Feminizing surgical management of intersex patients.

The study's objective was to evaluate the results of surgical modalities for children with ambiguous genitalia. The records of 55 patients who were reared as females between 1985 and 2001 were reviewed regarding diagnosis, age at surgery, operative procedures, and outcome. The mean age at surgery was 3.5 years, and the follow-up period averaged 4.1 years with a range of 2 months-17 years. The types of reconstructive surgical techniques were clitorovaginoplasty in 29, staging clitoral surgery and vaginoplasty in seven, clitoroplasty in five, total urogenital mobilization (TUM) in three, vaginal bowel substitution in two, clitoridectomy in one, and gonadectomy in six, and two are waiting for vaginal substitution surgery after gonadectomy. The main complications were vaginal stenosis in four patients. All of the TUM patients had good appearances of their urethral orifice and vagina, all of them were continent, and none of them had urinary tract infections. With our limited experience with the TUM procedure, we feel that it is possible to obtain a better cosmetic and functional result with an easier technique. Among the 10 patients of postpubertal age, none of them had had sexual experience. Eight of the postpubertal patients asked questions about their reproductive status. Patients with an intersex disorder should be informed about their problems, especially about their reproductivity.

Evolving experience in the management of pleural empyema.

The objective of this study was to determine treatment strategies for children admitted with pleural empyema. We reviewed the medical records of 93 consecutive patients with pleural empyema who presented in the last three years. Mean age was 3.8 years (range 3.5 months to 14 years) and the male to female ratio was 52 : 41. The left side was involved in 51 (54.8 %) cases and there was only one case with bilateral effusion. After the chest X-ray and analysis of pus obtained by thoracocentesis, the initial treatment modality was chest tube drainage in 81 (87 %) patients. Children required chest tube drainage for an average of 11.9 +/- 4.8 days. Out of the 81 children, 6 needed thoracotomy due to organized pleural fluid and another 4, who developed loculated pleural effusions, were treated with intrapleural urokinase (UK) administration. Twelve children, who had a delayed presentation with organized loculated pleural fluid and pleural thickening, underwent decortication and one, who was found to have a foreign body, found during surgery, needed an upper lobe resection. Medical management with adequate chest tube drainage and appropriate antibiotics has resulted in full resolution in the majority of patients with empyema. In cases of loculated pleural effusions identified at the early stages of the chest tube drainage, intrapleural UK administration was found to be a safe and efficient treatment modality. Thoracotomy should be reserved for late and organized empyema cases.

Management of esophagogastric corrosive injuries in children.

There has been an increase in the number of patients admitted to our hospital with caustic esophageal injuries during the last five years. The aim of this study was to analyze the complications and results of the treatment of corrosive esophagogastric injury. Between 1990 and 2000, 120 caustic ingestion accidents were admitted to our unit. The mean age was 4 years, with a 2 : 1 male to female ratio. The average time between the caustic ingestion and admission to hospital was 14.9 days. The ingested substances were alkali in 80.9 % and acid in 19.1 % of the cases. Stenosis of the esophagus developed in 31 (25.8 %) and gastric outlet obstruction (GOO) in 6 (5 %) patients. Management of the esophageal stricture consisted of dilatation in 28 patients. Three children underwent colonic interposition without a dilatation attempt. Six children were lost to follow-up; 4 patients were successfully treated; 13 patients were still in the dilatation program at the time of writing with 6 improving and 2 patients waiting for interposition surgery; 4 patients underwent colonic interposition and 1 patient underwent resection of the stenotic part of the esophagus. Among the patients in the dilation program, we observed 4 esophageal perforations. Three of them were treated medically and further dilatations were carried out, while one was managed by colonic interposition. The treatment modalities for GOO cases consisted of pyloroplasty in 3, Billroth I in 2 and balloon dilation of the pylorus in 1 child. Although balloon dilatation of the esophagus carries the risk of perforation, it should be the first line of treatment in suitable cases. GOO cases may require surgical therapy following a detailed endoscopic evaluation.

Urodynamic findings of posterior urethral valve patients.

The most frequently observed cause of obstructed bladder in children is the posterior urethral valve (PUV). In this report, we analysed the urodynamic findings of 26 patients whose valves were fulgurated 12.6 months previously (range: 2 days - 8 years,after the fulguration). The mean age of the patients at the time of the procedure was 4.5 years (range: 2 months -13 years). Bladder capacity was decreased in 15, increased in 6, and normal in 5 patients; hypo-compliance was observed in 13, hyper-compliance was observed in 4, and normo-compliance was observed in 9 children. Generally, hypo-compliance and decreased bladder capacity was more frequent in patients younger than 4 years of age. There were 10 patients with instable detrusor contractions(IDC) and high residual urine was present in 8 patients. Eight patients developed chronic renal failure and 6 of these patients had high residual urine. All the patients who required bladder augmentation during follow-up were the ones treated after 2.5 years of age; 4 of these 5 patients had hypo-compliance and low bladder capacity on urodynamic studies. In conclusion, all patients with PUV had pathological urodynamic findings that could change with age, and early relief of the infravesical obstruction could have an improving effect on bladder function. Urodynamic investigations may help us to design the proper treatment according to the bladder function.

Catheterisable continent urinary diversion (Mitrofanoff principle)--clinical experience and psychological aspects.

48 Mitrofanoff principles (MTR) were performed on 46 patients (male : female ratio, 30 : 16) with a mean age of 9.1 years (range 2.5 to 24 years). The primary diagnoses were neurogenic bladder in 11, infravesical obstruction in 7 and bladder exstrophy-epispadias complex in 28 patients. The most common type of conduit was appendix (38 cases); other conduits were constructed from the ileum (seven) and ileocaecum (one). In two cases with bladder substitution the uterine tube and tubularised bladder stump were used as perineal MTR. 33 of the 46 children underwent augmentation cystoplasty in conjunction with the MTR procedure. The Malone procedure for antegrade colonic enema (ACE) was performed at the same stage with MTR in eight cases. To achieve continence, bladder neck reconstruction was performed in 32 patients and the bladder neck was closed in four patients during the same operation and MTR procedure. The mean follow-up period was 28.7 months (range one month to 57 months). To assess the psychological aspects of the MTR procedure, the Rosenberg Self-Esteem Scales were completed by 12 children older than eight years of age. Nine patients had problems with the MTR (19.5 %). Three appendiceal MTR had strictures at the skin level, 2 of which needed minor surgical revisions. A mucocele formation at the skin level of an appendix was removed successfully. We did not observe any complaints among the other appendiceal conduits. All the tapered ileum conduits were difficult to catheterise, and 1 of them had a leakage from the stoma. None of the three transversely tubularised ileum MTRs had problems with catheterisation or leakage. A stricture of the conduit from the uterine tube was observed. 36 of the 42 patients are now continent, giving a ratio of 86 %. The results of the Rosenberg Self-Esteem Scales revealed that there was an increase in the percentage of patients with high self-esteem, and a decrease in depressive feelings after the MTR procedure. We conclude that the MTR procedure provides excellent continence, offers good prospects of a socially acceptable life with increased self-esteem, and the appendix seems to be the ideal organ for continent urinary diversion, with the transverse tubularised ileal tube as a second choice.

Experience with laparoscopy in nonpalpable testis.

AIM: The aim of this study was to investigate the feasibility and benefits of diagnosis and interventional laparoscopy in those paediatric patients with nonpalpable testes (NPT). PATIENTS AND METHODS: Between 1992 and 1999, 75 patients with NPT (mean age 3 years, range 6 months to 14 years) were treated. 86 testes were evaluated. RESULTS: According to the laparoscopic findings 4 groups of testes were identified: Vanishing testis (n = 32), low abdominal testis (< 2 cm to the internal ring) (n = 26), high abdominal testis (> 2 cm to the internal ring) (n = 24) and intersex patients (n = 4). Of the first group, 19 testes (one bilateral) had blind-ending spermatic cord and vessels and if an atrophic testicular tissue was identified, it was removed laparoscopically. For those with spermatic cord and vessels beyond the internal ring (13 testes), atrophic testes were removed through a high scrotal incision. 19 testes of the second group had a laparoscopy-assisted orchidopexy. In the same group a laparoscopic orchidopexy was performed on 7 testes. 24 testes in the 3rd group had a Fowler-Stephens (FS) stage 1 and 18 testes had a laparotomy performed for FS stage 2 procedure (laparotomy and orchidopexy) after 6 months. At laparotomy there was no evidence of testicular atrophy in all but one testis, which was removed and the FS stage 2 procedure was completed in 17 testes. The follow-up period was between 6 months and 4 years, and two more testicular atrophies were noted after FS stage 2. The results were satisfactory in 15 out of 18 testes (83%). In the intersex group, the patient with testicular feminization underwent laparoscopic orchiectomy. The other patient with bilateral nonpalpable testis was identified as having an uterus and two intraabdominally located gonads on laparoscopy and gonadal biopsies were obtained for diagnosis. Histology demonstrated bilateral ovotestes, confirming the diagnosis of a true hermaphrodite. CONCLUSION: We are of the opinion that laparoscopy decreases the number of laparotomies in NPT, allows a single-stage procedure in low abdominal testis, and facilitates clip ligation of the testicular artery in high abdominal testis. Laparoscopy also provides diagnostic and therapeutic options for vanishing testis and intersex patients.

Sugiura procedure in portal hypertensive children.

Bleeding from esophageal varices is an important cause of morbidity and mortality in children with portal hypertension. The treatment protocol is planned according to the etiologic factors underlying the portal hypertension, which may be either intrahepatic or extrahepatic. Although portasystemic venous shunt operations were common previously, they are now regarded as nonphysiologic and are rarely used because of their unexpected results and complications. Today, in many centers, endoscopic procedures have become the first-step treatment modality in bleeding esophageal varices. More complicated surgical procedures, such as devascularization procedures in extrahepatic portal hypertension, and liver transplantation in patients with failing liver, should be performed when conservative measures fail. We followed up 69 patients with portal hypertension with endoscopic sclerotherapy in our department. Here we present a retrospective evaluation of the effect of the Sugiura operation on the prognosis of 12 children (6 with extrahepatic and 6 with intrahepatic portal hypertension) who were not responsive to the sclerotherapy program. No rebleeding was seen in 9 of the 12 (75%) patients after the procedure, and the mortality rate in this series was 1 of 12 (8.3%); this patient died of hepatic failure.

Corrosive injury-induced gastric outlet obstruction: a changing spectrum of agents and treatment.

METHODS: During the last 5 years, 61 children were admitted to the authors' hospital because of corrosive substance ingestion, and among them 6 patients were seen with gastric outlet obstruction. Two of them had ingested acid substances, and the other 4 had ingested alkali corrosives. The mean age was 2.9 years (range, 1.5 to 3). Their common complaint was postprandial vomiting, which had begun 3 weeks after the event (range, 1 week to 10 weeks). Endoscopic evaluation and barium contrast radiographies were performed at admission. Four patients had a pyloric stricture, 1 had an antral stricture, and another had an antropyloric stricture. Balloon dilatation of the pylorus (in 1 patient), pyloroplasty (in 3 patients), and Billroth I procedures (in 2 patients) were performed. The mean follow-up period was 22 months (range, 6 weeks to 48 months). One patient, who had undergone a Billroth I procedure, underwent reoperation because of intestinal obstruction 3 months later. On follow-up they are all free of symptoms. CONCLUSIONS: The treatment of gastric outlet obstruction caused by corrosive ingestion should be treated surgically. Although endoscopic and radiologic evaluation helps to determine the time and necessity, once the diagnosis is confirmed, early definitive surgical intervention should be performed, and the type of the surgery depends mostly on the findings of the surgeon at laparotomy. Endoscopic balloon dilatation of the pylorus maybe attempted in suitable cases. Special care should be given to prevent children from accidental corrosive ingestion.

Bronchoscopic removal of tracheobroncheal foreign bodies: value of patient history and timing.

Between 1990 and 1999, 741 bronchoscopic procedures were performed in 698 children, 594 of whom were evaluated for foreign-body aspiration (FBA) (mean age 3.9 years, male:female 287/307). Based on the presenting symptoms, clinical outcome, and complications, two major groups were identified. Group 1 consisted of 438 patients with a definitive history of FBA. Most were admitted soon after the aspiration with sudden onset of symptoms such as coughing, choking, wheezing, and respiratory distress. Group 2 comprised 156 patients with chronic pulmonary infections and/or atelectasis without a definitive history of FBA. The most common radiographic finding was emphysema of one lung in group 1 (61.1%) and pneumonia in group 2 (70%). Among the patients in whom a FB was removed, the percentage of normal radiography was 17%. The FB was identified and removed in 83% of cases in group 1. The complication rate in this group was 9.8%, and all the complications were treated medically. Only 2 patients required intercostal drainage. In group 2, a FB was identified in 25% of bronchoscopic examinations and 17% of the patients developed complications. One of these patients underwent an urgent thoracotomy due to bilateral tension pneumothoraces and 2 required tracheostomies. Patients with a definitive history of FBA, even with a normal physical examination and radiographic findings, must undergo bronchoscopic investigation. Cases with late presentation and chronic pulmonary infection are at high risk. In this group care should be take in determining the indication and timing of bronchoscopy in order to prevent life-threatening complications.

Diagnostic laparoscopy in prolonged jaundice.

BACKGROUND: The early diagnosis of surgical jaundice in a neonate is an important step for the surgical success in extrahepatic biliary atresia. Diagnostic laparoscopy, as in many areas in surgery, is included in the conventional diagnostic methods of extrahepatic biliary atresia. METHODS: Since 1992, 24 infants with prolonged jaundice, in whom extrahepatic biliary atresia and neonatal hepatitis could not be differentiated with conventional diagnostic interventions, have been evaluated laparoscopically. RESULTS: A coarse, irregular, greenish-brown liver with some degree of fine angiomatous development and an atretic gallbladder are the findings of laparoscopic evaluation in an infant with extrahepatic biliary atresia. However, in neonatal hepatitis, the liver is smooth, sharp-edged, and chocolate brown in color, and simultaneously performed cholangiography should show the passage of the contrast material both into the proximal biliary tracts and the intestinal system. In this series, 10 of 24 cases were proved to be neonatal hepatitis diagnosed by laparoscopy, so unnecessary laparotomy was avoided in 42% of the cases. CONCLUSION: When the diagnostic laparoscopy, in which the liver and the gallbladder are directly visualized, is combined with the cholangiographic examination, the most accurate and earlier diagnosis in an infant with prolonged jaundice can be achieved, and the important period of time for the surgical success in extrahepatic biliary atresia will be minimally wasted.

A case of mesenteric cysts caused by abdominal tuberculosis.

We report on a 10-year old boy suffering from chronic abdominal pain. Clinical examination and imaging modalities revealed that the patient had mesenteric cysts. Exploratory laparotomy revealed two mesenteric cysts of various size and multiple enlarged mesenteric lymph nodes. M. tuberculosis was identified and histology of the specimens proved the existence of abdominal tuberculosis.

Systemic stress response during operations for acute abdominal pain performed via laparoscopy or laparotomy in children.

We compared the endocrine and metabolic changes during acute emergency abdominal surgery performed using either laparoscopy or laparotomy in children. Twenty-nine children aged 1.5-14 years were assigned to undergo laparoscopy (n = 15) or laparotomy (n = 14) with a standard anaesthesia technique. Arterial blood gases and blood prolactin, cortisol, interleukin-6, glucose, insulin, lactic acid and epinephrine levels were determined 5 min after the induction of anaesthesia, 30 min into surgery and at the end of surgery. Intra-operative heart rate and mean arterial pressure were stable in both groups. In the laparoscopy group, slight respiratory acidosis occurred during surgery (p < 0.01) but there were no changes in the laparotomy group. Insulin, cortisol, prolactin, epinephrine, lactate and blood glucose levels increased in both groups (p < 0.05) although there was no difference between the groups. The surgical stress and trauma imposed by laparoscopy seems similar to that caused by laparotomy in children undergoing emergency abdominal surgery.

Jeune's asphyxiating thoracic dystrophy of the newborn.

A 13-month-old patient with Jeune's thoracic asphyxiating dystrophy, was surgically treated using a methyl-methacrylate (acrylic) prosthesis. Although postoperative recovery was fast, the patient died of respiratory distress two months following surgery. Limitations of currently available surgical techniques and the need for long-term results will be discussed.

Wandering spleen in childhood: a report of three cases.

Wandering spleen is a rare cause of abdominal pain in children, and an accurate diagnosis is seldom made preoperatively. A splenectomy is the treatment of choice in cases of splenic torsion and infarction, while in patients with chronic symptoms splenopexy may also be attempted. We herein report three patients with wandering spleen, of whom two presented with acute torsion of the splenic pedicle and one demonstrated an asymptomatic mobile abdominal mass. In the first case splenopexy was attempted, but during follow-up the spleen was found to have undergone atrophy. The presentation, diagnostic procedures, and treatment modalities in pediatric wandering spleen are reviewed.

Is birth trauma responsible for idiopathic perforation of the biliary tract in infancy?

This case report details the clinical presentation and surgical management of a neonate with idiopathic perforation of the biliary tract. A three-day-old baby girl presented with a right-upper-quadrant mass and signs of peritonitis following a prolonged, difficult vaginal delivery. At surgery, she was found to have a perforation at the junction of the cystic and common bile duct. Simple drainage of the right upper quadrant was performed, and the patient recovered uneventfully. Early presentation and the nature of delivery suggests the possibility of birth trauma as an etiological factor in this condition.

Congenital tracheobiliary fistula.

A case of congenital tracheobiliary fistula with absent common bile duct is reported. The patient presented with repeated aspiration pneumonia, and the diagnosis was established during bronchoscopy. Excision of the fistula and cholecystoduodenostomy were successful. This is the fourth reported case of its type and the first one to survive surgical treatment. The condition is a rare cause of respiratory distress in neonates and should be considered in the differential diagnosis of intractable aspiration pneumonia.

Encopretic children: experience with fifty cases.

Fifty children with encopresis were admitted to the Pediatric Surgery Department of Sisli Children's Hospital between October 1, 1987 and January 1, 1990. Two treatment regimens were used. Thirteen patients received mineral oil and glycerine suppositories and 37 patients received rectal irrigations and Lactulose. Five patients were lost to follow-up and 40 patients have completed their six month treatment schedule. The results of our study suggest that encopresis is a complex abnormal motility disorder, requiring a multidisciplinary approach. Best results will be obtained by good parent-child-doctor cooperation and close follow-up.

Fused pelvic kidney drained by a single ureter.

A case of fused pelvic (discoid) kidney drained by a superiorly inserted single ureter is presented. This is the twentieth case of fused pelvic kidney, and the fifth case in which drainage was carried out by a single ureter, to be reported in the English literature. The diagnosis and treatment of this condition is discussed and the relevant literature is reviewed.

Traumatic esophageal perforation in a premature infant.

We presented a case of neonatal hypopharyngeal perforation resulting from extraction of a breech presentation, with symptoms of regurgitation at all feedings and excessive salivation. Inability to pass a nasogastric tube suggested the diagnosis of esophageal atresia. The diagnosis and treatment of this condition is discussed, and the literature is reviewed.