Rintala R J, Mildh L, Lindahl H
Children's Hospital, University of Helsinki, Finland.
J Pediatr Surg. 1996 Apr;31(4):559-62. doi: 10.1016/s0022-3468(96)90496-0.
To establish the incidence of H-type anorectal malformations (ARM), the hospital records of 629 patients with ARM were studied. Patients who had a congenital rectourogenital connection and an external anal opening in a normal or ectopic position were considered to have an H-type malformation. Twenty (3.2%; 14 females, 6 males) of the 629 patients studied had an H-type ARM. Major associated anomalies were found in 60% of these patients. Thirteen patients (12 females, 1 male) had a normally placed anus; the H fistula was low rectovestibular in 10, rectovaginal in 2, and rectourethral in 1. Seven patients had an ectopic anal opening. Three males had a rectourethral H fistula; in two of these there was a double fistula. Two males had a rectovesical H fistula. Of the two females with ectopic anal openings, one had a high rectovaginal H fistula and the other had a low fistula. In only one case was the diagnosis of H fistula made immediately after birth. Three patients died of severe cardiac abnormalities during the neonatal period or early infancy. Primary perineal repair or a limited posterior surgical anorectoplasty (PSARP) was used in 15 cases; formal PSARP was used for the other two. Four patients had between one and 4 recurrences; all but one of these fistulas were repaired subsequently. Long term (median follow-up period, 12 years; range, 4 to 38 years), 12 patients had good bowel function and no faecal soiling; four of these had constipation that was manageable with laxatives. Two patients (aged 10 and 12 years) had daily soiling. Two others were too young to evaluate, and one (with severe mental retardation) has a permanent colostomy.
为确定H型肛门直肠畸形（ARM）的发病率，对629例ARM患者的医院记录进行了研究。具有先天性直肠泌尿连接且肛门外口位置正常或异位的患者被认为患有H型畸形。在研究的629例患者中，有20例（3.2%；14例女性，6例男性）患有H型ARM。这些患者中有60%发现有主要相关异常。13例患者（12例女性，1例男性）肛门位置正常；其中10例H瘘为低位直肠前庭瘘，2例为直肠阴道瘘，1例为直肠尿道瘘。7例患者肛门异位开口。3例男性有直肠尿道H瘘；其中2例有双瘘。2例男性有直肠膀胱H瘘。在2例肛门异位开口的女性中，1例有高位直肠阴道H瘘，另1例有低位瘘。仅1例在出生后立即诊断为H瘘。3例患者在新生儿期或婴儿早期因严重心脏异常死亡。15例采用一期会阴修补术或有限的后路手术肛门直肠成形术（PSARP）；另外2例采用正规的PSARP。4例患者有1至4次复发；除1例瘘外，其余瘘均随后修复。长期（中位随访期12年；范围4至38年），12例患者肠道功能良好，无粪便污染；其中4例有便秘，使用泻药可控制。2例患者（年龄分别为10岁和12岁）每天有粪便污染。另外2例年龄太小无法评估，1例（重度智力障碍）有永久性结肠造口术。
