Repair of complete atrioventricular septal defect between 2 and 3.5 kilograms: Defining the limits of safe repair.

OBJECTIVES: Repair of complete atrioventricular septal defect (cAVSD) is routinely performed at around 3 months of age with good results. However, some patients require earlier surgery due to heart failure or failure to thrive. It is uncertain whether cAVSD repair performed on patients ≤3.5 kg leads to increased mortality and reoperation on the left atrioventricular valve. METHODS: All patients who underwent cAVSD repair from 1990 to 2019 at a single institution were included in the study. Data were obtained from retrospective review of medical records and correspondence with cardiologists. RESULTS: Of 456 patients, 12.9% (59/456) weighed ≤3.5 kg at time of repair. This group was younger (P < .01) and had greater rates of heart failure (P < .01) and failure to thrive (P = .02). There was no significant difference in early mortality between the 2 groups (1.7% [1/59] vs 3.0% [12/397], P = 1.0). Survival at 20 years was 83.8% in those ≤3.5 kg, compared with 90.4% in those >3.5 kg, with no significant difference between the 2 groups (P = .68). Freedom from left atrioventricular valve reoperation at 20 years was 73.6% in those ≤3.5 kg, compared with 74.5% in those >3.5 kg, with no significant difference between the 2 groups (P = .45). CONCLUSIONS: Repair of cAVSD in children ≤3.5 kg appears to be safe, with similar overall survival and freedom from reoperation compared with those >3.5 kg. These findings add further support to an approach of early complete repair in children with severe heart failure or failure to thrive.

A 3-Month-Old With Failure to Thrive and Persistent Vomiting.

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.

Treatment and Patient Reported Outcome in Children with Hirschsprung Disease and Concomitant Congenital Heart Disease.

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.

Piezosurgery an Asset in Treatment of Pierre Robin Sequence.

Pierre Robin sequence (formerly a syndrome) is named after the French stomatologist who, in 1923 and 1934, described the problems associated with micrognathia in newborn. It comprises mandibular micrognathia, U-shaped cleft palate, and glossoptosis. The typical symptoms are hypoxaemia, noisy breathing, snoring, stridor, cyanosis, bradycardia, feeding difficulties, and failure to thrive. Distraction osteogenesis has recently been considered as a surgical option for early intervention to lengthen the mandible and relieve respiratory problems. Piezosurgery offers a modality to make precise bone cuts preserving vital structures, minimizing the invasiveness of the surgical procedure, and offering bloodless field. We present case of 1-year-old male malnourished child with Pierre Robin sequence and tracheostomy in situ since day 11 of his birth. The staged treatment plan involving mandibular lengthening in which mandibular osteotomies were performed with the piezoelectric scalpel followed by decannulation of tracheostomy, which has been described in detail in this article.

Surgical Management of Pierre Robin Sequence: Using Mandibular Distraction Osteogenesis to Address Hypoventilation and Failure to Thrive in Infancy.

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.

Mandibular distraction osteogenesis for the treatment of neonatal tongue-based airway obstruction.

Early postnatal tracheostomy for airway compromise is associated with high morbidity and cost. In certain patients with tongue-base airway obstruction (TBAO), mandibular distraction osteogenesis may be preferred. We present a comprehensive analysis of surgical, airway, and cephalometric outcomes in a large series of neonatal patients with TBAO. A retrospective review was performed of patients with laryngoscopically proven TBAO who underwent mandibular distraction osteogenesis before 1 year of age at our institution. Demographic, operative, postoperative, polysomnographic, and radiographic data were analyzed with the appropriate statistical test. Between 2010 and 2013, 28 patients younger than 1 year underwent mandibular distraction for TBAO. Distraction was performed for documented TBAO and failure to thrive at an average age of 58 days (range, 11-312) days with distractor removal after an average of 90 days. Preoperative polysomnograms were obtained on 20 patients with an average apnea-hypopnea index of 39.3 ± 22.0/h; the apnea-hypopnea index on postoperative polysomnograms obtained after distraction completion was significantly reduced in all 14 patients in whom it was measured (mean, 3.0 ± 1.5/h; P < 0.0001). Twenty patients transitioned to oral feeding, and cephalometric and airway diameters were improved (P < 0.0001). Distraction was successful in all but 4 patients including all patients with GILLS scores of 2 or less and 66% of patients with GILLS scores of 3 or greater. Neonatal mandibular distraction is a powerful tool to treat critical obstructive apnea in patients with TBAO. Appropriate patient selection remains a challenge; however, mandibular distraction represents a compelling treatment modality.

Risk management protocol for gastrostomy and jejunostomy insertion in ventilator dependent infants.

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.

Outcomes of percutaneous endoscopic gastrostomy in children.

Percutaneous endoscopic gastrostomy (PEG) is a relatively safe and minimally invasive surgical method for providing enteral access in children. In pediatrics, the indications for PEG placement frequently include malnutrition or failure to thrive, as well as oropharyngeal dysphagia, especially in children with neurological impairment (NI). The risk for postoperative complications is low. However, among children with NI, gastroesophageal reflux disease (GERD) may necessitate fundoplication prior to gastrostomy tube placement. Preoperative pH probe testing has not been shown to be an effective screening tool prior to PEG placement among patients with GERD. Laparoscopic gastrostomy tube insertion was introduced in pediatric patients in an attempt to decrease complications associated with PEG. Although outcomes were reported to be similar to or better than PEG alone, future comparative studies are needed to better define the optimal patient demographic for this technique.

Management of gastroesophageal reflux associated with malrotation in children.

AIM: Children being investigated for gastroesophageal reflux (GOR) have a high incidence of malrotation. Current literature suggests these patients should be managed with a combined antireflux and Ladd's procedure. We review our experience, the largest series to date, of performing an elective Ladd's procedure as the first-line intervention. METHOD: Retrospective case note review of 20 children with significant symptoms of GOR and an incidental finding of malrotation. Children presenting immediately with bilious vomiting were excluded. All patients underwent a Ladd's procedure as their sole primary operative intervention. RESULTS: Median age at operation was 7 months (21 days-12 years). Fifteen patients (75%) had evidence of reflux on barium contrast study. All children were followed up for at least 6 months. Eighteen (90%) had resolution or significant improvement of their symptoms postsurgery. Only 3 have not managed to tolerate a full oral diet, all unrelated to GOR. None of our series required an antireflux procedure. CONCLUSION: In children with debilitating vomiting necessitating surgical management, a contrast study is imperative in the work up. The high incidence of GOR and the significant improvement after correction of malrotation show the relationship between delayed gastric emptying and GOR. We suggest that when an abnormally placed duodenojejunal flexure is found, a Ladd's procedure alone is sufficient and may obviate the need for a more invasive antireflux procedure.

Survival of children following Nissen fundoplication.

BACKGROUND: Analyses of survival after fundoplication in childhood are often restricted to 30-day mortality, or to the neurologically impaired. The objective of this study was to report actuarial survival and variables associated with mortality for all children undergoing fundoplication. METHODS: This was a prospective observational study of fundoplication surgery by one surgeon; the endpoint was survival. Using a Cox proportional hazards model, gastrostomy, neurological status, tracheostomy, congenital cardiac disease, syndromic status, presence of congenital anomaly, other chronic disease, weight z-score at time of surgery, need for revisional fundoplication, use of laparoscopic surgery, gastric drainage procedures, age and sex were assessed for their influence on survival. RESULTS: Two-hundred and thirty children underwent 255 fundoplications at a median age of 3·6 years. Forty-six children (20·0 per cent) died during a median follow-up of 2·8 (range 0·5-11·2) years. Statistical modelling showed gastrostomy (relative risk of death 11·04, P < 0·001), cerebral palsy (relative risk 6·58, P = 0·021) and female sex (relative risk 2·12, P = 0·015) to be associated with reduced survival. Revisional fundoplication was associated with improved survival (relative risk of death 0·37, P = 0·037). Survivors had significantly higher weight z-scores (-1·4 versus - 2·9 for those who died; P = 0·001). The 5-year survival rate after fundoplication for children with cerebral palsy and gastrostomy was 59 per cent. CONCLUSION: Survival of children following fundoplication is related principally to the presence of a gastrostomy and neurological status. Estimates of children's life expectancy should take account of the poorer survival of neurologically impaired children who undergo fundoplication, presumably due to the related co-morbidities that lead to a gastrostomy.

Gastric heterotopia with extensive involvement of the small intestine associated with congenital short bowel syndrome and intestinal malrotation.

We present a case of extensive gastric heterotopia involving the small intestine associated with congenital short bowel syndrome and malrotation. The infant showed a normal mesenteric artery, without signs of "apple peel" deformity. Gastric heterotopia extended from the duodenum to the mid-ileum involving the short bowel. Gastric mucosa heterotopia may involve any segment of the gastrointestinal tract. It can be associated with pancreatic heterotopia and Meckel diverticulum. However, our case showed involvement of two-thirds of the small intestine without pancreatic heterotopia. To our knowledge, this is the first report of gastric heterotopia with congenital short gut syndrome and malrotation.

Weight gain following vertical expandable prosthetic titanium ribs surgery in children with thoracic insufficiency syndrome.

STUDY DESIGN: Retrospective review of patients from a multicenter database. OBJECTIVE: To evaluate the nutritional status of children with thoracic insufficiency syndrome (TIS) and to determine if treatment with vertical expandable prosthetic titanium ribs (VEPTR) leads to improvements in weight percentile. SUMMARY OF BACKGROUND DATA: Children with pulmonary insufficiency characteristically have poor nutrition as the energy expenditure from the extra work of breathing approaches the nutritional gain of eating. To our knowledge, no previous studies have examined the relation between VEPTR and potential nutritional improvements in children with TIS. METHODS: Seventy-six patients at 7 different institutions underwent placement of VEPTR devices for treatment or prevention of TIS. Mean age at surgery was 3.7 years (range, 8 months-14 years). All patients were observed for a minimum of 24 months with an average follow-up of 3.3 years (range, 2-6 years). Before surgery and at each postoperative visit, patients were weighed and the Cobb angle was measured. All weights were converted to normative percentiles based on the patient's age. RESULTS: Overall, we found a significant increase in the percentile of patients' weights after VEPTR surgery (P = 0.0004). Of the 76 patients in our series, 60 (79%) were < or =5 percentile in weight before surgery. Of these most nutritionally depleted patients, 40% (24/60) had increase in percentile weight after surgery; more subjects may have improved, but due to the basement effect < or =5 percentile any improvement in which the final weight was under 5 percentile may not have been detected. Of the 16 patients who were >5 percentile weight before surgery, 50% (8/16) had increased weight percentiles after surgery. For most patients, the majority of weight gain occurred between 4 and 8 months after surgery; weight gain continued up to 48 months after surgery. The change in Cobb angle had no relation to the change in weight percentile. CONCLUSION: A total of 79% of patients with TIS were less than 5 percentile in weight, thus meeting the criteria for "failure to thrive." Our study demonstrates a significant improvement in the nutritional status of these children after VEPTR surgery, which is an important outcome measure in this population.

[Percutaneous endoscopic gastrostomy (PEG) in children: indications, the procedure, outcomes, short and long-term complications].

BACKGROUND: Feeding through tubes placed in the intestine is a useful way of nutritional support in a patient who is unable to eat but has a well functioning gastrointestinal system. Till 1980, the acceptable technique to place a gastrostomy tube was surgical. However, in the past twenty years percutaneous endoscopic gastrostomy (PEG) has replaced surgical gastrostomy in most settings. GOALS: In this study we explored the indications, the age of the patients, the conditions in which the procedure took place and its outcome in children. The study aimed to investigate the effectiveness of this method in children. METHODS: The data was gathered retrospectively according to information found in the files and a questionnaire the parents answered. Fifty-two children from the Tel Aviv Medical Center and from the Sheba Medical Center who underwent a PEG procedure were studied. The data gathered from the study included age, gender, origin, sequence of the procedure, indications, place, performers, complications and the effectiveness of the method according to parents' satisfaction and weight of the patient. In addition, the following parameters were studied: type of anesthesia, sort of antibiotics that were provided, number of cases in which enteral nutrition was given permanently or temporarily, type of formula that was given and the way it was introduced, number of failures and fundoplications. RESULTS: Mean age of the children was 5.4 years. Ninety six percent of the procedures succeeded. The most common indications were failure to thrive (35%) and neurological disorders. In 56% of cases the performer was a gastroenterologist alone. When the procedures were performed by gastroenterologists, a lower rate of complications was seen than with a gastroenterologist and a surgeon (20.8% vs. 52.6% accordingly). In 57% of the children there were no complications observed. In addition, most of the complications which were observed were minor (abdominal pain, nausea and vomiting and reflux). CONCLUSIONS: According to this study we concluded that PEG is a minimally invasive technique, associated with a low rate of severe complications and provides significant support for children who need enteral nutrition.

A case of severe Pierre Robin sequence with failure to thrive and tachycardia resolved after redo-fundoplication and hiatoplasty.

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.

Five-year follow-up after pediatric living related small bowel transplantation between two monozygotic twins.

Two 13-year-old monozygotic twins were used for living related small bowel transplantation (SBTx). The recipient presented with short gut syndrome secondary to complicated abdominal surgery. The indication for SBTx was based on a failure to thrive and a poor tolerance of TPN. The donor was an identical twin, as demonstrated by skin graft acceptance, which allowed performance of SBTx without immunosuppression. Growth charts were used to follow intestinal absorption functions and body composition. The donor was used as a control for the recipient. The recipient, who was transplanted with 160 cm of donor ileum, was discharged on postoperative day 62 on a regular diet. Before SBTx the recipient was 10 kg lighter in body weight than the donor, a gap that was progressively reduced over the follow-up period. A height deficit of 3 cm reversed within 1 year after SBTx. A 10-kg deficit in fat-free body mass was completely extinguished within 18 months. By 18 months posttransplant, recipient serum albumin and prealbumin were normal and comparable to donor values. d-Xylose absorption in the recipient remained lower than that in the donor. Within 6 months fecal fat excretion normalized in the recipient. d-Xylose absorption and fecal fat excretion were always within a normal range in the donor.

Indications for cesarean deliveries in Norway.

OBJECTIVE: The purpose of this study was to investigate the indications for cesarean deliveries in Norway, related to type of operation, parity, and gestational age. STUDY DESIGN: This was a prospective survey that used information provided by clinicians at 24 maternity units. Two thousand seven hundred seventy-eight cesarean deliveries were included, which represents 69.7% of all cesarean deliveries in Norway during the study period. RESULTS: The cesarean delivery rate varied by maternal and gestational age, parity, and hospital of delivery. Seven indications accounted for 77.7% of the operations: fetal stress (21.9%), failure to progress (20.7%), previous cesarean delivery (8.9%), breech presentation >or=34 weeks of gestation (8.4%), maternal request (7.6%), preeclampsia (6.2%) and failed induction (4.0%). Of the total deliveries, 64.3% were emergency operations. CONCLUSION: Accurate information about indications for cesarean deliveries in Norway has been obtained. Two thirds of all deliveries were emergency operations; the most important indications were fetal stress and failure to progress. In the elective cesarean delivery group, the two most important indications were previous cesarean delivery and maternal request.