A cross-sectional nationwide survey on esophageal atresia and tracheoesophageal fistula.

BACKGROUND: Our study aims at disclosing epidemiology and most relevant clinical features of esophageal atresia (EA) pointing to a model of multicentre collaboration. METHODS: A detailed questionnaire was sent to all Italian Units of pediatric surgery in order to collect data of patients born with EA between January and December 2012. The results were crosschecked by matching date and place of birth of the patients with those of diagnosis-related group provided by the Italian Ministry of Health (MOH). RESULTS: A total of 146 questionnaires were returned plus a further 32 patients reported in the MOH database. Basing on a total of 178 patients with EA born in Italy in 2012, the incidence of EA was calculated in 3.33 per 10,000 live births. Antenatal diagnosis was suspected in 29.5% patients. 55.5% showed associated anomalies. The most common type of EA was Gross type C (89%). Postoperative complications occurred in 37% of type C EA and 100% of type A EA. A 9.5% mortality rate was reported. CONCLUSIONS: This is the first Italian cross-sectional nationwide survey on EA. We can now develop shared guidelines and provide more reliable prognostic expectations for our patients.

Cryptorchidism with short spermatic vessels: staged orchiopexy preserving spermatic vessels.

PURPOSE: Patients with cryptorchidism can have such short spermatic vessels that it is impossible to place the testicle in a satisfactory scrotal position using conventional orchiopexy. In these cases the most commonly used operation is 1 to 2-stage Fowler-Stephens orchiopexy. We present our surgical experience using staged inguinal orchiopexy without section of the spermatic vessels in patients with short spermatic vessels. MATERIALS AND METHODS: We used 2-stage inguinal orchiopexy in 38 children with intra-abdominal testis or testis peeping through the internal ring and short spermatic vessels (7 bilateral). Spermatic vessels were not sectioned, but were lengthened through progressive traction of the spermatic cord wrapped in polytetrafluoroethylene pericardial membrane (Preclude). In the first stage we mobilized the spermatic cord in the retroperitoneal space and then wrapped it in the polytetrafluoroethylene membrane. We subsequently attached the testis to the invaginated scrotal bottom. At 9 to 12 months we performed the second stage, which involved removing the polytetrafluoroethylene membrane. RESULTS: From the first to the second stage we observed progressive descent of the testicle toward the scrotum. At 1 to 8-year followup after the second stage all 45 testicles were palpable in a satisfactory scrotal position with stable or increased testicular volume. CONCLUSIONS: This technique represents an alternative to Fowler-Stephens orchiopexy, which can be associated with a greater risk of testicular ischemia.

Reflux oesophagitis in children; the role of endoscopy. A multicentric Italian survey.

BACKGROUND: The decision whether to perform endoscopy in children with suspected reflux oesophagitis is not a straightforward one. Few symptoms are specific for oesophagitis and the diagnosis is not always correlated even to visual findings on endoscopy. AIM: The aim of this study was to define the role of endoscopy and especially of histology in the diagnosis of reflux oesophagitis and to examine the correlations between symptoms, endoscopic findings and histology in children with suspected gastroesophageal reflux disease. PATIENTS AND METHODS: One hundred and thirty-six patients with a clinical diagnosis of reflux oesophagitis, aged 1-18 years (mean 8.43; standard deviation +/-4.4), were enrolled from 12 Italian Paediatrics Gastroenterology Centres; symptom score, endoscopic and histologic oesophagitis scores were observed before and after therapy with proton pump inhibitors. RESULTS: Before therapy, a high correlation between the prevailing symptom score and endoscopic score was demonstrated, but not with histologic score: there was a significant tendency for histologic grade to exceed visual findings. After therapy, endoscopic score and histologic score were significantly improved. CONCLUSIONS: Oesophageal biopsies increase the diagnostic accuracy of upper endoscopy. Histologic grading is often much more important than the endoscopic appearance, so that endoscopic oesophageal biopsies are very important aids in the diagnosis of oesophagitis. Appropriate clinical evaluation of symptoms must occur before endoscopic examination.

Laparoscopic relief of obstructing folded muscular cuff after transanal pull-through for aganglionosis.

Transanal endorectal resection and colonic pull-through (TERPT) is a good technique for the management of Hirschsprungs disease. This procedure is feasible in the vast majority of patients and is associated with excellent results, early postoperative recovery, and no visible scars. We report the case of a patient who developed early postoperative severe constipation after TERPT due to unusual folding of the muscular cuff rim, which tightly narrowed the pulled-through colon. This complication was diagnosed and treated by laparoscopy. To prevent it, we recommend splitting of the aganglionic muscular cuff during TERPT.

Laparoscopic relief of obstructing folded muscular cuff after transanal pull-through for aganglionosis.

Transanal endorectal resection and colonic pull-through (TERPT) is a good technique for the management of Hirschsprung's disease. This procedure is feasible in the vast majority of patients and is associated with excellent results, early postoperative recovery, and no visible scars. We report the case of a patient who developed early postoperative severe constipation after TERPT due to unusual folding of the muscular cuff rim, which tightly narrowed the pulled-through colon. This complication was diagnosed and treated by laparoscopy. To prevent it, we recommend splitting of the aganglionic muscular cuff during TERPT.

Italian multicenter survey on laparoscopic treatment of gastro-esophageal reflux disease in children.

BACKGROUND: Skepticism is still present today about the laparoscopic treatment of gastro-esophageal reflux (GER) in children. We present the prospective experience and short-term results of eight Italian pediatric surgical units. METHODS: We included all the children with complicated GER, operated after January 1998 by single surgeons from eight different centers. Diagnostic aspects, type of fundoplication, and complications were considered. All the patients were followed for a minimum period of 6 months in order to detect complications or recurrences. RESULTS: 288 children were prospectively included. Mean age was 4.8 years (3 m-14 y). Nissen fundoplication was done in 25%, floppy Nissen in 63%, Toupet in 1.7%, and anterior procedures (Lortat Jacob, Thal) in 10%. Gastrostomy was associated, if neurological impairment or feeding disorders were present. Mean follow-up was 15 months and reoperation was necessary in 3.8% of cases. CONCLUSIONS: This experience underlines that minimal invasive access surgery in children is safe and that the laparoscopic approach is considered in eight centers the golden standard for surgical repair of gastro-esophageal reflux disease maintaining the same indications and techniques of the open approach.

Food entrapped in papilla of Vater: uncommon cause of vomiting.

CASE REPORT: A 20 month old girl was admitted for intractable vomiting over several days, with no other symptoms. Family and personal history were not contributive. Clinical and neurological examination, and routine blood tests and investigations (plain abdominal x ray, upper gastrointestinal tract contrast study, abdominal ultrasonography) were normal. The upper gastrointestinal endoscopy showed a mild antral gastritis and the second portion of duodenum was occupied by a tough, fibrous mass partially embedded into the papilla of Vater. The foreign body was removed and proved to be vegetable fibre (pineapple). Symptoms subsided immediately and the child was discharged with gastroprotective therapy. After two months, on endoscopic examination, the signs of gastropathy had cleared; the papilla of Vater was undamaged, but unchomped food debris was again found in the duodenum. DISCUSSION: There are sporadic reports of foreign bodies retained into the papilla of Vater, all of them in adults. This child, though her papilla was tiny, had no jaundice or pancreatitis, unlike most of the reported cases. This is the first report of this finding in a child. The cause of the vomiting was not shown on abdominal ultrasonography or contrast study. It should be added to the list of unusual causes of vomiting.

Asymptomatic giant gastric lipoma: What to do?

Gastrointestinal (GI) lipomas are benign, usually single, slowly growing tumors. Their occurrence in the GI tract is most common in the colon, but they can be found also in the small bowel and very rarely in the stomach, where they account for 5% of all GI lipomas. Although most gastric lipomas (GL) are usually detected incidentally, they can cause severe symptoms such as obstruction, invagination, and life-threatening hemorrhages. To date, only three cases of GL have been reported in childhood. We describe the case of an 11-yr-old girl with asymptomatic giant GL, who has not received any treatment until now. New diagnostic insights, therapeutic options, and indications for treatment in asymptomatic patients are discussed.

Treatment of extrahepatic portal hypertension in children by mesenteric-to-left portal vein bypass: a new physiological procedure.

OBJECTIVE: To achieve hepatic portal revascularisation and decompression of extrahepatic portal hypertension in children with cavernoma and obstruction caused by idiopathic portal vein thrombosis. DESIGN: Selected cases. SETTING: Teaching hospitals. Belgium and Italy. SUBJECTS: 11 children who weighed between 5.9 and 54 kg (2 emergencies) with symptomatic extrahepatic portal hypertension. INTERVENTION: Interposition of venous autograft between the superior mesenteric vein and the distal (umbilical) portion of the left portal vein. MAIN OUTCOME MEASURES: Improvements in symptoms and endoscopic appearance after operation. RESULTS: 2 bypasses had to be redone because they stenosed; all 11 were patent at the time of writing (median follow-up 6 months, range 1-32 months). CONCLUSION: The bypass effectively relieved symptoms of extrahepatic portal hypertension by restoring normal hepatic portal blood flow.

Adenomyomatosis of the gallbladder in childhood.

Adenomyomatosis of the gallbladder (ADMG) is defined as an acquired disease characterized by localized or diffuse hyperplastic extensions of the mucosa into, and often beyond, the thickened gallbladder muscular layer (Rokitansky-Aschoff's sinuses). In recent years, attention has been drawn to its malignant potential. The occurrence of ADMG has never been reported in children. The authors report the case of a 5-year-old boy with symptomatic ADMG, who was successfully treated by laparoscopic cholecystectomy.

Direct bypassing of extrahepatic portal venous obstruction in children: a new technique for combined hepatic portal revascularization and treatment of extrahepatic portal hypertension.

BACKGROUND: Decompression of extrahepatic portal hypertension by directly bypassing the thrombosed portal vein has never been reported in cases of children with idiopathic (or neonatal) portal vein obstruction and cavernoma. METHODS: Seven children (15 years or younger) with portal vein obstruction requiring surgical decompression (urgently in two cases), and in whom preoperative Doppler had shown that the intrahepatic portal branches were hypoplastic but free of thrombus, were included in a pilot study. The cavernoma was bypassed by interposing a venous jugular autograft between the superior mesenteric vein and the distal portion of the left portal vein. Patients received follow-up using routine clinical parameters, upper gastrointestinal endoscopy, and Doppler ultrasound. RESULTS: The mesenterico-portal bypass restored a direct (physiological) hepatopetal portal flow. The operation resulted in effective portal decompression as demonstrated by decrease of the pressure gradient, rapid regression of clinical signs of portal hypertension, and definitive control of bleeding. CONCLUSIONS: This study shows that direct bypassing of portal cavernoma is possible and results in effective portal decompression. Restoration of the hepatic portal flow is a major advantage compared with conventional surgical shunting procedures. This new technique is potentially applicable to two thirds of children with portal vein thrombosis and should be considered when shunting procedures are indicated.

Microemulsion formulation of cyclosporine in pediatric liver transplantation.

The oral bioavailability of Sandimmun can be impaired by cholestasis, external biliary diversion, and diarrhea. We report two cases of pediatric liver transplant recipients who experienced chronic rejection and diarrhea secondary to proximal bowel resection. These conditions resulted in poor oral absorption of Sandimmun; the children were converted to the new oral microemulsion formulation Neoral, which significantly improved oral absorption, allowing intravenous cyclosporine weaning and patient discharge. Comparative pharmacokinetic studies were performed in both cases, and the relative Neoral/Sandimmun bioavailabilities were 32.9 and 5.4, respectively. Accordingly, Neoral may constitute to good alternative to ensure the effectiveness of oral cyclosporine administration, particularly in liver-transplanted children with severe cholestasis or shortened small bowel.

[The laparoscopic treatment of Hirschsprung's disease]. / Trattamento laparoscopico della malattia di Hirschsprung.

Swenson's procedure, first described in 1948 for Hirschsprung's disease, consists in resection of aganglionic intestine and distal colo-anal anastomosis provided a precise dissection of extra peritoneal rectum. Potential jeopardy of pelvic vessels and nerves stimulated alternative surgical techniques to prevent complications on bladder and genital function. We performed in laparoscopy Swenson's procedure after Toupet, taking advantage from closer view and magnification of this technique, in a 15 months girl. Laparoscopy simplified and made safer pelvic dissection and resulted in a better postoperative period and cosmetic outcome.

Autologous buccal mucosa graft for hypospadias repair: an initial report.

Autologous buccal mucosa alone or combined with bladder mucosa was used as a free graft for urethral reconstruction in 8 patients with hypospadias. Of these patients 3 with medium penile hypospadias and chordee received a buccal mucosa graft alone and 5 with severe hypospadias received a combined buccal/bladder mucosa graft. Followup at 6 to 18 months showed no complications apart from a fistula in 1 patient with a buccal mucosa graft alone. Our initial results are encouraging. Buccal mucosa seems to be a good material for hypospadias repair.

[Variations of the cardiac rhythm during drug treatment. Presuppositions of cardiac protection]. / Variazioni del ritmo cardiaco durante trattamento farmacologico. Presupposti di cardioprotezione.

In the present study, the authors assessed the therapeutic efficacy of three drugs, each representing a different pharmacological class; i.e. indapamide as diuretic, doxazosin as alpha 1-blocker, and slow-release nifedipine as calcium blocker. The action of these drugs on blood pressure and on the reduction of both atrial and ventricular ectopic beats was observed taking into account the dynamic evolution of the heart as a result of increased blood pressure. This evolution goes from normal heart to hypertrophic and subsequently to dilated heart with attendant alterations of cardiac rhythm.

Liver transplantation in children with biliary atresia and polysplenia syndrome.

Biliary atresia is the most common indication for orthotopic liver transplantation (OLT) in children. The polysplenia syndrome anomalies, which occur in approximately 10% of children with biliary atresia, may represent special difficulties at liver transplantation. We have reviewed our experience with this syndrome in 116 children with biliary atresia who underwent liver transplantation between March 1984 and December 1989. The main features of the polysplenia syndrome, which included absence of the inferior vena cava, preduodenal portal vein, midgut malrotation, aberrant hepatic artery, and situs inversus, were encountered in 12 of the 116 children (10.3%). Severe portal vein hypoplasia (3.5 mm or smaller) was also present in 7 of these children. Eight patients received a complete and four received a reduced liver graft. The vascular anomalies increased the technical difficulty of OLT but could be surmounted, although they did contribute to the peroperative death of one child. The 1-month survival rate was 83% for the 12 children with features of the polysplenia syndrome and 88% for the other 92 children with biliary atresia alone.