Gastrostomy Button Placement in Infants With Cyanotic Versus Acyanotic Congenital Heart Disease.

BACKGROUND: Infants with congenital heart disease (CHD) may exhibit increased metabolic demands, and many will undergo placement of a gastrostomy to achieve adequate nutritional intake. There is a paucity of data, however, comparing the operative risks and overall complications of gastrostomy placement in cyanotic versus acyanotic infants with CHD. We hypothesized that patients with cyanotic CHD would have a higher rate of gastrostomy-associated complications than infants with acyanotic CHD. METHODS: We retrospectively reviewed patients who underwent gastrostomy button placement after cardiac surgery for CHD between 2013 and 2018. Patients were stratified into cyanotic CHD and acyanotic CHD cohorts. Patient data were extracted from the Society of Thoracic Surgeons database and merged with clinical data related to gastrostomy placement and complications from chart review. Unadjusted analyses were used to find covariates associated with cyanotic CHD and acyanotic CHD, using a t-test or Wilcoxon rank-sum test for continuous data, depending on normalcy, and χ2 or Fisher's exact tests for categorical data depending on the distribution. RESULTS: There were 257 infants with CHD who underwent gastrostomy placement during the study period, of which 86 had cyanotic CHD. There were no significant differences in baseline weight or preoperative albumin levels between the two groups. Patients with cyanotic CHD had a lower incidence of comorbid syndromes (P = 0.0001), higher Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery scores (P < 0.0001), and higher postoperative mortality rate (P = 0.0189). There was a higher rate of granulation tissue formation in patients with acyanotic CHD (48.5% versus 22.1%, P < 0.0001). There were no differences in other gastrostomy button-related complications, including leakage, wound infection, or dislodgement. CONCLUSIONS: Patients with acyanotic CHD demonstrated a higher incidence of granulation tissue. We found no difference in gastrostomy-specific complication rates between the two groups, with the notable exception of granulation tissue formation. Based on this study, the diagnosis of cyanotic CHD does not increase the risk of gastrostomy-related complications.

The Nissen Unwrapped: Predictors and Outcomes of Nissen Fundoplication in Congenital Heart Disease.

BACKGROUND: Infants with congenital heart disease (CHD) often require the placement of a gastrostomy button to ensure proper nutrition. Some also require a Nissen fundoplication (NF) to further improve nutrition capabilities in the setting of reflux, however, the clinical and diagnostic imaging characteristics that support NF are variable. The aims of this study were as follows: (1) identify the factors associated with NF in patients with CHD and (2) determine the incidence of NF complications in patients with CHD. METHODS: All patients with CHD who underwent cardiac repair and subsequent creation of a gastrostomy at a single institution between 6/1/2013 and 9/1/2018 were included. We then identified which patients underwent NF. RESULTS: Two-hundred fifty-seven CHD patients who had a gastrostomy button placed after CHD repair, with 17% undergoing a simultaneous NF or an NF at a later time. The presence of acyanotic heart disease, neurologic comorbidities, and vocal cord dysfunction was not univariately associated with a higher likelihood of NF. On multivariable model, only prematurity was significantly associated with NF (P = 0.022). Abnormal findings on imaging studies (upper gastrointestinal series, gastric emptying studies, motility studies, upper endoscopies, swallow studies, and pH probe studies) were not associated with an NF (all P's > 0.05). The overall complication rate was 23%. CONCLUSIONS: Prematurity was the only factor associated with an NF. Surprisingly, cyanotic heart disease, neurologic comorbidities, age at first cardiac surgery, and vocal cord dysfunction were not associated with an NF. We identified an area for quality improvement at our institution given the lack of standardized work-up for the NF in this high-risk population.

Unsedated transnasal esophagoscopy for monitoring therapy in pediatric eosinophilic esophagitis.

BACKGROUND AND AIMS: Unsedated transnasal endoscopy (TNE) is safer and less costly than sedated EGD. The aim of this study was to evaluate the performance of TNE with biopsies in monitoring the esophageal mucosa of pediatric patients with eosinophilic esophagitis. METHODS: Patients between 8 and 17 years of age with eosinophilic esophagitis and their parents were enrolled. Unsedated TNE was performed. A 2.8-mm (1.2-mm channel) or a 4-mm flexible bronchoscope (2-mm channel) was used, and esophageal biopsy specimens were obtained. Biopsy specimen analysis, duration, adverse events, and billing charges of TNE were assessed. Immediately after TNE and a minimum of 2 weeks later, a modified Group Health Association of America 9 survey and a preference questionnaire were completed, respectively. RESULTS: Twenty-one of 22 enrolled patients underwent TNE. TNE was performed with no serious adverse events. Histopathological analysis revealed 0 eosinophils per high-power field (n = 12), fewer than 15 eosinophils per high-power field (n = 4), and more than 15 eosinophils per high-power field (n = 5). The total epithelial surface area of mucosal biopsy samples from either TNE Forceps (1.2 mm or 2 mm biopsy channel forceps) compared with those obtained during the subject's previous EGD by using standard endoscopic forceps was not statistically different (P = .308 [1.2 mm]/P = .492 [2 mm]). All parents and 76.2% of subjects would undergo the TNE again. TNE was preferred over EGD by 85.7% of parents and 52.4% of subjects. The modified Group Health Association of America 9 survey revealed a high degree of satisfaction (average, 43.19 ± 2.6; maximum score, 45). Charges associated with TNE were 60.1% lower than for previous EGDs. CONCLUSIONS: Unsedated TNE is an effective, lower-cost procedure for monitoring the esophageal mucosa of children with eosinophilic esophagitis.

Failure of resolution of portal fibrosis during omega-3 fatty acid lipid emulsion therapy in two patients with irreversible intestinal failure.

Parenteral omega-3 fatty acid lipid emulsions have been evaluated for their potential role in reversing intestinal failure-associated liver disease. We report our experience using Omegaven in 2 patients with irreversible intestinal failure and intestinal failure-associated liver disease. Despite biochemical and histologic improvement in cholestasis, both patients had persisting, significant portal fibrosis on liver biopsy.

Hepatic veno-occlusive disease and human herpes virus 7 infection in primary agammaglobulinemia.

Hepatobiliary disease has been described in Bruton's x-linked agammaglobulinemia; however, veno-occlusive disease has not been reported in this setting. We report a case of end-stage liver disease in a patient with x-linked agammaglobulinemia who was found to have VOD and evidence of human herpes virus 7 infection in the explanted liver after transplantation.

First successful antegrade single-balloon enteroscopy in a 3-year-old with occult GI bleeding.

BACKGROUND: Balloon enteroscopy is an emerging technique to allow access to the small intestine for both diagnostic and therapeutic purposes. To date, there have been few published data documenting the safety and efficacy of balloon enteroscopy in small children. OBJECTIVE: To describe our experience with single-balloon enteroscopy (SBE) in a 37-month-old toddler with occult GI bleeding. DESIGN: A single case report. SETTING: A free-standing, academic children's hospital in Denver, Colorado. PATIENT: The patient was a 37-month-old, 13.5-kg toddler with persistent heme-positive stools, severe microcytic anemia, and hypoalbuminemia. Previous workup was significant for eosinophilic inflammation in the antrum and a video capsule study showing erythematous lesions in the small bowel. INTERVENTION: An antegrade SBE was performed with the child under general endotracheal anesthesia, with biopsy specimens obtained from identified lesions in the jejunum and ileum. MAIN OUTCOME MEASUREMENTS: Complications and successful treatment of symptoms were the primary endpoints. RESULTS: The procedure was performed successfully in 85 minutes, passing an estimated 200 cm beyond the pylorus, without complications. Identification of the lesions as consistent with eosinophilic enteropathy led to successful treatment with an elimination diet and corticosteroids. LIMITATIONS: The primary limitation of this study is that it is a single case report. Therefore, it is difficult to make a generalized statement regarding the safety and efficacy of balloon enteroscopy in toddlers of this size. CONCLUSIONS: Antegrade SBE can be a well-tolerated and effective procedure to evaluate occult GI bleeding in children as young as 3 years of age. Further study is needed to better establish safety parameters for balloon enteroscopy in small pediatric patients.

Resistance of young rat hepatic mitochondria to bile acid-induced permeability transition: potential role of alpha-tocopherol.

Retention of bile acids within the liver is a primary factor in the pathogenesis of cholestatic liver disorders, which are more common in human infants. The objective of this study was to evaluate developmental changes in mitochondrial factors involved in bile acid-induced hepatocyte injury. Hepatic mitochondria from adult rats (aged 9 wk) underwent a mitochondrial permeability transition (MPT) and release of cytochrome c upon exposure to glycochenodeoxycholic acid. In contrast, mitochondria from young rats (age 6-36 d) were resistant to MPT induction and cytochrome c release. Neither mitochondrial levels of MPT-associated proteins (voltage-dependent anion channel, cyclophilin D, or adenine nucleotide translocase), Bcl-2 family proteins, nor antioxidant enzymes explained this resistance. Mitochondria from young rats contained 2- to 3-fold higher alpha-tocopherol (alpha-TH). In vivo alpha-TH enrichment of adult hepatic mitochondria increased their MPT resistance. Tetra-linoleoyl cardiolipin (TL-CL), the primary molecular species of CL, was reduced in mitochondria of the young rat; however, enrichment with CL and TL-CL only modestly increased their MPT susceptibility. In conclusion, we observed an unexpected resistance in young rats to bile acid induction of mitochondrial cell death pathways, which may be related to developmental differences in membrane composition.

Clinical assessment of the child with intestinal failure.

The management of the child with intestinal failure is complex, and it is developing into a multispecialty field of its own led by expert teams of both transplant and nontransplant surgeons, gastroenterologists, and dieticians. Patients are at risk for medical, surgical, and nutritional complications that should be anticipated so that they can be prevented or managed appropriately. Catheter associated infections and intestinal failure associated liver diseases are important complications that impact the likelihood of bowel adaptation and long-term survival. The clinical assessment of a pediatric intestinal failure patient should include evaluation of the child within the context of recognized prognostic factors.

Laparoscopic suture rectopexy for full-thickness anorectal prolapse in children: an effective outpatient procedure.

BACKGROUND/PURPOSE: Our approach to full-thickness anorectal prolapse has transitioned to laparoscopic suture rectopexy (LSRP). The purpose of this study was to describe the indications, technique, and postoperative outcomes for LSRP. METHODS: Rectopexy was performed using 3 or 4 laparoscopic ports. Redundant rectum was retracted from the pelvis, and the posterior rectal wall was secured to the sacral promontory using 3 permanent sutures. RESULTS: Nineteen children (7 girls) underwent LSRP from March 2003 to January 2008. Mean age was 6.2 ± 3.6 years. Three patients had prior perineal operations: 2 sacrococcygeal teratoma resections and 1 pull-through for Hirschsprung disease. One patient had cystic fibrosis, and another had Prader-Willi syndrome. The remaining children had either chronic constipation or idiopathic prolapse. All patients were treated preoperatively with laxatives. Two patients received antegrade continent enemas. Length of stay was 1 ± 0.8 days, with only the first 5 patients admitted to the hospital. The patient with Prader-Willi syndrome had a full-thickness recurrence (5%) owing to obsessive-compulsive behavior. Partial mucosal prolapse occurred in 2 patients. There were no other complications. CONCLUSIONS: Laparoscopic suture rectopexy is an effective minimally invasive method to treat full-thickness rectal prolapse in children from various etiologies. It can be performed as an outpatient procedure with minimal morbidity and low recurrence rate (5%).

Subcutaneous vitamin E ameliorates liver injury in an in vivo model of steatocholestasis.

UNLABELLED: Several genetic metabolic liver diseases share the pathological features of combined steatosis and cholestasis, or steatocholestasis. The aims of this study were to develop and characterize an in vivo model for steatocholestasis and to evaluate the effects of an antioxidant treatment on liver injury, oxidative stress, and mitochondrial perturbations in this model. Obese and lean Zucker rats received intravenous (IV) injections of glycochenodeoxycholic acid (GCDC) and were killed 4 hours later. Liver enzymes were measured; the liver histology was assessed, and hepatic mitochondria were analyzed for mitochondrial lipid peroxidation. In separate experiments, rats received daily injections of subcutaneous (SQ) vitamin E before GCDC infusion. Bile acid-induced injury (serum AST and ALT and liver histology) was more severe in the obese rats than in the lean rats, characterized predominantly by extensive cell necrosis with minimal evidence of apoptosis. SQ vitamin E provided significant protection against IV GCDC-induced hepatic injury, in vitro GCDC-induced permeability transition, and cytochrome C and apoptosis-inducing factor release from isolated mitochondria. CONCLUSION: Steatosis sensitizes the liver to bile acid-induced necrotic hepatocyte injury, which is responsive to vitamin E therapy.