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A 3-month-old boy was admitted from his pediatrician's office for failure to thrive and vomiting. On admission, he weighed barely more than his birth weight and was cachectic with muscle wasting. His abdomen was grossly distended but soft and nontender. A trial of nasogastric feeds resulted in a worsening of his clinical status. He was transferred to the ICU, and diagnostic imaging was concerning for a distal bowel obstruction. Surgical pathology revealed a surprising diagnosis, which is described in detail in the following case., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2017 by the American Academy of Pediatrics.)

Purpose . Congenital heart disease (CHD) is reported to be associated with Hirschsprung disease (HD). The aim was to evaluate any differences between children with HD with and without CHD, respectively, with regard to patient characteristics, medical care, and patient reported bowel function. Method . This is a retrospective chart study and a cross-sectional long-term follow-up of patients older than 4 years old, including all children with HD operated on with transanal endorectal pull-through (TERPT) at a tertiary center of pediatric surgery. Information about patient characteristics, diagnostics, surgery, and medical care was compiled. At long-term follow-up, bowel function was assessed by Bowel Function Score. Results . Included were 53 HD-patients, 13 with CHD and 40 without CHD. Children with CHD more commonly presented with failure to thrive; 4 (23%) compared to those without CHD (0%) ( p < 0.01). In the long-term follow-up, including 32 patients (6 with CHD), constipation was more commonly reported by children with CHD 5 (83%) than by children without CHD 4 (27%) ( p = 0.01). No differences were shown in the other parameters such as fecal control and incontinence. Conclusion . HD-patients with CHD more commonly presented with failure to thrive and more frequently reported constipation than HD-patients without CHD. The findings indicate that HD-patients with CHD might need special consideration in their initial care and long-term follow-up.

Mandibular hypoplasia may present in isolation or in the context of glossoptosis and a U-shaped, incomplete cleft palate. This latter triad is referred to as Pierre Robin sequence. Deleterious effects of micrognathia that may present during infancy are due primarily to glossoptosis or posterior displacement of the tongue. This tongue base prolapse may cause varying degrees of upper airway obstruction. A surgical option for management of tongue base airway obstruction secondary to mandibular hypoplasia is neonatal mandibular distraction osteogenesis. Herein, the author seeks to outline the benefits and limitations of early mandibular distraction osteogenesis as a way of managing airway obstruction and feeding difficulty in newborns with micrognathia. A description of the author's operative technique as well as potential complications and pitfalls will also be discussed., (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)

Gastrostomy, gastrojejunostomy and anti-reflux surgery in infants and children who are chronically ventilator dependent are associated with significant risk of morbidity and mortality. We report outcomes of 22 high risk children who underwent these procedures at our centre. Pre-operative investigations included: overnight oxygen and carbon dioxide monitoring and subsequent optimisation of ventilatory support, echocardiography, video fluoroscopy, and assessment of gastroesophageal reflux. We carried out 24 procedures under general anaesthesia. Twenty-one children used ventilatory support pre-operatively. Median age of first surgical procedure was 18 months (range 3-180). Supplementary feeding was commenced in 20 children prior to procedure, median age 9 months (1-31). Median PICU length of stay was 1 (1-8) days. No children died in the post-operative period. Extubation was possible within 24h in 87% of cases. Complications included; atelectasis (n=2), ileus (n=2), abdominal distension (n=4) and loose stools (n=1). We conclude that, in this high risk cohort of ventilator dependent children with predominantly neuromuscular disorders, with careful assessment, operative intervention can be carried out under general anaesthesia, with the child being extubated early back onto their routine ventilatory support and aggressive airway clearance. Additionally this protocol can minimise post-operative complications and is associated with a good outcome in the majority., (Copyright © 2013 Elsevier B.V. All rights reserved.)

Unlabelled: We report an infant suffering from Pierre Robin sequence complicated by gastro-oesophageal reflux and failure to thrive, which were resistant to conservative therapy and a hemifundoplication. Gastro-oesophageal reflux was accompanied by supraventricular tachycardia, treated with propafenone. Tachycardia may be present in Pierre Robin sequence as a consequence of cardiac parasympathetic imbalance. The patient recovered completely from the gastro-oesophageal reflux and tachycardia after redo-fundoplication (Nissen) and a hiatoplasty were performed., Conclusion: This case shows that a thorough search for gastro-oesophageal reflux is indicated in each case of Pierre Robin sequence with failure to thrive.

Atrial septal defects are usually asymptomatic, and are closed surgically or by a catheter implanted device in preschool age children. Rarely, they may cause symptoms in infancy, and management at this age is debated. We report our experience of six infants who underwent surgical closure, with variable outcomes. Five of these had significant extracardiac pathology. Our data suggest the need to exclude other causes of symptoms, both cardiac and non-cardiac. The question should be asked as to whether surgery is of benefit in these children, and particularly whether they may have underlying primary pulmonary vascular disease.

Objective: To document catch-up growth in children in the first 18 months after gastrostomy surgery and characterize how weight and length growth differ according to medical and nutritional risks., Design: Repeated measures study to evaluate weight and linear growth in gastrostomy-fed children., Subjects/setting: Seventy-five subject met the selection criteria; gastrostomy placement anytime from birth to age 6.5 years, diagnosis of failure to thrive before gastrostomy. surgery, absence of nonmedical barriers to adequate nutrition. Children were seen in specialty outpatient clinics., Outcome Measures: Three measurements of weight and length: at the time of surgery and 12 and 18 months after surgery., Statistical Analyses: Paired t tests of z scores were used to determine catch-up growth. Analysis of variance used variables (age of placement, ambulatory status, prematurity, mode of feeding) to determine statistically significant predictors of growth., Results: After gastrostomy surgery, catch-up growth was observed in height and weight for children regardless of prematurity or age at the time of gastrostomy placement. Ambulatory children did not achieve catch-up growth, but nonambulatory children did. At 18 months after surgery, catch-up growth occurred in children whose sole source of nutrition was through occurred in children whose sole source of nutrition was through the gastrostomy, as well as in those who were able to receive nutrition by mouth. Children with a diagnosis of cerebral palsy experienced better growth than children with other diagnoses. CONCLUSION/APPLICATION: Failure to thrive in children up to age 6.6 years can be corrected when adequate nutrition is provided. Benefits of gastrostomy surgery observed in catch-up growth reinforce the importance of medical nutrition therapy.

Occult pulmonary artery is an uncommon cardiovascular defect. Associated symptoms include recurrent pulmonary infections and congestive heart failure. The authors describe a one-year-old boy initially diagnosed as having broncho-pulmonary dysplasia who developed severe failure to thrive, recurrent pneumonias, and pulmonary hypertension. The presence of an occult right pulmonary artery was suspected by lung perfusion scan and diagnosed by cardiac catheterization and angiography. After surgical repair, his clinical course improved and his growth and development were normal. This case demonstrates the importance of including occult pulmonary artery in the differential diagnosis of infants with failure to thrive associated with recurrent pulmonary infection.