Marc H. W. A. Wijnen

Closure of giant omphaloceles by the abdominal wall component separation technique in infants

BACKGROUND/PURPOSE Several techniques have been described to repair giant omphaloceles. There is no procedure considered to be the criterion standard worldwide. The aim of the present prospective study was to analyze the early and late results of secondary closure of giant omphaloceles using the component separation technique (CST) in infants. METHODS From January 2004 to January 2007, 10 consecutive pediatric patients with a giant omphalocele were treated at our department. Initially, patients were treated conservatively. After epithelialization of the omphalocele, the abdominal wall was reconstructed using CST. Patients were monitored for complications during admission, and all patients were seen for follow-up. RESULTS Component separation technique was performed at median age of 6.5 months (range, 5-69 months). The median diameter of the hernia was 8 cm (range, 6-9 cm). There was no mortality. The postoperative course was uneventful in 7 patients. Complications were seen in 3 patients (infection, skin necrosis, and hematoma). Median hospital stay was 7 days. After median follow-up of 23.5 months (range, 3-39 month), no reherniations were found. CONCLUSIONS The CST is a safe 1-stage procedure for secondary closure in children with a giant omphalocele without the need for prosthetic material and with good clinical outcome.

Pancreatic injury in children: good outcome of nonoperative treatment.

PURPOSE Treatment of blunt injury of the pancreas in children remains controversial. Some prefer nonoperative treatment, whereas others prefer operative management in selected cases. This report reviews the treatment of patients with blunt pancreatic trauma admitted to a level I pediatric trauma center in The Netherlands. METHODS Medical records of all children less than 15 years with blunt pancreatic trauma admitted to the University Medical Center St Radboud in the period 1975 to 2003 were retrospectively analyzed. RESULTS Thirty-four children were included, age 3 to 14 years. Most injuries were because of bicycle accidents (58%). On admission, amylase was raised in 90% of the patients. Five patients had pancreatic duct injuries identified by imaging (endoscopic retrograde cholangiopancreaticography was used once, magnetic resonance cholangiopancreaticography twice) or at surgery. Thirty-one children were initially managed nonoperatively. Pancreatic surgery was performed in 3 children (1 Roux-Y, 2 drainage only). Mean hospital stay was 29 days in the operative group and 24 days in the nonoperative group. Fluid collections developed in 2 operated patients. Both resolved spontaneously. In 14 of the 31 nonoperated patients, a pseudocyst developed. Only 6 of these needed secondary intervention. Of these, 3 were drained percutaneously. There was no mortality and no long-term morbidity in both groups. CONCLUSIONS Nonoperative management of pancreatic injury in children has good clinical outcome. Only 10% need secondary surgery. In 50%, pseudocysts develop of which half can be managed nonoperatively. The reliability of computed tomographic scan grading is of limited value to decide whether to operate primarily. There is little to gain with ERCP and stenting. The place of MRCP as a noninvasive diagnostic tool remains to be determined.

Neurological symptoms in children with intussusception.

Aim:  The classical combination of abdominal pain, vomiting, rectal blood loss and a palpable abdominal mass is only present in a minority of children with intussusception. Neurological signs and symptoms have been described, but are not a well understood phenomenon. We performed a retrospective study to ascertain the frequency and nature of these symptoms and to describe the characteristics of the patients presenting in this atypical way.

Transanal endorectal pull-through for classic segment Hirschsprung's disease: With or without laparoscopic mobilization of the rectosigmoid?

BACKGROUND It has been suggested that the outcome of transanal endorectal pull-through for classic Hirschprungs disease can be improved by laparoscopically mobilizing the colon before the pullthrough. METHODS Charts of 43 patients (2005-2009) with proven recto-sigmoid aganglionosis were retrospectively analyzed with respect to postoperative outcomes. Twenty-one had been treated with the transanal endorectal pull through (TERPT) and 22 with the laparoscopically assisted TERPT (LTERPT). RESULTS Gender ratio, congenital anomalies, preoperative enterostomy, and follow up did not differ between the groups. More colon was resected in the TERPT group: median 25 cm vs. 15 cm in the L-TERPT group (p<0.001). The TERPT-procedure took less time: median 153 min. vs. L-TERPT 263 min (p<0.001). Postoperatively, three patients showed colonic torsions after TERPT (p=0.07). The long-term clinical outcomes did not differ significantly between both groups. There was a significant association between length of resection and obstructive symptoms (OR=0.92, p=0.01). CONCLUSION Postoperative and clinical outcomes are similar using the TERPT or L-TERPT to correct classic segment Hirschsprungs disease. Prevention of colonic torsion should be the prime concern during the TERPT procedure. L-TERPT requires laparoscopic equipment and takes more operation time, whereas TERPT leaves no visible scars. The positive relation between the larger length of resection and obstructive symptoms requires additional research.

Recurrences after thyroglossal duct cyst surgery: Results in 207 consecutive cases and review of the literature.

A thyroglossal duct cyst is the most common form of congenital anomaly in the neck. Surgical removal is very effective. However, in some cases, a cyst recurs. The purpose of this study was to identify factors that predispose to recurrence of a thyroglossal duct cyst.

Malignant transformation in sacrococcygeal teratoma and in presacral teratoma associated with Currarino syndrome: a comparative study

BACKGROUND/PURPOSE The risk of malignant transformation of sacrococcygeal teratoma (SCT) and of presacral teratoma in Currarino syndrome (CS) may differ despite the similar position and appearance. METHODS Malignant transformation and teratoma recurrence were assessed in a national retrospective comparative analysis of 205 SCT and 16 CS patients treated in one of the six pediatric surgical centers in the Netherlands between January 1981 and December 2010. RESULTS The malignancy free survival of patients with SCT was lower than for patients with a presacral teratoma associated with CS (80% and 58% after one and two years in SCT versus 100% after two years in CS, p=0.017) CONCLUSIONS: In SCT, malignancy and recurrence risk are high. Therefore, early and complete resection is mandatory. Our data show that the risk of malignant transformation of a presacral teratoma in CS is small.

Outcome after anal intrasphincteric Botox injection in children with surgically treated Hirschsprung disease.

Objectives: A nonrelaxing internal anal sphincter is present in a relatively large proportion of children with surgically treated Hirschsprung disease (HD) and can cause obstructive gastrointestinal symptoms. The short- and long-term outcome and adverse effects of intrasphincteric botulinum toxin (Botox) injections in children with obstruction after surgically treated HD are evaluated. Methods: The outcome of children with surgically treated HD treated with intrasphincteric Botox injections for obstructive symptoms was analyzed with a retrospective chart review between 2002 and 2013 in the University Medical Centers of Maastricht and Nijmegen. Results: A total of 33 patients were included. The median time of follow-up was 7.3 years (range 1–24). A median of 2 (range 1–5) injections were given. Initial improvement was achieved in 76%, with a median duration of 4.1 months (range 1.7–58.8). Proportion of children hospitalized for enterocolitis decreased after treatment from 19 to 7. A good long-term response was found in 49%. Two children experienced complications: transient pelvic muscle paresis with impairment of walking. In both children symptoms resolved within 4 months without treatment. Conclusions: Intrasphincteric Botox injections in surgically treated HD are an effective long-term therapy in approximately half of our patients with obstructive symptoms. The possibility of adverse effects should be noticed.

No major role for periconceptional folic acid use and its interaction with the MTHFR C677T polymorphism in the etiology of congenital anorectal malformations

BACKGROUND Both genetic and nongenetic factors are suggested to be involved in the etiology of congenital anorectal malformations (ARM). Maternal periconceptional use of folic acid supplements were inconsistently suggested to play a role in the prevention of ARM. Therefore, we investigated independent associations and interactions of maternal periconceptional folic acid supplement use and the infant and maternal MTHFR (methylenetetrahydrofolate reductase) C677T polymorphisms with the risk of ARM and subgroups of ARM. METHODS A case-control study was conducted among 371 nonsyndromic ARM cases and 714 population-based controls born between 1990 and 2012 using maternal questionnaires and DNA samples from mother and child. Cases were treated for ARM at departments of Pediatric Surgery of the Radboud university medical center, Sophia Childrens Hospital-Erasmus MC Rotterdam, and the University Medical Center Groningen in The Netherlands and hospitals throughout Germany. RESULTS No association with folic acid use was present (odds ratio = 1.1; 95% confidence interval: 0.8-1.4) for ARM as a group. Infant and maternal MTHFR C677T polymorphisms were weakly associated with isolated ARM in particular. Lack of folic acid supplement use in combination with infants or mothers carrying the MTHFR C677T polymorphism did not seem to increase the risk of ARM or subgroups of ARM. The relative excess risks due to interaction did not clearly indicate interaction on an additive scale either. CONCLUSION This first study investigating interactions between periconceptional folic acid supplement use and infant and maternal MTHFR C677T polymorphisms in the etiology of ARM did not provide evidence for a role of this gene-environment interaction.

Parental subfertility, fertility treatment, and the risk of congenital anorectal malformations

Background: Fertility treatment seems to play a role in the etiology of congenital anorectal malformations, but it is unclear whether the underlying parental subfertility, ovulation induction, or the treatment itself is involved. Therefore, we investigated the odds of anorectal malformations among children of subfertile parents who conceived with or without treatment compared with fertile parents. Methods: We performed a case-control study among 380 cases with anorectal malformations treated at 3 departments of pediatric surgery in The Netherlands and 1973 population-based controls born between August 1988 and August 2012. Parental questionnaires were used to obtain information on fertility-related issues and potential confounders. Results: In singletons, increased risks of anorectal malformations were observed for parents who underwent intracytoplasmic sperm injection (ICSI) or in vitro fertilization (IVF) treatment compared with fertile parents (odds ratio = 2.4 [95% confidence interval = 1.0–5.9] and 4.2 [1.9–8.9], respectively). For subfertile parents who conceived after IVF treatment, an elevated risk was also found when they were compared with subfertile parents who conceived without treatment (3.2 [1.4–7.2]). Among children of the latter category of parents, only the risk of anorectal malformations with other major congenital malformations was increased compared with fertile parents (2.0 [1.3–3.3]). No associations were found with intrauterine insemination or use of hormones for ovulation induction. Conclusions: We found evidence of a role of ICSI and IVF treatments in the etiology of anorectal malformations. However, subfertility without treatment increased only the risk of anorectal malformations with additional congenital malformations.

Irreversible Respiratory Failure in a Full-Term Infant with Features of Pulmonary Interstitial Glycogenosis as Well as Bronchopulmonary Dysplasia

Pulmonary interstitial glycogenosis (PIG) is a rare interstitial lung disease in the newborns. We report on the clinical presentation and pathological findings of a full-term male infant with pulmonary hypertension requiring extracorporeal membrane oxygenation (ECMO). An open lung biopsy demonstrated interstitial changes resembling pulmonary interstitial glycogenosis as well as bronchopulmonary dysplasia (BPD), without convincing evidence of maturational arrest, infection, alveolar proteinosis, or alveolar capillary dysplasia. The boy was treated with glucocorticoids and, after a few days, was weaned from ECMO. A few hours later, the patient died due to acute severe pulmonary hypertension with acute right ventricular failure. The etiology and underlying pathogenic mechanisms of PIG are unknown. The clinical outcomes are quite varied. Deaths have been reported when PIG exists with abnormal lung development and pulmonary vascular growth and congenital heart disease. No mortality has been reported in PIG together with BPD in full-term infants. In this article, we reported on a full-term infant with interstitial changes resembling PIG and BPD who expired despite no convincing evidence of an anatomical maturational arrest or congenital heart disease.