Extraperitoneal kidney transplantation: a comparison between children weighting ≤15 kg and >15 kg. Experience of a single institution.

Extraperitoneal approach is sometimes recommended for kidney transplantation (KT) in children weighting <15 kg. We hypothesized that this approach might be as successful as in patients with normal weight. Data of all consecutive KTs performed between 2013 and 2019 were retrospectively reviewed. Early outcomes and surgical complications were compared between children weighing ≤15 kg (low-weight (LW) group) and those weighing >15 kg (Normal-weight (NW) group). All the 108 KTs were performed through an extraperitoneal approach. The LW group included 31 patients (mean age 3.5 ± 1.4 years), whose mean weight was 11.1 ± 2.0 kg. In the LW group,-a primary graft nonfunction (PNGF) occurred in one patient (3.2%), surgical complications occurred in nine (29%), with four venous thrombosis. In the NW group, PNGF occurred in one case (1.3%), delayed graft function (DGF) in eight (10%), surgical complications in 11 (14%) with only one case of venous thrombosis. In both groups, no need for patch during wound closure and no wound dehiscence were reported. The extraperitoneal approach can be effectively used in LW children. No differences were observed in the overall complication rate (P = 0.10), except for the occurrence of venous thrombosis (P = 0.02). This might be related to patients' characteristics of the LW group.

Laparotomic versus laparoscopic Duhamel pull-through for Hirschsprung disease in infants and children.

PURPOSE: The aim of this study was to analyze the results from laparotomic and laparoscopic Duhamel pull-through in the treatment of Hirschsprung disease. MATERIALS AND METHODS: In our department, the Duhamel pull-through technique was adopted in most patients and the laparoscopic technique used since 1999 follows the original Duhamel procedure. Patients were identified retrospectively and followed up in our outpatient clinic. Preoperative, operative, and postoperative data were collected and analyzed. RESULTS: Between January 1992 and March 1999, 21 children with Hirschsprung disease underwent primary surgical correction using the classic open Duhamel pull-through. The mean age at operation was 14 months, the median operating time was 297 minutes, and the average postoperative stay was 10 days. From April 1999 to December 2003, 22 children underwent laparoscopic Duhamel pull-through. The mean age at operation was 14.6 months, the mean operative time was 253 minutes, and the mean postoperative stay was 6.8 days. The rates of early (4.7% vs. 4.7%) and late (19% vs. 23.8%) complications were similar in the two groups, but in the laparoscopic group the mean operative time and postoperative hospital stay were lower. CONCLUSION: With these encouraging results, laparoscopic Duhamel pull-through has become our procedure of choice in the surgical management of Hirschsprung's disease.

Outcome comparison among laparoscopic Duhamel, laparotomic Duhamel, and transanal endorectal pull-through: a single-center, 18-year experience.

PURPOSE: Transanal endorectal pull-through has changed the treatment of Hirschsprung's disease (HD) in the past decade. The aim of the study was to compare outcomes, obtained in a single center, with laparotomic Duhamel (LTD), laparoscopic Duhamel (LSD), and laparoscopic-assisted transanal endorectal pull-through (LTEPT). MATERIALS AND METHODS: We retrospectively reviewed the charts of all patients operated on for HD since 1992. Preoperative, operative, and postoperative data were collected to compare short- and long-term outcomes among the three groups. RESULTS: From 1992 to 2010, 70 children were treated for HD. Patients were divided into three groups based on the surgical technique used: 14 LTEPT, 32 LSD, and 24 LTD. Mean ages at surgery were 4.67, 14.61, and 13.28 months, respectively. Patients in the LTEPT group had significant shorter operating times (195 versus 257 versus 291 minutes, P=.03), earlier start of feeding (1.2 versus 3.1 versus 4.7 days, P<.01), and shorter length of hospital stay (4.4 versus 6.8 versus 9.7 days, P<.011). Overall complications rate was lower in the LTEPT (14%) than in the LSD (31.2%) and LTD (29.7%) groups. Postoperative enterocolitis incidence was 3%-4% in the Duhamel groups and none in LTEPT. Long-term outcome showed less constipation and better continence for age in the LTEPT group at the 1-year follow-up (P=.033). CONCLUSIONS: This study further supports technical advantages, lighter impact of the surgical procedure on infants, lower incidence of complications, and better long-term outcome of the transanal pull-through compared to the Duhamel approaches.

Partial bowel obstruction in a 2-month-old child. A delayed diagnosis of anal abuse.

We present the case of an anal sexual abuse involving a 2-month-old boy, who was admitted to the Pediatric Surgery Unit of the University of Padua for low bowel obstruction. The infant had been already hospitalized for 3 days in a peripheral hospital and treated with daily rectal wash-outs for a fecaloma. Only after a careful interpretation of the plain abdominal radiograph, along with the performance of a rectoscopy and a laparotomy, a vegetable foreign body (about 3 cm in diameter and 7 cm in length) was discovered in the sigma. The morphology and dimensions of the foreign body, as well as its location, left no doubt about the etiology of the partial bowel obstruction, proving that it was clearly related to an anal sexual abuse.

Complex pathogenesis of Hirschsprung's disease in a patient with hydrocephalus, vesico-ureteral reflux and a balanced translocation t(3;17)(p12;q11).

Hirschsprung's disease (HSCR), a congenital complex disorder of intestinal innervation, is often associated with other inherited syndromes. Identifying genes involved in syndromic HSCR cases will not only help understanding the specific underlying diseases, but it will also give an insight into the development of the most frequent isolated HSCR. The association between hydrocephalus and HSCR is not surprising as a large number of patients have been reported to show the same clinical association, most of them showing mutations in the L1CAM gene, encoding a neural adhesion molecule often involved in isolated X-linked hydrocephalus. L1 defects are believed to be necessary but not sufficient for the occurrence of the intestinal phenotype in syndromic cases. In this paper, we have carried out the molecular characterization of a patient affected with Hirschsprung's disease and X-linked hydrocephalus, with a de novo reciprocal balanced translocation t(3;17)(p12;q21). In particular, we have taken advantage of this chromosomal defect to gain access to the predisposing background possibly leading to Hirschsprung's disease. Detailed analysis of the RET and L1CAM genes, and molecular characterization of MYO18A and TIAF1, the genes involved in the balanced translocation, allowed us to identify, besides the L1 mutation c.2265delC, different additional factors related to RET-dependent and -independent pathways which may have contributed to the genesis of enteric phenotype in the present patient.