Major surgical conditions of childhood and their lifelong implications: comprehensive review

Abstract

Background: In recent decades, the survival of children with congenital anomalies and paediatric cancer has improved dramatically such that there has been a steady shift towards understanding their lifelong health outcomes. Paediatric surgeons will actively manage such conditions in childhood and adolescence, however, adult surgeons must later care for these 'grown-ups' in adulthood. This article aims to highlight some of those rare disorders encountered by paediatric surgeons requiring long-term follow-up, their management in childhood and their survivorship impact, in order that the adult specialist may be better equipped with skills and knowledge to manage these patients into adulthood.

Methods: A comprehensive literature review was performed to identify relevant publications. Research studies, review articles and guidelines were sought, focusing on the paediatric management and long-term outcomes of surgical conditions of childhood. The article has been written for adult surgeon readership.

Results: This article describes the aforementioned conditions, their management in childhood and their lifelong implications, including: oesophageal atresia, tracheo-oesophageal fistula, malrotation, short bowel syndrome, duodenal atresia, gastroschisis, exomphalos, choledochal malformations, biliary atresia, Hirschsprung disease, anorectal malformations, congenital diaphragmatic hernia, congenital lung lesions and paediatric cancer.

Conclusion: The increasing survivorship of children affected by surgical conditions will translate into a growing population of adults with lifelong conditions and specialist healthcare needs. The importance of transition from childhood to adulthood is becoming realized. It is hoped that this timely review will enthuse the readership to offer care for such vulnerable patients, and to collaborate with paediatric surgeons in providing successful and seamless transitional care.

Fig. 1

Gross classification system of the five subtypes of oesophageal atresia/tracheo-oesophageal fistula (OA/TOF)

Fig. 2

Variant anatomy of the midgut in three distinct morphologies: normal rotation, malrotation and non-rotation

Fig. 3

Kimura duodenoduodenostomy—a popular procedure for duodenal atresia

Fig. 4

Newborn baby with gastroschisis

Fig. 5

Infant with exomphalos major

Fig. 6

Todani classification divides choledochal malformations into five distinct groups: type i (80–90%)—dilatation only involving the extrahepatic bile duct, type II (3%)—a diverticulum of the extrahepatic bile duct, type III (5%)—a choledochocoele which involves dilatation of the intraduodenal part of the common bile duct, type IV (10%)—dilatation involving both the intra- and extrahepatic bile ducts, and type v (rare)—Caroli’s disease

Fig. 7

Schematic illustration of the three major subtypes of biliary atresia (adapted from Hartley et al.)

Fig. 8

The Kasai portoenterostomy

Fig. 9

Schematic illustration of the three common operations for Hirschsprung disease—Yancey-Soave, Swenson and Duhamel procedures

Fig. 10

Male newborn with a high anorectal malformation

Fig. 11

Illustrations of common subtypes of congenital lung lesions

Fig. 12

Transverse CT image showing a right lower lobar congenital pulmonary airway malformation (CPAM)

Fig. 13

Plain radiograph of a newborn with left congenital diaphragmatic hernia (CDH)

Fig. 14

Congenital diaphragmatic hernia (CDH) study group classification system popular for grading severity of diaphragmatic hernia

Fig. 15

Scoliosis which has developed after chest wall tumour resection