Division of Pediatric Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
J Pediatr Surg. 2013 Oct;48(10):2043-6. doi: 10.1016/j.jpedsurg.2013.02.040.
Genetically female cloacal exstrophy (46XX CE) patients develop complications later in life due to their abnormal uterine anatomy, resulting in various invasive gynecologic procedures. Furthermore, they have difficulty becoming pregnant, and if they do conceive, they are unlikely to carry the pregnancy to term. We performed this review to determine the rate of gynecological complications, the fate of the uterus, and the rate of pregnancy in 46XX cloacal exstrophy patients.
All charts for 46XX CE patients treated by us were reviewed following IRB approval. Patient age at last follow-up, surgical management of the uterus, uterine complications, and pregnancies were recorded.
The charts of all 16 of our 46XX CE patients who survived past the neonatal period were reviewed. Two patients underwent hemi-hysterectomy (HH): 1 for an atretic hemi-uterus at birth, another for abnormal uterine insertion at 3 years. A third patient initially had HH for hydrometrocolpos leading to ureteral and colonic obstruction at 14 years but she required a completion hysterectomy a year later. Four patients underwent total hysterectomy (TH) at the outset: 2 neonates for a diminutive uterus with extreme disparity in the halves, another for uterine prolapse at 1 month of age, and a fourth for hematometrocolpos at 16 years of age. Six patients reached adulthood without requiring gynecologic intervention; one of these six is now being managed at another institution. Two patients are prepubertal and one was lost to follow-up. The only patient in the series who became pregnant miscarried at 11 weeks gestation.
Out of 13 post-pubertal patients 6 have retained the entire uterus and another 2 had a hemi-hysterectomy. One patient who became pregnant miscarried at 11 weeks gestation. We believe it is appropriate to avoid ablative genital surgery as far as possible and for these patients to become pregnant after detailed discussion with physicians experienced in the care of 46XX CE patients.
患有 46XX 型先天性泄殖腔外翻（CE）的女性患者由于其子宫解剖结构异常，在生命后期会出现各种侵袭性妇科并发症，需要进行各种侵入性妇科手术。此外，她们怀孕也较为困难，即使怀孕，也不太可能足月分娩。我们进行这项回顾性研究，旨在确定 46XX 型先天性泄殖腔外翻患者的妇科并发症发生率、子宫结局和妊娠率。
在获得机构审查委员会批准后，我们对所有由我们治疗的 46XX 型 CE 患者的病历进行了回顾性分析。记录患者的末次随访年龄、子宫的手术处理、子宫并发症和妊娠情况。
对所有 16 例在新生儿期后存活的 46XX 型 CE 患者的病历进行了回顾性分析。2 例患者接受了半子宫切除术（HH）：1 例患者因出生时半子宫闭锁，另 1 例患者因子宫插入异常而在 3 岁时接受了 HH。第 3 例患者最初因 Hydrometrocolpos 接受 HH，导致 14 岁时输尿管和结肠梗阻，1 年后需要行全子宫切除术。4 例患者在发病时接受了全子宫切除术（TH）：2 例新生儿因两半子宫大小差异悬殊，1 例因 1 个月大时子宫脱垂，另 1 例因 16 岁时的血肿性子宫阴道积血接受 TH。6 例患者成年后无需妇科干预；其中 1 例在另一家机构接受治疗。2 例患者为青春期前，1 例失访。该系列中唯一怀孕的患者在 11 周妊娠时流产。
在 13 例青春期后患者中，6 例保留了整个子宫，另外 2 例接受了半子宫切除术。1 例患者怀孕后流产，发生在 11 周妊娠时。我们认为，应尽可能避免生殖器切除术，并在与有 46XX 型 CE 患者治疗经验的医生进行详细讨论后，让这些患者怀孕。
