Topical mitomycin C can effectively alleviate dysphagia in children with long-segment caustic esophageal strictures.

Caustic ingestion in children and the resulting long esophageal strictures are usually difficult to be managed, and eventually, esophageal replacement was required for cases refractory to frequent dilatation sessions. Topical mitomycin C (MMC) application has been used recently to improve the results of endoscopic dilatation for short esophageal strictures. The study aims to assess the role of MMC application in management of long-segment caustic esophageal strictures. From January 2009 to June December 2013, patients presented with long caustic esophageal stricture (>3 cm in length) were included in this study and subjected to topical MMC application after endoscopic esophageal dilatation on multiple sessions. Regular follow-up and re-evaluation were done. A dysphagia score was used for close follow-up clinically; verification was done radiologically and endoscopically. During the specified follow-up period, 21 patients with long caustic esophageal stricture were subjected to topical MMC application sessions. Clinical, radiological, and endoscopic resolution of strictures occurred in 18 patients (85.7% cure rate). Number of dilatation sessions to achieve resolution of dysphagia was (n = 14.3 ± 5.7) with application of mitomycin two to six times. There was no recurrence in short- and mid-term follow-up. No complications were encountered related to topical MMC application. MMC is a promising agent in management of long-segment caustic esophageal strictures. Long-term follow-up is needed to prove its efficacy and to evaluate potential long-term side-effects of MMC application.

Heterotopic salivary gland presenting as a supra-clavicular swelling.

A 5-month-old female was referred to the paediatric surgery clinic with a neck swelling in the right supra-clavicular region. This was thought clinically to be cystic hygroma. Pathology showed an ectopic salivary gland. This should be added to the list of differential diagnoses of neck swelling in childhood.

Transanal one-stage endorectal pull-through for Hirschsprung's disease: a multicenter study.

BACKGROUND/PURPOSE: Transanal endorectal pull-through (TEPT) is the latest development in treatment of Hirschsprung's disease (HD). This prospective study was designed to evaluate the safety and efficacy of 1 stage TEPT technique in the management of patients with HD. METHODS: One hundred forty-nine children (116 boys and 33 girls) aged 8 days to 14 years underwent 1 stage TEPT procedure over an 18-month period at 5 Egyptian academic pediatric surgical centers and affiliated hospitals. Median follow-up was 12 months (range, 3 to 21 months). These patients were evaluated with regard to age, sex, length of the aganglionic segment, intraoperative details, and postoperative functional results or complications. An electromyogram (EMG), endorectal ultrasound scan, and lower gastrointestinal (GI) motility studies were reserved for patients with postoperative problems with bowel control. RESULTS: Mean operating time was 120.2 +/- 27.8 minutes (range, 60 to 210 minutes). The average length of resected bowel was 26.8 +/- 12.4 cm (range, 15 to 45 cm). Thirteen patients required laparotomy because of extension of aganglionic segment beyond the sigmoid colon in 9, tear in the mesenteric vessels in 2, and difficulties in getting to the submucosal plane in 2. Three deaths (2%) occurred 3 days, 4 days, and 4 weeks postoperatively, respectively. Postoperative complications included transient perianal excoriation in 48 patients (30 were <3 months of age), enterocolitis (n = 26), anastomotic stricture (n = 7), recurrent constipation (n = 6), hypoganglionosis at distal end of pulled through segment (n = 2), cuff abscess (n = 3), anastomotic leak (n = 1), adhesive bowel obstruction (n = 1), and rectal prolapse (n = 1). Complete anorectal continence was noted in 35 of 42 (83.3%) children older than 3 years, whereas soiling and frequent accidents still occur in 7, who showed a steady improvement in their continence status. CONCLUSIONS: One-stage TEPT technique is both feasible and safe technique in properly selected children with rectosigmoid HD in all ages. The technique is easily learned and is associated with excellent clinical results.

Total urogenital sinus mobilization in the repair of cloacal anomalies and congenital adrenal hyperplasia.

PURPOSE: Urogenital sinus mobilization has facilitated markedly the vaginal reconstruction in cases of cloacal anomalies. Application of the same technique in cases of congenital adrenal hyperplasia has resulted in cosmetic and functional improvement. METHODS: Total urogenital sinus mobilization was used as a part of the repair in 9 patients: 6 with congenital adrenal hyperplasia (4 high and 2 mid vaginal confluence), and 3 with cloacal anomalies; sinus mobilization to the level of the symphesis pubis allowed the vagina to reach the perineum in all cases. Their age ranged from 6 to 8 months, and follow-up ranged from 6 months to 2 years. In the former group, the mobilized sinus was split dorsally and used as an anterior vaginal flap. Cases of cloaca needed weekly dilatation early postoperatively. RESULTS: All patients are below 3 years of age, so urinary control assessment is not yet objective; however, all mothers reported dry intervals. Examination 6 months postoperatively showed wide vagina and excellent cosmetic appearance in all cases. CONCLUSIONS: Total urogenital mobilization provides an easier way for vaginal reconstruction. The improved cosmetic appearance and the absence of vaginal stenosis provided by this technique is a major advantage in the management of these difficult surgical situations.

Testicular descent: when to interfere?

Spontaneous descent of testes after birth can occur in up to 70% of cases, yet the factors contributing to it are still controversial. This study aims to evaluate factors contributing to spontaneous descent of palpable undescended testes. Eighty-four newborns with 126 palpable undescended testes (42 unilateral and 42 bilateral) were followed up for a period of one year to study the occurrence and time of testicular descent and its relation to gestational age, birth weight, uni- or bilaterality and levels of FSH, LH and testosterone. A total of 58 testes (46%) descended between 3 and 6 months. Spontaneous descent occurred in 10 premature patients (14 testes 63%) compared to 44 testes of full-term patients (43%). Descent occurred in 14 unilateral undescended testes (33%) compared to 44 (52%) in bilateral cases. In patients with spontaneous testicular descent there was postnatal peak of LH and testosterone at 2 to 3 months of age which returned to basal level at 6 months of age. In patients with permanent undescended testes the peak of LH and testosterone was very low and almost absent in some of them; no significant difference was found between the mean values of FSH in both groups. No spontaneous testicular descent occurred after the 4th month in the full-term group, whereas in the pre-term group spontaneous descent occurred up to 6 months of age. This study concluded that spontaneous descent of palpable undescended testes is closely related to the presence of LH and testosterone surge. Therapy of undescended testes should start at 4 months of age in a full-term baby and at 6 months of age in a pre-term baby.

Neonatal jaundice: the role of laparoscopy.

BACKGROUND: When managing neonatal jaundice, despite continual improvement of diagnostic tests and increasing knowledge regarding its pathogenesis, there is no single test or imaging modality that can reliably define biliary atresia. Early diagnosis is essential for a better surgical outcome. In many situations, mini-laparotomy and operative cholangiography may be needed to settle the definitive diagnosis, with the risk of having negative exploration in those high-risk patients with medical etiology. The use of laparoscopy may help in avoiding unnecessary exploration for such group of patients. METHODS: Thirty-three patients aged between 1 and 4 months with conjugated hyperbilirubinemia were the subject for this study. All had a HIDA scan result suggestive of biliary atresia. They underwent a diagnostic laparoscopy before surgical exploration. When the gallbladder was not visualized we proceeded to laparotomy. In patients with a good size gallbladder visualized at laparoscopy, a laparoscopic-guided cholangiogram was then performed, and laparoscopic liver biopsy done for those who had patent biliary tree. RESULTS: Two groups of patients were identified: the first group (21 patients) showed small atretic gallbladder; 18 patients had biliary atresia with complete intra- and extrahepatic atresia; these patients underwent a Kasai hepatic-portoenterostomy. Two patients showed a patent gallbladder and common bile duct with atresia of the common hepatic and intrahepatic ducts, and they underwent a portocholecystostomy. The last patient showed left-sided gallbladder arising from the left lobe of the liver that was missed during laparoscopy, and operative cholangiogram showed hypoplastic biliary ducts. The second group included 12 patients with good-sized gallbladder, and laparoscopic-guided percutaneous cholangiogram showed normal communicating patent biliary system, hypoplastic in 2, and they underwent laparoscopic liver biopsy. No mortality related to the laparoscopic procedure was encountered in this series, and one patient with hypoplastic gallbladder had adhesive intestinal obstruction on the fifth day after laparoscopy necessitating exploration. CONCLUSION: Laparoscopy with laparoscopic-guided cholangiography may be a very useful tool used in accurately diagnosing infants with conjugated hyperbilirubinemia, and in avoiding unnecessary laparotomies performed on these critical babies.

Cardiac achalasia in children. Dilatation or surgery?

Surgical treatment of cardiac achalasia in children is still the main line of treatment with a success rate of 70-80%. Balloon dilatation is less widely used due to inappropriate size of balloons. The authors report on their experience in 11 children with cardiac achalasia over the last 7 years using balloon dilatation as the treatment of choice, 8 boys and 3 girls with ages ranging from 1.5-14 years (average 7.5 years) were investigated. One family (brother and sister) presented with no glucocorticoid deficiency or other anomalies, one patient had mental retardation, the rest had no associated anomalies. All patients presented with vomiting, 7 with dysphagia, 6 with loss of weight, 5 with recurrent chest infection and 2 with retrosternal pain. Radiological diagnosis was accurate in all patients, endoscopy with biopsy were done to confirm diagnosis and exclude other pathology, manometry yielded positive results in 4 patients. Dilatation was done under general anesthesia with fluoroscopic control, balloons were used over a guide wire (balloon sizes were 18-35 mm). Seven patients had 2 sessions and 4 had 3 sessions with radiological follow-up after the second dilatation. Follow-up ranged from 2-7 years: excellent results were achieved in 8 patients (72.7%) with disappearance of symptoms and marked radiologic improvement, 2 still have mild symptoms with overall success (90.9%), one had mild gastroesophageal reflux, controlled medically, and one had mild dysphagia but his status was improved compared to that before dilatation. One patient had recurrent dysphagia necessitating cardiomyotomy (9.1%). Results were not related to age or sex. The authors recommend balloon dilatation in children with cardiac achalasia as the treatment of choice or even as the only feasible treatment.