Descriptive Epidemiology of Anorectal Malformations in Chinese Population

• Main Authors: Christoph Heinrich Houben (Author), Kin Wai Edwin Chan (Author), Kit Yi Kristine Pang (Author), Xiang-Nan Feng (Author), Wai Cheung Jennifer Mou (Author), Yuk Him Tam (Author), Kim Hung Lee (Author)
• Format: Book
• Published: EL-Med-Pub, 2017-10-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.
• Notes: 10.21699/jns.v6i4.531; 2226-0439
• Keywords: Anorectal malformation; Perineal fistula; Rectourethral fistula; VACTERL association; Down Syndrome; Krickenbeck classification; Pediatrics; RJ1-570; Surgery; RD1-811
• Source: Journal of Neonatal Surgery, Vol 6, Iss 4 (2017)

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.