Congenital vaginal obstructions: Varied presentation and outcome
Congenital vaginal obstructions: Varied presentation and outcome
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DOI：
10.1007/s00383-006-1730-8
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发表时间：
2006-09-01
影响因子：
1.8
通讯作者：
Khan, Zarak
中科院分区：
文献类型：
--
作者：
Nazir, Zafar;Rizvi, Raheela M.;Khan, Zarak
先天性阴道阻塞性病变、子宫阴道积水和阴道积血，在儿童早期和青春期的不同时期出现于不同的内科和外科专业。将18年（1987-2005年）内的26例病例分为三组：A组：新生儿（6例），B组：青少年（18例），C组：成人（2例）。新生儿（A组）的常见表现为腹部肿块（5例）、新生儿败血症（3例）和呼吸窘迫（2例）;而腹痛（18例）、排尿功能障碍（13例）和背痛（7例）在青少年（B组）中较为常见。成人（C组）表现为无法完成和不孕症（2）。4例患者接受了错误治疗;剖腹探查术（1例）和阑尾切除术（3例）。超过50%的病例存在泌尿系统症状和相关的泌尿系统异常，特别是那些有复杂异常的病例。经会阴入路20例，腹会阴入路4例，其中处女膜切除16例，阴道横隔切除8例。尿生殖窦（1例）和泄殖腔畸形（1例）患者在出生时进行初步膀胱造口术和结肠造口术后，在2.5岁时进行分期重建。随访（范围1-15年，平均7年），超过60%的患者有月经不调（11例），子宫内膜异位症（5例）和不孕症（4例）。总之，先天性阴道梗阻的罕见和多变的表现可能导致延误诊断和错误的管理。高度怀疑和横断面成像有助于早期诊断和相关的肾脏异常。一个全面的管理是必不可少的，以保持生殖潜力，因为很大一部分患者可能会遇到性困难，月经不规律，不孕不育。
Congenital obstructing lesions of vagina, hydrometrocolpos, and hematocolpos, present at a variable time during early childhood and adolescence to different medical and surgical specialties. Twenty-six cases presenting over an 18-years period (1987-2005) were divided into three groups; Group A: neonates (6), Group B: adolescents (18), and Group C: adults (2). Common presentations in neonates (Group A) were abdominal mass (5), neonatal sepsis (3), and respiratory distress (2); whereas abdominal pain (18), voiding dysfunctions (13), and backache (7) were prevalent in adolescents (Group B). Adults (Group C) presented with inability to consummate and infertility (2). Four patients received erroneous treatment; exploratory laparotomy (1) and appendectomy (3). Urinary symptoms and associated urinary abnormalities were present in more than 50% of cases, especially those with complex anomalies. Management included excision of imperforate hymen (16) and transverse vaginal septum (8) through perineal (20) and abdominoperineal approach (4). Patients with urogenital sinus (1) and cloacal malformation (1) had staged reconstruction at 2.5 years of age following preliminary vesicostomy and colostomy at birth. On follow up (range 1-15 years; mean 7) more than 60% patients have menstrual irregularity (11), endometriosis (5), and infertility (4). In conclusion, rarity and variable presentation of congenital vaginal obstructions can lead to delayed diagnosis and erroneous management. A high index of suspicion and cross-sectional imaging help in early diagnosis and associated renal anomalies. A comprehensive management is imperative to preserve the reproductive potentials, as significant proportion of patients may experience sexual difficulties, menstrual irregularity, and infertility.