Clinical Review

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Clinical Review Clinical review Recent advances Paediatric surgery Paul D Losty Department of Introduction Paediatric Surgery, Institute of Child Paediatric surgery—the surgical care of children from Summary points Health, Alder Hey Children’s Hospital, the fetus to adolescent—is a comparatively new surgical Liverpool L12 2AP specialty, which began shortly after the second world Exploring mechanisms to modulate abnormal Paul D Losty, war.12 Pioneering work during the formative years of fetal development pharmacologically may provide senior lecturer the specialty has enabled many lethal congenital alternatives to fetal surgery and invasive fetal Paul.Losty@rlchtr. malformations to be corrected. The surgical treatment interventions nwest.nhs.uk of childhood disease has also progressed enormously. Progress in neonatal surgery has resulted in the BMJ 1999;318:1668–72 This review focuses on recent advances in paediatric surgery and its relation with scientific research, which is survival of children with many types of congenital rapidly translating state of the art care from research to lethal malformations the patient. Advances in paediatric gastroenterology include a greater understanding of biological mechanisms Methods regulating normal and abnormal gut motility This article is based on a personal awareness of devel- New treatments are needed to induce biological opments in paediatric surgery and relevant contribu- regression of tumours and tumour maturation tions from major paediatric surgical journals, review articles, and the proceedings from meetings of various Several laparoscopic techniques are now feasible, international associations. safe, and effective Fetal surgery and interventions Future developments include tissue engineering, antiangiogenic agents, scarless healing, in utero The close liaison between paediatric surgeons and col- gene replacement, and treatments with growth leagues in fetomaternal medicine enabled fetal defects factors to be diagnosed with ultrasonography and the clinical course and pathophysiology of many of these disorders to be observed (box). surgery immediately after birth can be effectively The antenatal detection of a fetal structural abnor- planned and a good outcome anticipated (fig 1). Some mality often entails referral to a paediatric surgeon, abnormalities, however, may result in the death of the who can counsel parents and discuss the likely fetus or neonate. In such cases fetal surgery or outcomes for the fetus. For some structural abnormali- intervention may be required.3 This has led to the ties, such as the abdominal wall defect, gastroschisis, emergence of fetal surgery programmes, with a few centres mainly concentrated in North America.3 Abnormalities considered amenable to surgery in Fetal malformations detected by antenatal utero include benign forms of cystic lung lesions, ultrasonography which may lead to hydrops. Fetal lung resection is Spina bifida and hydrocephalus technically feasible, with reasonable outcome for high 3 Gastroschisis risk cases treated at specialist centres. Oesophageal atresia In fetuses affected by moderate to severe maternal Exomphalos oligohydramnios associated with reduced amniotic Cystic lung lesions fluid volume and obstruction of the urinary tract in Renal tract disease males, intervention can protect renal function and Congenital diaphragmatic hernia 3 Bladder exstrophy maintain lung growth. In such cases, usually Cardiac defects secondary to posterior urethral valves, lung hypoplasia Cloacal exstrophy can be avoided by bladder decompression using ultra- Duodenal atresia sound guided percutaneous catheters to divert urine from the fetal bladder to the amniotic cavity 1668 BMJ VOLUME 318 19 JUNE 1999 www.bmj.com Clinical review (vesicoamnioticshunting) or at open fetal surgery to create a vesicostomy.3 Attention has also focused recently on the potential role of fetal surgery in spina bifida (1 in 2000 live births), a disability causing lower limb paralysis, skeletal deformity, incontinence, hydrocephalus, and mental impairment.4 Exposure of fetal neural tissue to the effects of chemicals in the amniotic fluid, and mechani- cal injury during birth and vaginal delivery, may cause deterioration in neurological function. In an effort to ameliorate ongoing neurological damage, experimen- tal work in fetal sheep has confirmed that repair of the spina bifida defect in utero stops spinal cord destruction and restores normal neurological function at birth. This work has led to the first successful repair of an extensive thoracolumbar open spina bifida lesion in utero associated with hydrocephalus in a human 4 fetus. Although fetal surgery for spina bifida is at an Fig 1 Gastroschisis defect in neonate experimental stage, such pioneering advances merit careful consideration. Neonatal surgery Other fetal structural abnormalities have proved more difficult to treat—for example, congenital Neonatal surgery has undergone many major develop- diaphragmatic hernia, which occurs in 1 in 2500 live ments.213Oesophageal atresia (1 in 5000 live births) is births (fig 2). Despite diagnosis by antenatal ultra- most commonly characterised by the presence of a sonography, innovations in intensive care, and the sim- blind ending upper oesophagus and a fistula between ple nature of the defect, 40%-50% of cases die.5 This the lower oesophagus and airway. Such babies are high mortality is due to a primary abnormality in lung unable to swallow and classically present with excessive development and the prolapse of abdominal organs salivation, and choking episodes or respiratory distress into the chest, which severely further compromises on feeding due to pulmonary aspiration (fig 3) Survival fetal lung growth and development. Fetal surgery has from this condition remained poor until the early been restricted to fetuses with a poor outcome. 1960s, and neonates weighing as little as 1500 g Selection criteria now include early antenatal diagnosis currently have a predicted survival of 97% in specialist (less than 25 weeks of gestation), isolated congenital centres.14 diaphragmatic hernia defect with liver herniated into Hirschsprung’s disease (1 in 5000 live births) is the the chest, and fetuses without chromosomal abnor- commonest cause of intestinal obstruction in neonates malities.6 Two decades of experimental work have now (fig 4). Infants typically present with bile stained vomi- refined the surgical procedure to exploit mechanisms tus after initiation of feeds, abdominal distension, and that promote normal lung growth.78 The seminal delayed passage of meconium stool beyond 24 hours observation that congenital laryngeal obstruction in an after delivery. Fewer babies are now presenting beyond infant with an absence of both kidneys and Fraser’s the neonatal period owing to an increased awareness syndrome resulted in large lungs stimulated research of this disorder: the gold standard for diagnosis is a into “plugging” the trachea to encourage lung rectal biopsy that shows an absence of ganglion cells. growth.910 This procedure—now termed PLUG (plug Traditionally, a “levelling” colostomy was performed in the lung until it grows)—is performed in some centres by open fetal surgery.10 Advances in minimally invasive surgery with a laparoscope, coupled with attempts to override the problem of preterm labour, have further led to tracheal plugging using video fetoscopic technology (FETENDO) to visualise, access, and occlude the fetal airway.6 Six of eight (75%) fetuses with congenital diaphragmatic hernia have survived after surgery using video fetoscopic technology. Less invasive strategies are being explored to stimu- late fetal lung development pharmacologically com- bined with postnatal repair of the diaphragmatic hernia defect.11 This is based on the observation that the lungs of babies with severe congenital diaphragmatic hernia resemble the immature lungs of premature newborn babies with respiratory distress syndrome.11 In experi- mental models of congenital diaphragmatic hernia, antenatal steroid treatment, widely used to stimulate lung maturation in premature babies, has been shown to reverse immature lung growth at the biochemical, mor- 11 12 phological, functional, and molecular level. An inter- Fig 2 Chest x ray film of neonate with congenital diaphragmatic national collaborative clinical trial is currently evaluating hernia. Note presence of air filled intestinal loops in thoracic cavity its efficacy in humans. and shift of mediastinum and heart BMJ VOLUME 318 19 JUNE 1999 www.bmj.com 1669 Clinical review to appreciate the significance of normal and abnormal anorectal anatomy,17 and PSARP (posterior sagittal anorectoplasty) has significantly improved the quality of life, continence, and functional outcome for affected children. This operation comprises an initial colostomy then a major anorectal reconstruction in the first year of life. Although Goon previously studied the posterior sagittal anorectoplasty procedure as a primary operation in neonates with imperforate anus, few surgeons considered it of practical advantage to the infant.18 Albanese and colleagues have recently evaluated the operation as a single stage procedure, thereby avoiding colostomy in the newborn period, and outcome data are encouraging in the short term (CT Albanese et al, report of a meeting on one stage correction of high imperforate anus in the male neonate, Surgical Section of the American Academy of Pediatrics, San Francisco, 1998). Surgical gastroenterology Paediatric surgical gastroenterology is evolving
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