Descriptive Epidemiology of Anorectal Malformations in Chinese Population

Christoph Heinrich Houben, Kin Wai Edwin Chan, Kit Yi Kristine Pang, Xiang-Nan Feng, Wai Cheung Jennifer Mou, Yuk Him Tam, Kim Hung Lee


Over a twelve year period one hundred and five neonates (30 females) with an anorectal malformation (ARM) were studied at a tertiary referral centre in Hong Kong. A perineal fistula was the most frequent presentation of ARM in males closely followed by rectourethral fistula. Vestibular fistula was the most common anomaly amongst females. An ARM without a fistula was documented in 11 % of patients and dominated by males. Associations and syndromes were present in nearly 30 % of the group with VACTERL association and Down syndrome at the forefront. Isolated anomalies were most frequently seen in the renal tract (23 %), the CNS (20 %) and the heart (18 %). A quarter of the cohort was small for gestational age with syndromes/associations featuring in half of this subgroup. A death rate of 9.5 % was recorded, of which all except one had an underlying syndrome or association. In addition a pre-existing heart condition constituted the most significant factor affecting the mortality in 50 % of this group.


Anorectal malformation; Perineal fistula; Rectourethral fistula; VACTERL association; Down Syndrome; Krickenbeck classification

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