Right heterotaxy with Hirschsprung's disease - A new association

C. R Thambi Dorai, Hamzaini Abdul Hamid, R. Rohana

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

A baby girl with prenatal diagnosis of complex cardiac anomalies and diaphragmatic hernia was born at 36 weeks of gestation. At 4 hr of life, the baby developed respiratory distress and was intubated. She was found to have right hetetrotaxy with total anomalous pulmonary venous drainage into the portal vein, five hepatic veins draining the liver and intrathoracic herniation of the stomach. The child also developed abdominal distension on the second day of life with passage of scanty meconium. The diagnosis of Hirschsprung's disease (HD) was confirmed by histology. HD in association with right heterotaxy has not been reported earlier. The association of heterotaxy with HD in our patient raises a possible genetic link between the two anomalies that needs further research.

Original languageEnglish
Pages (from-to)455-459
Number of pages5
JournalClinical Anatomy
Volume23
Issue number4
DOIs
Publication statusPublished - May 2010

Fingerprint

Hirschsprung Disease
Meconium
Diaphragmatic Hernia
Hepatic Veins
Portal Vein
Prenatal Diagnosis
Drainage
Stomach
Histology
Pregnancy
Lung
Liver
Research

Keywords

  • Asplenia
  • Hetetrotaxy
  • Hirschsprung's disease
  • Intrathoracic stomach

ASJC Scopus subject areas

  • Anatomy
  • Histology

Cite this

Right heterotaxy with Hirschsprung's disease - A new association. / Dorai, C. R Thambi; Abdul Hamid, Hamzaini; Rohana, R.

In: Clinical Anatomy, Vol. 23, No. 4, 05.2010, p. 455-459.

Research output: Contribution to journalArticle

Dorai, C. R Thambi ; Abdul Hamid, Hamzaini ; Rohana, R. / Right heterotaxy with Hirschsprung's disease - A new association. In: Clinical Anatomy. 2010 ; Vol. 23, No. 4. pp. 455-459.
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