532 Dodd

DS. 3 Behrman E, Vaughan VC, eds. Jaundice and hyperbilrubinemia in the new- 17 Pearlman MA, Gartner LM, Lee K-S, Morecki R, Horoupian Arch Dis Child: first published as 10.1136/adc.68.5_Spec_No.532 on 1 May 1993. Downloaded from born. Nelson textbook of pediatics. 13th Ed. Philadelphia: WB Saunders, Absence of kernicterus in low-birth-weight infants from 1971 through 1987: 405-7. 1976: comparison with findings in 1966 and 1967. Pediatrics 1978; 62: 4 Oski FA. Physiologic jaundice. In: Avery ME, Taeusch HW Jr, eds. Diseases 460-4. ofthe newborn. 5th Ed. Philadelphia: WB Saunders, 1988: 625. 18 Valaes T, Gellis SS. Is kernicterus always the definitive evidence of bilirubin 5 Maisels MJ. Neonatal jaundice. In: Avery GB, ed. Neonataology, patho-phy- toxicity. Pediatrics 1981; 67: 940-1. isology and management ofthe newborn. 3rd Ed. Philadelphia: JB Lippincott, 19 Scheidt PC, Mellits ED, Hardy JB, et al. Toxicity to bilirubin in neonates; 1987: 534-629. infant development during the first year in relation to maximum neonatal 6 Maisels MJ, Gifford K. Norman serum bilirubin levels in the newborn and serum bilirubin concentration. J Pediatr 1977; 91: 292-7. the effect of breast-feeding. Pediatrics 1986; 78: 837-43. 20 Naeye RL. Amniotic fluid infections, neonatal hyperbilirubinemia and 7 Newman TB, Easterling MJ, Goldman ES, Stevenson DK. Laboratory eval- psychomotor impairment. Pediatrics 1978; 62: 497-503. uation of jaundiced newborns: frequency, cost and yield. Am J Dis Child 21 Corchia C, Ruiu M, Orzalesi M. Breast-feeding and hyperbilirubinema in 1990; 144: 364-8. full-term newborn infants. Pediatrics 1985; 75: 617-8. 8 Osborn LM, Lenarsky C, Oakes RC, Reiff MI. Phototherapy in full-term 22 Chukwuma GN, Van Aerde J, Boyden M, Perlman M. Changes in auditory infants with hemolytic disease secondary to ABO incompatibility. Pediatrics brainstem responses in hyperbilirubinemic infants before and after 1984; 74: 371-4. exchange transfusion. Pediatrics 1984; 74: 800-3. 9 Quinn MW, Weindling AM, Davidson DC. Does ABO incompatibility 23 Chin KC, Taylor MJ, Perlman M. Improvement in auditory and visual matter? Arch Dis Child 1988; 63: 1258-60. evoked potentials in jaundiced preterm infants after exchange transfusion. 10 Maisels MJ, Kring E. Full-term infants with severe hyperbilirubinemia: do Arch Dis Child 1985; 60: 714-7. they need a septic workup? Pediatr Res 1991; 29: 224A. 24 Schedit PC, Bryla DA, Nelson KB, Hirtz DG, Hoffman, HJ. Phototherapy 11 Hsai DY-Y, Allen FH, Gellis SS, Diamond LK. Serum bilirubin in relation for neonatal hyperbilirubinemia; six-year follow-up of the National to kernicterus. NEnglJ Med 1952; 247: 668-7 1. Institute of Child Health and Human Development clinical trial. Pediatrics 12 Mores A. Fargasova I, Minarikova E. The relation of hyperbilirubinemia in 1990; 85: 455-63. newborns without isoimmunization to kernicterus. Acta Paediatr Scand Dis 1959;48: 590-602. 25 Moseley MJ, Fielder AR. Phototherapy: an ocular hazard revisited. Arch 13 Killander A, Michaelsson M, Muller-Eberhard E, et al. Hyperbilirubinemia Child 1988; 63: 886-7. in full term newborn infants: a follow-up study. Acta Paediatr Scand 1963; 26 Finlay HVL, Tucker SM. Neonatal plasma bilirubin chart. Arch Dis Child 52: 481-4. 1978; 53: 90-1. 14 Wishingrad L, Cornblath M, Takakuwa P, et al. Studies of non-haemolytic 27 Lau SP, Fung KP. Serum bilirubin kinetics in intermittent phototherapy of hyperbilirubinemia in premature infants: prospective randomized selection physiological jaundice. Arch Dis Child 1984; 59: 892-4. for exchange transfusion with observation on the levels of serum bilirubin 28 Hussein M, Howard ER, Mieli-Vergani G, Mowat AP. Jaundice at 14 days with and without exchange transfusion and neurologic evaluations one year of age: exclude biliary atresia. Arch Dis Child 1991; 66: 1177-9. after birth. Pediatrics 1965; 36: 162-72. 29 Winfield CR, MacFaul R. Clinical study ofprolonged jaundice in breast- and 15 Crosse VM, Meyer TC, Gerrard JW. Kernicterus and prematurity. Arch Dis bottle-fed babies. Arch Dis Child 1978; 53: 506 -7. Child 1955; 30: 501-8. 30 Osborn LM, Reiff MI, Bolus R. Jaundice in the full-terrn neonate. Pediatrics 16 Harris RC, Lucey JF, MacLean JR. Kernicterus in premature infants associ- 1984; 73: 520-5. ated with low concentrations ofbilirubin in the plasma. Pediatrics 1958; 21: 31 Beeken S, Waterston T. Health service support of breast feeding - are we 875-83. practising what we preach? BMJ 1992; 305: 285-7.

Laryngo-tracheo-oesophageal cleft

A cleft is a rare but significant cause of congenital from defects in cricoid chondrification or fusion (types 1 respiratory distress and aspiration. Management of this and 2) and or failure of fusion of the laryngotracheal defect is complex and despite recent advances the overall septum (types 3 and 4). This embryological difference is mortality remains unacceptably high. Although over 179 highlighted by the occurrence of isolated tracheo- cases have been reported to date there continues to be oesophageal fistula that occurs in the absence of any laryn- significant delay in diagnosis, perhaps due to a reluctance to geal abnormality. The exact cause of the disorder is http://adc.bmj.com/ perform neonatal , and appropriate inter- unknown but substance abuse in the first trimester and vention which contributes to increased morbidity and neural crest abnormalities have been cited as potential mortality. However these patients often have other signifi- factors.2 cant abnormalities which may independently affect their The result is an abnormal communication between the outcome. Of these gastro-oesophageal reflux, microgastria, larynx, , and oesophagus. The infant is thus at risk ventricular septal defect, bronchial and tracheal stenosis are from repeated aspiration offood and saliva, recurrent pneu- among the most clinically significant. monia and respiratory distress, although minor type 1 clefts on September 25, 2021 by guest. Protected copyright. The approximate incidence is one in 10 000 to 20 000 may be asymptomatic. A laryngeal cleft should be con- live births, although the true incidence may be higher.' sidered in any infant who develops aspiration and cyanosis There is no consistent pattern of inheritance but males are after feeding and respiratory distress, although other causes affected more commonly than females and sporadic famil- such as choanal atresia, tracheo-oesophageal fistula, ial occurrences have been described. Laryngeal clefts have tracheomalacia, and laryngopharyngeal dysmotility should also been reported with the G syndrome (X linked or auto- also be considered. Typically, cleft patients are said to somal dominant) and in the Pallister-Hall syndrome. Clefts present with a classical triad of increased salivation, stridor, are classified into four types based on the length of involve- and a low soundless cry.1 However this triad is rare and ment of the trachea. The more extensive the defect the most infants develop aspiration and cyanosis after feeding greater the potential for fatal pulmonary complications and and this combined with postpartum respiratory distress poor outcome. The defect extends to the cricoid in type 1, should stimulate urgent bronchoscopy. The cry is usually involves the cricoid and/or proximal trachea in type 2, harsh and not silent and stridor is uncommon. Secretions extends to the carina in type 3, and into one or both main- are increased and there is significant difficulty with feeding. stem bronchi in type 4. Fortunately types 1 and 2 cleft Contrast radiography may show the abnormal communica- constitute the most common abnormality seen. tion but this is often difficult to distinguish from simple spill A laryngeal cleft arises from abnormal separation of the over. A lateral neck x ray film may show an anteriorly dis- larynx-trachea and the oesophagus. At 25 days the laryngo- placed nasogastric tube or a posteriorly displaced endotra- tracheal septum develops and begins to fuse in a cephalad cheal tube. Successful management of these infants direction and so separate the distal trachea from the devel- requires a low threshold for bronchoscopy and thus early oping oesophagus. At the same time the diagnosis, a secure and stable airway, and provision of develops as two lateral centres of chondrification from the adequate nutrition. Most require a tracheostomy and sixth branchial arch. Dorsal fusion of the cricoid plate is gastrostomy at the outset. A fundoplication may be complete by day 50-54 and laryngeal muscular develop- required if there is troublesome gastro-oesophageal reflux. ment then ensues. The range of abnormalities seen results Other procedures such as gastric transection are not Laryngo-tracheo-oesophageal cleft 533 routinely required since the availability of total parenteral the airway during and after surgery. Further surgery is Arch Dis Child: first published as 10.1136/adc.68.5_Spec_No.532 on 1 May 1993. Downloaded from nutrition. Alternatively a feeding jejunostomy or trans- often required to correct gastro-oesophageal reflux, pyloric jejunal tube may be used. There is considerable , and microgastria. Recurrent controversy over the timing of surgical intervention. Should tracheo-oesophageal fistula remains a problem and wound these patients undergo early single staged correction or is it breakdown may occur in as many as 50% of patients.3 appropriate to plan a staged procedure with an initial Interposition of a vascularised muscle flap may help to tracheostomy, gastrostomy, and fundoplication followed by prevent breakdown. delayed repair? If the diagnosis can be made shortly after Overall survival with aggressive treatment is 70% but is birth and the patient is stable then early repair should be significantly less for those patients with extensive type 4 considered; however many infants experience a delay in defects and severe associated anomalies. While noteworthy diagnosis and require considerable respiratory and nutri- advances have been made in surgical and anaesthetic care tional treatment before correction. Early repair reduces the there remains an unwillingness to refer neonates for diag- severity of associated pulmonary dysfunction and facilitates nostic endoscopy. A lower threshold for intervention postoperative weaning from the ventilator. Conversely coupled with further improvements in surgical technique delayed repair facilitates weight gain and may make surgical especially in the treatment of microgastria and tracheo- correction easier. Whatever the timing of surgery it is essen- bronchial stenosis may further improve survival. tial that it be performed in a centre with paediatric surgical expertise. MARTIN T CORBALLY Type 1 defects may be corrected endoscopically if not Department ofPaediatric Surgery, Our Lady's Hospitalfor Sick Children, responding to standard antireflux treatment. There is no Crumlin, Dublin 12, consensus as to the appropriate surgical approach for the Republic ofIreland remaining type 2, 3, and 4 clefts. An anterior approach may exacerbate coexistent laryngeal weakness and may be diffi- 1 Roth B, Rose KG, Benz-Bohm G, Gunther H. Laryngo-tracheo-oesophageal cult in the presence of a tracheostomy, whereas a lateral cleft: clinical features, diagnosis and therapy. EurJ Pediatr 1983; 140: 41-6. 2 Ryan DP, Muehrcke DD, Doody P, Kim SH, Donahoe PK. Laryngo-tracheo- approach may potentially injure the recurrent laryngeal esophageal cleft (type IV): management and repair of lesions beyond the nerve. Correction of type 4 defects is more difficult and carina.J PediatrSurg 1991; 26: 962-70. 3 DuBois nJ, Pokerny WJ, Harberg FJ, Smith RJH. Current management of requires a cervical-thoracic approach in addition to laryngeal and laryngo-tracheo-esophageal clefts. J Pediatr Surg 1990; 25: innovative anaesthetic techniques to maintain and secure 855-60. http://adc.bmj.com/ on September 25, 2021 by guest. Protected copyright.