasia of Mullerian ducts, testicular feminisation and androgen insensitivity syndromes. Many methods of vaginal construction have been described. We report here the outcomes of six adolescent patients who underwent sigmoid colon vaginoplasty with special emphasis on the surgical technique and outcomes. PATIENTS AND METHODS: Between 1990 and 2003, six patients underwent sigmoid vaginoplasty after a diagnosis of 5alpha-reductase deficiency (n = 3), testicular feminisation (n = 2) or vaginal atresia (n = 1). The mean age was 16 years (13 to 18). Wide spectrum antibiotics and whole-gut preparation were used in all cases. A 15-20 cm segment of sigmoid colon was pulled through the retrovesical tunnel. The proximal end was closed in two layers in patients with 5alpha-reductase deficiency and with testicular feminisation. A distal anastomosis was carried out to the opening made on the vaginal plate (5alpha-reductase deficiency) or on the tip of the shallow rudimentary vagina (testicular feminisation). The sigmoid segment was interposed between the blind end of the atretic vagina and the perineum in the patient with vaginal atresia. Patients were instructed to perform daily vaginal irrigation. The neovagina was examined and calibrated under anaesthesia. No routine vaginal dilatation was recommended. RESULTS: All but one patient had an uneventful postoperative period and were discharged within 7-8 days. All patients had an excellent cosmetic result with an appropriate vaginal length. One of the patients experienced late stenosis of the introitus which responded to dilatations. Mucus discharge was not a significant problem. The patient with vaginal atresia (Bardet-Biedl syndrome) experienced deep vein thrombosis, renal failure and sepsis, resulting in death. CONCLUSION: Sigmoid colon vaginoplasty is a special procedure which appears appropriate for the construction of a new vagina in children. A sigmoid colon neovagina meets all necessary criteria after a vaginoplasty. It provides an adequate diameter and length, and produces less scar tissue in the perineum. It is self-moistening, easily adaptable to the uterus, cervix and rudimentary atretic vaginal segments and does not require routine dilatation. Mild stenosis of the introitus can be treated by dilatations and revision can be easily performed in severely stenotic cases. On the other hand, the patient may face morbidity after laparotomy and other serious complications may occur due to accompanying diseases.

Bilateral Poland anomaly.

Bilateral absence of the pectoralis major muscle with accompanying abnormalities of shoulder muscles has been reported in patients without Poland anomaly (PA). However, symmetric absence of pectoralis major muscles, hypoplasia of breasts and nipples with symmetric chest wall deformity and bilateral hand anomaly has not previously been reported. A 6-year-old girl with bilateral absence of pectoralis major muscles and hand involvement and symmetric chest wall deformity is, to our knowledge, the first known case of bilateral Poland anomaly.

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.

Distribution and morphometry of fiber types in cremaster muscles of boys with inguinal hernia or undescended testis.

In the present study, we determined and compared the distribution and mean diameters of fiber in the cremaster muscles (CM) of boys with either inguinal hernia (IH) or undescended testis (UT). Samples of CM were obtained from 20 patients (10 boys with IH, and 10 boys with UT) of similar age. The CM muscles of two boys each, without inguinal pathology, were sampled during autopsy. Sections were stained for oxidative and glycolytic enzymes, as well as for ATP-ase reactions after acid (pH: 4.6) and alkaline (pH: 10.6) preincubations. Specimens were also analyzed morphometrically using a KONTRON 400 computerized image analysis system. The Mann- Whitney U test was applied to compare the percentages of fiber types and mean diameters of fibers according to the types of the CM of boys with IH or UT. In boys, the CM is mainly composed of type 1 fibers. The CMs of patients with UT reveal alterations of neurogenic origin. Although both type 1 and type 2 fibers reveal alterations, type 2 fibers appear to be affected more profoundly and characterized by significantly decreased mean diameters. Significantly decreased mean diameters of type 2 fibers in CM may support disuse, lack of sensitivity to the hormonal influences, or an alteration in the corticospinal tracts of boys with UT.

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.

Male gender assignment in penile agenesis: a case report and review of the literature.

The authors describe a patient with penile agenesis who was reared as a boy and died of chronic renal failure. A literature review was performed to clarify the management and outcome of patients with aphallia who are raised as males.

Acute scrotum due to edidymo-orchitis associated with vasal anomalies in children with anorectal malformations.

Epididymo-orchitis, an uncommon cause of acute scrotum in prepubertal boys, is infection or inflammation of epididymis and testis. Epididymo-orchitis may be associated with urinary tract infections or reflux of urine predisposed by an underlying vasal anomaly. Two infants with anorectal malformations who presented with acute scrotum are reported. The surgical exploration of the testes showed findings consistent with epididymo-orchitis. Further radiological investigations of urinary tract showed vasal anomalies in both patients. If a patient with anorectal malformation presents with acute scrotum, epididymo-orchitis should be suspected initially. Evaluations should be directed toward defining predisposing vasal anomaly, and appropriate therapeutic measures should be undertaken to prevent recurrences.

Urinary obstruction caused by factitious urethral stones: an amazing manifestation of Munchausen syndrome by proxy.

The diagnosis of Munchausen syndrome by proxy is frequently difficult to document and often delayed. A unique presentation of a case of Munchausen syndrome by proxy involving insertion of factitious stones in the lower urinary tract is reported.

Epidural abscess complicating Swenson procedure: a case report and a review of the literature.

A case is reported in which an anastomotic leak following the Swenson procedure for Hirschsprung's disease was complicated by the development of a pelvic abscess that communicated freely with the epidural space. The child presented with signs and symptoms of an epidural abscess, but his myelogram was normal. The diagnosis was made by urografin enema. The child was treated by defunctioning colostomy and drainage of the pelvic abscess by enlarging the defect at the anastomosis site digitally. The epidural space drained freely to the pelvis and therefore laminectomy was not required. The possible etiology of such a communication is discussed.

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.

Complete duplication of the bladder and urethra: a case report and review of the literature.

Although complete duplication of the bladder and urethra (CBDU) has been considered to be an isolated anomaly, the present report involves an infant who had CBDU and associated anomalies of the gastrointestinal and central nervous systems. Clarification of the upper-tract renal anatomy is mandatory in planning a staged approach to reconstruction, especially in the presence of associated anomalies.

Adhesive small bowel obstruction caused by familial Mediterranean fever: the incidence and outcome.

Familial Mediterranean fever (FMF) is a disease characterized by recurring and self-limiting attacks of febrile serosal inflammation involving the peritoneal, synovial, and pleural membranes. Peritonitis is the most common clinical picture of FMF, and repeated acute abdominal episodes may result in formation of peritoneal adhesions that may cause adhesive small bowel obstruction (ASBO) requiring surgical intervention. This subject has neither been clarified nor thoroughly evaluated in the literature. The records of 355 pediatric patients diagnosed to have FMF were reviewed in order to clarify the incidence and outcome of ASBO without prior laparotomy during the course of FMF. The incidence rate has been found as 3% with no mortality. This figure shows ASBO to be the most frequent complication of FMF. Therefore this life-threatening surgical emergency should be kept in mind in the differential diagnosis of acute abdominal attacks during the course of FMF.

Surgical management of the pancreatic pseudocyst in children: a long-term evaluation.

Pseudocyst of the pancreas is an uncommon disorder in children. During the period of 1977 to 1990, the authors reviewed the records of 10 consecutive patients with pancreatic pseudocyst (PP) who were surgically treated in our institution. In 8 patients, the surgical procedure that was used was transgastric cystogastrostomy, and in 1 patient it was total excision of the pseudocyst. The remaining 1 patient underwent external drainage. After an average follow-up of previous operation except the 1 patient who was treated with external drainage. Biochemical tests, abdominal ultrasonographies, and barium meals were also normal in 9 patients. The analysis of the results indicates that despite new percutaneous therapeutic modalities, it is still an internal drainage through transgastric cystogastrostomy that should be favored in treatment of PP in children.