The spectrum of surgical jaundice in infancy

doi:10.1016/S0022-3468(03)00498-6

Journal of Pediatric Surgery

Volume 38, Issue 10, October 2003, Pages 1471-1479

https://doi.org/10.1016/S0022-3468(03)00498-6 Get rights and content

Abstract

Background

Conjugated jaundice arising during infancy may be caused by a number of different surgical conditions. The aim of this study was to compare clinical features, management, and outcome of all types of surgical jaundice presenting in the first year of life.

Methods

A retrospective review was conducted of all infants born in the United Kingdom with jaundice caused by a surgical cause referred to the authors’ institution from January 1992 to December 1999.

Results

There were 171 infants who could be separated into 3 specific groups: biliary atresia (BA, n = 137), inspissated bile syndrome (IBS; n = 14), and choledochal malformation (CM; n = 12) together with a group containing various miscellaneous conditions (n = 8). Infants with BA had higher bilirubin (P < .01) and aspartate aminotransferase levels (P < .001) and came to surgery earlier (P < .01) than infants with either IBS or CM. Infants with IBS and CM were more likely to be premature and have other malformations, respectively. Ultrasound scan was the principal investigation in the differentiation of BA from other causes of jaundice. Accurate prelaparotomy diagnosis was made by percutaneous liver biopsy in 87% of cases later shown to be BA. Currently, 88 (64%) of children with BA are alive with their native liver postportoenterostomy, 4 have died, and 45 have undergone liver transplantation (with 1 death postoperatively). A policy of primary portoenterostomy for BA followed by transplantation, if necessary, resulted in a survival rate of over 95%. All children in the other diagnostic groups are alive and anicteric after appropriate surgical intervention.

Conclusions

Approximately 80% of infants presenting with surgical jaundice have biliary atresia, whereas those with inspissated bile syndrome and choledochal malformations make up most of the remainder. Mortality in this age-group is confined to infants with BA, but even on these infants an overall survival rate of greater than 95% is currently expected.

Section snippets

Materials and methods

King’s College Hospital (KCH), London, is a tertiary referral unit for pediatric hepatobiliary disease predominantly, but not exclusively, from the South of England. A retrospective review of all medical records of infants who presented to our institution over the 8-year period, January 1992 to December 1999, was carried out. The criteria for inclusion into the study were (1) origin from within the United Kingdom, (2) jaundice presenting within the first year of life, and (3) confirmation of a

Results

During the 8-year study period, 682 infants were referred to the pediatric hepatobiliary unit for investigation of jaundice. Within this group were 171 (25%) infants, born within the United Kingdom, where a “surgical” cause for jaundice was identified (Fig 1). Those infants were divided into 4 etiological groups for ease of comparison: (A) biliary atresia, (B) inspissated bile syndrome, (C) choledochal malformation, and (D) miscellaneous.

Discussion

The range of possible causes of surgical jaundice in infancy is well illustrated in this study with at least 8 different diagnoses. The most frequent diagnosis remains biliary atresia, accounting for about 80% of total cases. The incidence of other conditions can now be estimated if we assume that no selection or retention of cases occurred before referral. The calculated incidence of biliary atresia in the United Kingdom during the 1990s was 1 in 16,700 live births5 which suggests that the

Acknowledgements

The contribution of the whole of the multidisciplinary unit is acknowledged but particularly Dr P. Kane and Dr J. Karani (radiology); Professor B. Portmann (histopathology); Drs A. Baker, N. Hadzic, and A. Dhawan (pediatric hepatology); and N. Heaton and M. Rela (transplant surgery).

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Citation Excerpt :
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The spectrum of surgical jaundice in infancy

Abstract

Background

Methods

Results

Conclusions

Section snippets

Materials and methods

Results

Discussion

Acknowledgements

Lancet

J Pediatr Surg

Hepatology

J Pediatr Surg

Am J Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

Clinical study of prolonged jaundice in breast and bottle-fed babies

Arch Dis Child

Investigation and management of neonatal jaundiceA problem-orientated case record

Arch Dis Child

Hepatitis syndrome in infancy—An epidemiological survey with 10 year follow-up

Arch Dis Child

Jaundice in babiesImplications for community screening for biliary atresia

Br Med J