Complications of Percutaneous and Surgical Gastrostomy Placements in Children: a Single-Centre Series.

Gastrostomy placement is a standard procedure for children requiring enteral feeding for more than 3-6 weeks. Various techniques have been described (percutaneous endoscopic, laparoscopy, and laparotomy), and many complications have been reported. In our center, gastrostomy placement is performed either percutaneously by pediatric gastroenterologists, by laparoscopy/laparotomy by the visceral surgery team, or jointly, that is laparoscopic-assisted percutaneous endoscopic gastrostomy. This study aims to report all complications and identify risk factors and ways to prevent them. Methods: This is a monocentric retrospective study including children younger than 18 years who underwent gastrostomy placement (percutaneous or surgical) between January 2012 and December 2020. Complications that occurred up to 1 year after placement were collected and classified according to their time of onset, degree of severity, and management. A univariate analysis was conducted to compare the groups and the occurrence of complications. Results: We established a cohort of 124 children. Sixty-three (50.8%) presented a concomitant neurological disease. Fifty-nine patients (47.6%) underwent endoscopic placement, 59 (47.6%) surgical placement, and 6 (4.8%) laparoscopic-assisted percutaneous endoscopic gastrostomy. Two hundred and two complications were described, including 29 (14.4%) major and 173 (85.6%) minor. Abdominal wall abscess and cellulitis were reported 13 times. Patients who underwent surgical placement presented more complications (major and minor combined) with a statistically significant difference compared with the endoscopic technique. Patients with a concomitant neurological disease had significantly more early complications in the percutaneous group. Patients with malnutrition had significantly more major complications requiring endoscopic or surgical management. Conclusion: This study highlights a significant number of major complications or complications requiring additional management under general anesthesia. Children with a concomitant neurological disease or malnutrition are at greater risk of severe and early complications. Infections remain a frequent complication, and prevention strategies should be reviewed.

Digestive involvement in a severe form of Snyder-Robinson syndrome: Possible expansion of the phenotype.

Snyder-Robinson syndrome (OMIM #309583) is a rare X-linked condition, caused by mutation in the SMS gene (MIM *300105), characterized by a wide spectrum of clinical signs including developmental delay, epilepsy, asthenic habitus, dysmorphism, osteopenia, and renal or genital anomalies. Here we describe two maternal half-brothers who both presented with severe neurodevelopmental delay, seizures, hearing loss, facial dysmorphism, renal and ophthalmologic anomalies, failure to thrive and premature death. A novel p.(Gly203Asp) variant was found at the hemizygous state in the two boys, and an elevated Spermidine/Spermine ratio confirmed the diagnosis of Snyder-Robinson syndrome. One of the brothers presented with gastrointestinal symptoms, with jejunal stenosis, enteral feeding intolerance, failure to thrive due to a dysfunctional gastrointestinal system, cholestasis and exocrine pancreatic insufficiency. Although more studies will be needed to understand its mechanisms, this observation lends further support to the possibility of severe digestive involvement in Snyder Robinson syndrome.

Pyloric Mucosal Diaphragm Associated with Hypertrophic Pyloric Stenosis: An Unusual Combination in a Male Neonate.

We describe herein the case of a 3-week-old baby with persistent nonbilious vomiting, due to a hypertrophic pyloric stenosis (HPS) associated with a congenital pyloric mucosal diaphragm. So far, an association between the two conditions has not been described. The diagnosis of a pyloric mucosal diaphragm was delayed because of its cooccurrence with HPS.

Long-Term Outcomes and Quality of Life in Patients after Soave Pull-Through Operation for Hirschsprung's Disease: An Observational Retrospective Study.

INTRODUCTION: Patients after pull-through operation for Hirschsprung's disease (HD) are at high risk of defecation disorders. This study aimed at investigating their long-term outcomes and quality of life (QoL) in comparison with controls. PATIENTS AND METHODS: Patients older than 5 years operated on for HD were interviewed to complete detailed questionnaires on bowel function. Patients without neurologic impairment were enrolled in a QoL survey to compare with controls matched for sex and age and selected randomly from the general population using sampling set in a ratio of four controls to one case of HD. RESULTS: In total, 53 operated patients were enrolled. Mean age of the patients was 16 ± 8 years, with 68% boys. Rectosigmoid aganglionosis was the most seen form of HD in 38 (72%) cases. Open Soave was performed in 40 (75.5%) cases, and minimally invasive surgery Soave (MIS Soave) in 13 (24.5%) cases. At investigation, prevalence of fecal incontinence and constipation were 22.6 and 13.2%, respectively. Regarding QoL survey, 45 patients and 180 controls were enrolled, excluding 8 patients with neurologic impairment. Thirty-seven (82.2%) patients were classified as having a good QoL (score ≥ 9 points); whereas six had a fair QoL (5-8 points) and two had a poor QoL (< 5 points). QoL score in the cases and the controls were 10.2 ± 2.5 and 11.9 ± 0.4 points, respectively. Long aganglionosis form of HD was significantly associated with a low QoL (score < 8 points), adjusted odds ratio = 9, 95% confidence interval [1.3; 64.1] (p < 0.05). In subscales analyses, the prevalence of each dimension including fecal continence, school absenteeism, unhappiness or anxiety, food restriction, and peer rejection was significantly higher in operated patients than in controls (p <0.001). CONCLUSION: Although the QoL of patients operated on for HD in general was with good outcomes, fecal incontinence and constipation still are problematic issues and challenges in a high percentage of patients. Therefore, a long-term and multidisciplinary follow-up is essentially required for these patients.

Thoracoscopic thoracic duct sealing with LigaSure in two children with refractory postoperative chylothorax.

PURPOSE: Chylothorax is an uncommon complication of cardiac surgery. We report two cases of the thoracoscopic management of persistent postoperative chylothorax by thoracic duct thermofusion and section with the LigaSure device. MATERIALS AND METHODS: A 6-year-old boy and a 3-year-old girl developed persistent chylothorax following an aortic coarctation repair and a Fontan procedure, respectively. The former presented with a left chylothorax due to direct surgical injury, and the latter, a right chylothorax due to indirect lymphatic rupture secondary to increased venous pressure. In both patients, voluminous chylous drainage persisted for more than 3 weeks despite maximal medical treatment. Ultimately, a right thoracoscopic coagulation and section of the thoracic duct with the 5-mm LigaSure device was performed. The LigaSure is a computer-controlled bipolar diathermy system designed to seal blood vessels up to 7 mm in diameter, with no specific recommendations regarding the lymphatics. RESULTS: The lymph leakage ceased within 2 days in the first patient and immediately in the second one, with no recurrence after oral intake resumption on day 5. Both patients are free of recurrence at 1 year. CONCLUSION: Thoracoscopic coagulation and section of the thoracic duct above the diaphragm with the LigaSure device appears to be a simple, effective, and safe therapeutic option for treatment of refractory postoperative chylothorax in children.

Laparoscopic Repair for Perforated Peptic Ulcer in Children.

Introduction A perforated peptic ulcer (PPU) is a rare but major complication of gastroduodenal peptic ulcer disease. Literature is scarce on this subject in the pediatric population and most articles describe a surgical treatment by laparotomy. We aim to review all our cases of pediatric PPU treated over the past 16 years and compare these to literature to deduce potential benefits and disadvantages regarding laparoscopic treatment of PPU in children. Materials and Methods A retrospective study of all cases of PPU treated at the Lenval Hospital in Nice (France) and the Queen Fabiola University Hospital for Children in Brussels (Belgium) between 1998 and 2015 was performed. Results A total of five children were treated for PPU (2 females). The average age was 11 years (range, 3-17). All of them were surgically treated with laparoscopic simple suture of the perforation and placement of an omental patch. There were no mortalities, no conversions, and no extra-abdominal complications or wound dehiscences. Mean operating time was 78.6 minutes (range, 70-115 minutes). Mean duration of intravenous treatment was 6 days (range, 4-12 days). One reintervention was performed for abdominal infection. In one patient, an abdominal drain was left in place for 2 days. The mean time before refeeding was 3.4 days (range, 3-4 days) and mean length of stay was 12 days (range, 7-30 days). Conclusion Laparoscopic repair is safe and feasible for PPU and should be the gold standard for treatment of PPU in children.

Laparoscopic partial splenectomy using radiofrequency ablation for nonparasitic splenic cysts in two children.

Nonparasitic splenic cysts are uncommon lesions, for which partial splenectomy offers a definitive treatment while preserving splenic function. We report the use of radiofrequency energy for laparoscopic partial splenectomy to treat two children with nonparasitic splenic cysts by creating a 1-cm thick coagulated parenchyma resection plane, allowing bloodless splenic transection. This approach spares the need for blood transfusions, reduces hospital stay, and offers great promise for partial splenectomy in children.

Characteristics and management of congenital esophageal stenosis: findings from a multicenter study.

BACKGROUND: Congenital esophageal stenosis (CES) is a rare condition frequently associated with esophageal atresia (EA). There are limited data from small series about the presentation, treatment, and outcomes of CES. METHODS: Medical records of all patients with CES included in the French Network on Esophageal Malformations and Congenital Diseases were reviewed retrospectively with regard to diagnosis, treatment, and outcome. RESULTS: Over 18 years, 61 patients (30 boys) had CES, and 29 (47%) of these patients also had EA. The mean age at diagnosis was 24 months (1 day to 14 years) and was younger in patients with CES and EA than in those with isolated CES (7 vs. 126 months, p < 0.05). Twenty-one of the 61 patients with CES had no clinical symptoms: in three patients, the findings were incidental, and in 18 of the 29 patients with associated EA, CES was diagnosed at the time of surgical repair of EA or during a postoperative systematic esophageal barium study. In the 40 other patients, at diagnosis, 50% presented with dysphasia, 40% with vomiting, 50% with food impaction, and 42% with respiratory symptoms. Diagnosis of CES was confirmed by esophageal barium study (56/61) and/or esophageal endoscopy (50/61). Sixteen patients had tracheobronchial remnants (TBR), 40 had fibromuscular stenosis (FMS), and five had membrane stenosis (MS). Thirty-four patients (56%) were treated by dilation only (13/34 remained asymptomatic at follow-up); 15 patients were treated by dilation but required later surgery because of failure (4/15 remained asymptomatic at follow-up); and nine patients had a primary surgical intervention (4/9 were asymptomatic at follow-up). Dilation was complicated by esophageal perforation in two patients (3.4%). At follow-up, dysphagia remained in 36% (21/58) of patients, but the incidence did not differ between the EA and the isolated CS groups (10/29 vs. 7/32, p = 0.27). CONCLUSIONS: CS diagnosis can be delayed when associated with EA. Dilation may be effective for treating patients with FMS and MS, but surgical repair is often required for those with TBR. Our results show clearly that, regardless of the therapeutic option, dysphagia occurs frequently, and patients with CES should be followed over the long term.

The evaluation by magnetic resonance imaging of hepatic periportal fibrosis in infants with neonatal cholestasis: preliminary report.

PURPOSE: The aim of this study was to evaluate the potential role of magnetic resonance (MR) imaging cholangiography for the assessment of periportal fibrosis associated with neonatal cholestasis. METHODS: The authors have compared the findings on MR imaging cholangiography and on pathology in 10 infants evaluated because of neonatal cholestasis. The series included 3 patients with biliary atresia (BA), 3 patients with choledocal cyst, 2 with a neonatal hepatitis, and 2 with an inspissated bile syndrome. MR examinations were performed on a 0.5 Tesla magnet unit using Turbo Field Echo T1 and Turbo spin echo T2 sequences. A periportal hyposignal paralleling the portal vein branches and disappearing after Gadolinium injection was considered consistent with periportal fibrosis. The final type of hepatobiliary anomaly was established based on surgery (n = 6) or on laparoscopic cholangiogram (n = 10). The degree of periportal fibrosis was evaluated on pathology using a grading system from grade 0 (no fibrosis) to 4 (fibrosis with cirrhosis). The relationship between periportal hyposignal and fibrosis was tested using the exact chi2 test. RESULTS: MR imaging assessed correctly and more completely than ultrasound scan the morphology of the biliary tract in all 10 patients. A periportal hyposignal was present in the 3 patients with BA (2 patients displayed a grade 3 and one a grade 4 fibrosis on pathology) and in one with choledocal cyst (grade 3 fibrosis on pathology). No hyposignal was visualized in the 2 other patients with a choledocal cyst (grades 1 and 2), in the 2 patients with neonatal hepatitis (grades 1 and 2), or in the 2 patients with inspissated bile syndrome (both grade 0). A relationship between the hyposignal seen on MR and the degree of fibrosis seen on pathology was confirmed by the exact chi2 test (P =.019). CONCLUSIONS: This preliminary series confirms the potential role of MR imaging for the assessment of the morphology of the abnormal biliary tract and of the degree of periportal fibrosis. The presence of an hyposignal on an echo gradient TFE T1 sequence suggests an advanced fibrosis (grade 3 and higher).