Not just an upset stomach: gastric perforation in a patient with anorexia nervosa.

Gastric perforation in patients with anorexia nervosa is a rare entity associated with high morbidity and mortality [Norris in Int J Eat Disord 49:216-237, 2016]. In cases reported in the literature, the perforation was often preceded by a binge episode, and the subsequent clinical presentation was rapid and acutely deteriorating with a fatality rate as high as 80% [Norris in Int J Eat Disord 49:216-237, 2016, Pitre in J Med Case Rep 15:61, 2021]. We present a case of gastric perforation in the context of restrictive anorexia nervosa unique both for the absence of a premorbid binge episode as well as delayed clinical manifestations of medical distress, leading to abdominal compartment syndrome.Level IV Evidence obtained from multiple time series analysis such as case studies.

Encephalocraniocutaneous Lipomatosis.

A 5-year-old boy presented with worsening headaches for 3 months. On examination, he was found to have a hairless fatty tissue nevus of the scalp (nevus psiloliparus), subcutaneous soft tissue masses on the right side of his face, neck, mandible and right buttock and epibulbar dermoid of the right eye (choristoma) (). Magnetic resonance imaging revealed a large suprasellar mass, which was debulked and found to be a pilocytic astrocytoma. Testing was not performed for the BRAF/KIAA1549 fusion or BRAFV600E mutation. Seven years later, he was started on adjuvant chemotherapy for gradual tumor progression. Over the ensuing 3 years, he had further disease progression despite treatment with 3 frontline chemotherapy regimens: vinblastine, carboplatin/vincristine, and irinotecan/bevacizumab. Targeted sequencing of tissue from the right gluteal mass, revealed a mosaic activating FGFR1 c.1966A>G (p.Lys656Glu) mutation, absent in normal left gluteal tissue, confirming the diagnosis of encephalocraniocutaneous lipomatosis (ECCL), belonging to the family of RASopathies (including neurofibromatosis type I, Noonan syndrome, Costello syndrome), with constitutive activation of the mitogen-activated protein kinase (MAPK) pathway, and an increased risk of developing neoplasms. He was started on trametinib, a MEK inhibitor, off-label, targeting the MAPK pathway downstream from FGFR1, with stable tumor size at last follow-up, after 6 months on therapy.

Pancreaticoduodenectomy for choledochal cyst.

BACKGROUND/AIMS: Choledochal cyst (CDC) is a rare congenital cystic dilatation of the biliary system that can affect children and adults. Standard treatment for CDC includes surgical excision of the cystic bile duct with restoration of bilioenteric continuity by Roux-en-Y hepaticojejunostomy. In rare situations, however, surgical excision of a CDC may require pancreaticoduodenectomy (PD). The goal of this study was to identify instances where a PD may be deemed necessary. METHODOLOGY: We retrospectively reviewed the medical records of all patients treated for CDC at our institution from January 1988 through December 2011. From this cohort, patients who underwent PD were reviewed in detail. Data on preoperative parameters, operative details, cyst type, pathologic diagnosis, and postoperative course were collected. RESULTS: During the 24-year period, 59 patients with CDC were identified and four (7%) underwent formal PD. Final pathology revealed that none of the resected specimens harbored malignancy and all patients are well at follow-up. CONCLUSION: While the standard surgical care for CDC remains complete cyst excision with Roux-en-Y hepaticojejunostomy, there may be particular instances where PD appears more appropriate. While the need for PD in cases of CDC is rare, it is important to consider this possibility when contemplating surgery for CDC.

Umbilical hernia causing ventriculoperitoneal shunt dysfunction: illustrative cases.

BACKGROUND: When ventriculoperitoneal (VP) shunts and umbilical hernias coexist in the same patient, unique complications can occur. Typically, these are readily identified problems such as cerebrospinal fluid (CSF) fistulas or entrapment of the peritoneal catheter in the hernia. The authors present cases of two children whose VP shunt dysfunction resolved after repair of their umbilical hernias. OBSERVATIONS: The authors present two cases of infant patients with shunted hydrocephalus and umbilical hernias. In both cases, the patients presented with distal shunt malfunctions not due to infection. Their shunt function improved once the umbilical hernia was repaired by pediatric surgery. Neither has required shunt revision since umbilical hernia repair. LESSONS: Although there are case reports of VP shunts eroding through the umbilicus, developing CSF fistulas, or becoming trapped inside umbilical hernias, there is no case of VP shunt dysfunction caused by just the presence of an umbilical hernia. The authors suspect that the catheter may intermittently enter and exit the hernia. This may cause intermittent obstruction of the distal catheter, or inflammation in the hernia may occur that limits CSF absorption.

The effects of the addition of a pediatric surgery fellow on the operative experience of the general surgery resident.

Adding fellows to surgical departments with residency programs can affect resident education. Our specific aim was to evaluate the effect of adding a pediatric surgery (PS) fellow on the number of index PS cases logged by the general surgery (GS) residents. At a single institution with both PS and GS programs, we examined the number of logged cases for the fellows and residents over 10 years [5 years before (Time 1) and 5 years after (Time 2) the addition of a PS fellow]. Additionally, the procedure related relative value units (RVUs) recorded by the faculty were evaluated. The fellows averaged 752 and 703 cases during Times 1 and 2, respectively, decreasing by 49 (P = 0.2303). The residents averaged 172 and 161 cases annually during Time 1 and Time 2, respectively, decreasing by 11 (P = 0.7340). The total number of procedure related RVUs was 4627 and 6000 during Times 1 and 2, respectively. The number of cases logged by the PS fellows and GS residents decreased after the addition of a PS fellow; however, the decrease was not significant. Programs can reasonably add an additional PS fellow, but care should be taken especially in programs that are otherwise static in size.

A practical algorithm for accurate diagnosis and treatment of perinatally identified biliary ductal dilation: three cases that underscore the importance of an individualised approach.

Choledochal cysts (CDCs) identified during the prenatal or immediate postnatal period are unusual with a majority being identified in older children and adults. Although authors advocate surgical resection of these presumed CDCs with reconstruction, there has been no agreement on the optimal timing of such an intervention when the initial diagnosis of CDC is made prenatally or during the newborn period. We present the cases of three children with biliary ductal dilation who were presumed to have CDC by ultrasound and/or magnetic resonance imaging during the perinatal period and who ultimately required different approaches to treatment. The eventual findings in these cases support a period of close postnatal observation followed by carefully planned individualised therapy.

Button-loop feeding jejunostomy.

INTRODUCTION: Post-pyloric feeding via a surgical jejunostomy allows for enteral nutrition in patients that cannot receive oral or gastric feeding. Regardless of the technique used to create a jejunostomy, complications such as tube dislodgement, jejunostomy closure, or bowel obstruction can occur. SURGICAL TECHNIQUE: We present a simple and efficient jejunostomy technique that does not require a sewn anastomosis and employs an easily exchangeable feeding button.

Percutaneous distal perfusion of the lower extremity after femoral cannulation for venoarterial extracorporeal membrane oxygenation in a small child.

Femoral cannulation in pediatric patients requiring extracorporeal membrane oxygenation (ECMO) is commonly associated with distal limb ischemia. Authors have previously reported successful lower limb perfusion using various open techniques to cannulate a distal lower extremity artery at the time of initial ECMO cannulation. These procedures include open femoral artery antegrade cannulation and distal posterior tibial artery retrograde cannulation in older children and adults. Such approaches require ample vessel diameters to accommodate an arteriotomy and catheter insertion and, therefore, are of limited use in smaller children. We hypothesized that after femoral artery cannulation for ECMO, a percutaneous technique of distal limb perfusion might offer unique advantages when treating lower extremity ischemia in small pediatric patients. We report a technique for percutaneous antegrade cannulation in a 4-year-old patient shortly after her primary cannulation for venoarterial ECMO via the femoral artery.

Multivariate model for predicting recurrence in congenital diaphragmatic hernia.

PURPOSE: Risk factors that predispose children with congenital diaphragmatic hernia (CDH) to recurrence remain poorly defined. We report a large series of recurrent CDH and ask whether prenatal patient factors or postnatal treatment variables better predict recurrence. METHODS: Two hundred thirty-eight neonates with unilateral CDH underwent repair from 1990 to 2006. Data were assessed by chi(2) and Mann-Whitney U tests. Multivariate regression identified independent predictors of recurrence. Statistical significance was set at P < .05. RESULTS: We identified 24 recurrences (10%). Median time from repair to recurrence diagnosis was 4.9 months. Patients with recurrence were older (P = .02) and more often required abdominal wall patches at initial repair (P = .01) compared to nonrecurrence patients. Postoperative length of stay (LOS) after initial repair (P < .01) and morbidity (P = .01) were greater in recurrence patients. Use of diaphragm patch at initial repair was greater in patients with recurrence but only approached statistical significance (P = .05). Only 2 variables independently predicted recurrence by multivariate regression as follows: abdominal (not diaphragm) wall patch during initial repair (odds ratio [OR] 3.50; P = .04) and postoperative LOS (OR, 1.012; P = .01). CONCLUSION: Neonates at risk for CDH recurrence are better identified by postnatal treatment variables than by prenatal patient factors. Although age at repair and diaphragm patch use are greater in recurrence patients, the only factors to independently predict recurrence were postoperative LOS and abdominal wall patch use. These data can help optimize follow-up regimens.

Gastric tube-pericardial fistula: a remote complication of esophageal replacement for long gap esophageal atresia.

Several short and long-term complications of esophageal replacement have been described in the literature. We report the case of a gastric tube-pericardial fistula occurring 20 years after initial repair of long gap esophageal atresia with a reversed gastric tube.