Paediatric surgery

This chapter outlines the principles of managing paediatric surgical cases. It provides an overview of the assessment of a child with abdominal wall defects, inguinal or scrotal swellings and neck swellings. Conditions such as oesophageal atresia, phyloric stenosis, malrotation and volvulus, intussusception, hirschsprung’s disease, necrotizing enterocolitis, childhood hernias, undescended testis and solid tumours of childhood.

Heritability and De Novo Mutations in Oesophageal Atresia and Tracheoesophageal Fistula Aetiology

Tracheoesophageal Fistula (TOF) is a congenital anomaly for which the cause is unknown in the majority of patients. OA/TOF is a variable feature in many (often mono-) genetic syndromes. Research using animal models targeting genes involved in candidate pathways often result in tracheoesophageal phenotypes. However, there is limited overlap in the genes implicated by animal models and those found in OA/TOF-related syndromic anomalies. Knowledge on affected pathways in animal models is accumulating, but our understanding on these pathways in patients lags behind. If an affected pathway is associated with both animals and patients, the mechanisms linking the genetic mutation, affected cell types or cellular defect, and the phenotype are often not well understood. The locus heterogeneity and the uncertainty of the exact heritability of OA/TOF results in a relative low diagnostic yield. OA/TOF is a sporadic finding with a low familial recurrence rate. As parents are usually unaffected, de novo dominant mutations seems to be a plausible explanation. The survival rates of patients born with OA/TOF have increased substantially and these patients start families; thus, the detection and a proper interpretation of these dominant inherited pathogenic variants are of great importance for these patients and for our understanding of OA/TOF aetiology.

The timing of oesophageal dilatations in anastomotic stenosis after one-stage anastomosis for congenital oesophageal atresia

Background In infants with congenital oesophageal atresia, anastomotic stenosis easily occurs after one-stage oesophageal anastomosis, leading to dysphagia. In severe cases, oesophageal dilatation is required. In this paper, the timing of oesophageal dilatation in infants with anastomotic stenosis was investigated through retrospective data analysis. Methods The clinical data of 107 infants with oesophageal atresia who underwent one-stage anastomosis in our hospital from January 2015 to December 2018 were retrospectively analysed. Data such as the timing and frequency of oesophageal dilatation under gastroscopy after surgery were collected to analyse the timing of oesophageal dilatation in infants with different risk factors. Results For infants with refractory stenosis, the average number of dilatations in the early dilatation group (the first dilatation was performed within 6 months after the surgery) was 5.75 ± 0.5, which was higher than the average of 7.40 ± 1.35 times in the normal dilatation group (the first dilatation was performed 6 months after the surgery), P = 0.038. For the infants with anastomotic fistula and anastomotic stenosis, the number of oesophageal dilatations in the early dilatation group was 2.58 ± 2.02 times, which was less than the 6.38 ± 2.06 times in the normal dilatation group, P = 0.001. For infants with non-anastomotic fistula stenosis, early oesophageal dilatation could not reduce the total number of oesophageal dilatations. Conclusion Starting to perform oesophageal dilatation within 6 months after one-stage anastomosis for congenital oesophageal atresia can reduce the required number of dilatations in infants with postoperative anastomotic fistula and refractory anastomotic stenosis.

Vascular anatomical considerations in preparing colonic flaps to replace the oesophagus

Background The colon is among the best options to substitute the oesophagus; it is well known for its durability and good function that makes it most suitable for paediatric patients. The steps of the procedure, postoperative complications, and outcome assessment were thoroughly discussed in previous reports. However, in this report, we have tried to focus on one basic and essential step of the operation, which is fashioning of the colonic flap used to substitute the oesophagus. Results The study included 50 consecutive paediatric cases who underwent colonic replacement of the oesophagus during the period 2010 through 2020. The indication for oesophageal replacement was either oesophageal atresia (27 cases) or corrosive strictures (23 cases). Our standard technique was using a middle segment of the colon (transverse colon) based on the left colic vessels (vascular pedicle) after ligation of middle colic vessels. Variations of the middle colic vessels were encountered that included single, double, or absent vessels. In a single case (2%), the middle colic vessels were multiple, short, and non-branching with interrupted continuity of marginal vessels at that point. In the latter situation, we had to use a different technique by fashioning a right colonic flap based on the middle colic vessels. Conclusion In colonic replacement of the oesophagus, preparing a pedicled flap from the transverse colon based on the left colic vessels was almost always feasible owing to the stable collateral marginal vessels. On a rare occasion, the marginal vessels were interrupted by disturbed anatomy of the middle colic vessels when we had to shift to another technique using a right colonic flap.