Marija Lukac

International survey on the management of esophageal atresia

INTRODUCTION Because many aspects of the management of esophageal atresia (EA) are still controversial, we evaluated the practice patterns of this condition across Europe. METHODS A survey was completed by 178 delegates (from 45 [27 European] countries; 88% senior respondents) at the EUPSA-BAPS 2012. RESULTS Approximately 66% of respondents work in centers where more than five EA repairs are performed per year. Preoperatively, 81% of respondents request an echocardiogram, and only 43% of respondents routinely perform preoperative bronchoscopy. Approximately 94% of respondents prefer an open approach, which is extrapleural in 71% of respondents. There were no differences in use of thoracoscopy between Europeans (10%) and non-Europeans (11%, p = nonsignificant). Approximately 60% of respondents measure the gap intraoperatively. A transanastomotic tube (90%) and chest drain (69%) are left in situ. Elective paralysis is adopted by 56% of respondents mainly for anastomosis tension (65%). About 72% of respondents routinely request a contrast study on postoperative day 7 (2-14). Approximately 54% of respondents use parenteral nutrition, 40% of respondents start transanastomotic feeds on postoperative day 1, and 89% of respondents start oral feeds after postoperative day 5. Pure EA: 46% of respondents work in centers that repair two or more than two pure EA a year. About 60% of respondents opt for delayed primary anastomosis at 3 months (1-12 months) with gastrostomy formation without esophagostomy. Anastomosis is achieved with open approach by 85% of respondents. About 47% of respondents attempt elongation of esophageal ends via Foker technique (43%) or with serial dilations with bougies (41%). Approximately 67% of respondents always attempt an anastomosis. Gastric interposition is the commonest esophageal substitution. CONCLUSION Many aspects of EA management are lacking consensus. Minimally invasive repair is still sporadic. We recommend establishment of an EA registry.

Is abandonment of nonoperative management of hypertrophic pyloric stenosis warranted

AIM Evaluation of the effectiveness of oral atropine versus surgical therapy for hypertrophic pyloric stenosis (HPS). METHODOLOGY A total of 66 consecutive patients with HPS were treated at the University Childrens Hospital between January 2006 and December 2011. The diagnosis was initially based on medical history and confirmed by ultrasonography (US). The patients were divided into two groups according to the treatment preferred by their parents. The conservatively treated group, consisting of 33 boys and 7 girls, mean age 22.25 days, was given water-soluble atropine sulfate therapy at an initial dose of 0.05 mg/kg/day divided into 8 single doses, and administered after stomach decompression, 20 minutes prior to feeding. If vomiting persisted, the daily dose was progressively increased up to 0.18 mg/kg. If vomiting did not stop and full oral feeding was not reestablished in a week, surgery was done. The second group of 26 patients, mean age 20.86 days, underwent an operative procedure, Ramstedt extramucosal pyloromyotomy after the initial resuscitation. US evaluation was performed on days 7, 14, and 21. The outcome of the treatment was tested by Yates modification of the χ2 test. RESULTS In the group of patients treated with atropine sulfate, 10 (25%) failed to respond to therapy, therefore, 8 boys and 2 girls underwent surgical treatment between the fifth and seventh day following institution of therapy. The remaining patients who received atropine sulfate (75%) were discharged when vomiting ceased, between the sixth and eighth day. They continued to take oral medication for 4 to 6 weeks, and were followed up by an ultrasound examination. The operated patients were discharged between the third and fifth day after surgery. There was a significant statistical difference between the groups regarding the outcome at a significance level of p < 0.05 (Yates χ2 = 5.839), with no complications regardless of the treatment option. However, at the significance level of p < 0.01 (Yates χ2 = 7.661), these methods demonstrate a difference in favor of surgical treatment. CONCLUSION Further investigation of oral, intravenous or combined atropine sulfate treatment may clarify its position as an alternative to pyloromyotomy.

Longitudinal Cystic Fibrosis Care

Cystic fibrosis is a complex disease entity that presents considerable lifelong challenges. Implementation of medical and surgical treatment options involves multisystem interventions to prevent and treat lung and gastrointestinal manifestations of cystic fibrosis and associated comorbidities. From birth through adulthood, cystic fibrosis care entails a longitudinal regimen aimed at achieving relief of disease symptoms and enhanced life expectancy. With increased knowledge of the molecular behavior of the cystic fibrosis transmembrane conductance regulator (CFTR) in health and disease, clinical practice has been enriched by the prospect of novel strategies, including mutation‐specific drug and gene therapy targeting restoration of corrupted transepithelial ion transport. Emerging paradigms of comprehensive care increasingly enable personalized solutions to address the root cause of disease‐transforming management options for individuals with cystic fibrosis.

Surgical treatment of cloacal anomalies

Abstract From 1989–1998 14 patients were treated with cloacal anomalies: 5 typical cloacas (PC), 5 posterior cloacas, and 4 cloacal exstrophies (CE); 12 underwent surgery. Four typical cloacas were resolved with posterior sagittal anorectovagino-urethroplasty (PSARVUP), whereas in the 5th total urogenital mobilization (TUM) was used. Three PCs were managed with transanorectal TUM and 2 with anterior TUM without opening the anal canal and rectum (without a protective colostomy). Two CEs were treated with atypical procedures. Two patients with CE died without surgery and 2 died after surgery due to complex associated anomalies. During postoperative follow-up of 1–8 years, 5 children had voluntary bowel movements and no soiling while the others had soiling with or without enemas; 1 had stress incontinence; 3 were on clean intermittent catheterization due to neurogenic bladder and were dry.PSARVUP provides a satisfactory result if there is no sacral anomaly. TUM makes this operation easier to perform. In patients with a PC it is sometimes possible using TUM to separate the urinary from the genital tract and remove the accessory urethra without opening the anus and rectum.

Current Management of Congenital Pulmonary Airway Malformations: A “European Pediatric Surgeons' Association” Survey

Abstract Aim To define current management of congenital pulmonary airway malformation (CPAM). Methods A total of 181 European Pediatric Surgeons’ Association members (91% senior) from 48 countries completed an online questionnaire. Main Results Prenatal: 93% respondents work in centers with prenatal diagnosis facilities, and 27% in centers offering in utero surgery. Prenatal counseling is performed by 86% respondents, 22% of whom see >10 cases per year. Risk of single pre‐/postnatal complications is deemed low (<5%) by more than 60% of respondents. Eighty‐six percent respondents do not offer pregnancy termination for prenatally diagnosed CPAM. Fetal hydrops is the most frequent indication for termination (87%), followed by parental willingness (52%). Prenatal surgery is an option for 44% respondents, preferring thoracoamniotic shunt (82%). Postnatal: 75% respondents operate on asymptomatic patients, 18% before 6 months of age, 62% between 6 and 12 months of age, and 20% after 12 months of age. Risk of infection (86%), cancer (63%), and symptoms development (62%) are indications for surgery in asymptomatic CPAM. Sixty‐three percent prefer a thoracotomy. Lobectomy is the preferred procedure (58% respondents). Motivations against surgery include lesion <1 cm (64%), risk of postoperative complications (37%), and lack of evidence favoring surgery (27%). Seventeen percent respondents have seen at least one patient with CPAM with lung cancer, in 89% of the cases within the CPAM. Of all the respondents, 83% and 22% offered dedicated follow‐up and genetic screening, respectively. Conclusion Current pre‐ and postnatal management of CPAM lacks uniformity, particularly for surgical indication, timing, and approach. Efforts should be made toward standardization. Risk of CPAM‐associated cancer is not clear.

Thoracoschisis associated with Limb Body Wall Complex

Thoracoschisis is a rare condition. A female newborn presented with right-sided thoracoschisis, associated with diaphragmatic hernia and protrusion of an accessory liver lobe through the chest wall defect along with deformity of the right forearm and hand duplication. Diagnosed as part of the limb-body wall complex (LBWC), management included resection of the exteriorized liver lobe followed by right hemidiaphragm and thoracic wall reconstruction.

Impact of bowel management in alleviating symptoms of urinary incontinence in patients with spina bifida associated with overactive bladder and detrusor sphincter dyssynergia

To examine the effects of bowel management on urinary incontinence in patients with spina bifida associated with overactive bladder (OAB) and detrusor sphincter dyssynergia (DSD).

Ectodermal Defects and Anal Atresia in a Child with a TP63 Mutation--Expanding the Phenotypic Spectrum.

Ectodermal dysplasias caused by mutations in the TP63 gene comprise a group of disorders characterized by a spectrum of ectodermal changes, orofacial clefting, and split hand or foot malformation. We report on a boy with a mutation located in the DNA‐binding domain of the TP63 gene with atypical phenotype. These data provide additional evidence of the great variability seen in TP63‐related disorders and further delineation of genotype‐phenotype correlations.