处女膜修补手术及麻醉方法的改进

目的；增大处女膜粘膜瓣的接触面，减少因麻醉造成的处女膜粘膜水肿，提高修复手术的成功率。方法：手术全部采用1％的卡因行粘膜表面麻醉。53例采用瓦合粘膜瓣法，3例用瓦合粘膜瓣联合阴道粘膜瓣修复法。结果：56例术后一月随访，53例处女膜孔径为一指，成功率94．64％。结论：采用1％的卡因粘膜表面麻醉，瓦合粘膜瓣法及瓦合粘膜联合阴道粘膜瓣修复法对处女膜修复是行之有效的。     

无孔处女膜临床分析

目的 分析总结无孔处女膜的诊断要点，提高诊断率。方法 回顾性分析我院近10余收治的5例无孔处女膜的临床资料。结果 5例患均无月经初潮，仅有程度不同的下腹疼痛，尤其是周期性的下腹疼痛。3例表现为急性下腹痛并急性尿潴留。2例误诊为卵巢囊肿、卵巢畸胎瘤。结论 少女己进入月经来潮年龄，但无月经初潮，仅有程度不同的下腹疼痛，尤其是有周期性的下腹疼痛，应想到无孔处女膜的可能。肛诊触及盆腔囊性包块，不活动；B超探查子宫与附件形态正常，相当于阴道的解剖部位有液性暗区，提示经血潴留，有助于本病的诊断。     

FG syndrome in a premature male

A 30-week premature male infant is presented with dolichocephaly, frontal bossing, down-slanting palpebral fissures, hypertelorism, long philtrum, micrognathia, cleft palate, and imperforate anus. He is the fifth patient to be presented with FG syndrome and sensorineural deafness. The patient's syndromic manifestations became more obvious during an inpatient observation period of 3 months.     

Lower mesodermal defects: A common cause of fetal and early neonatal death

Among the first 1,130 referrals to the Wisconsin Stillbirth Service Program 17 infants have been recognized to share phenotypic characteristics involving the genital, urinary, lower gastrointestinal, and axial skeletal systems. The pattern of abnormalities identified appears to be limited to structures sharing a common embryologic origin. These features, for the most part, are shown to be non-randomly associated. No clearly definable sub-groups within this population are demonstrable. The pattern of abnormalities is defined to include abnormalities of the following structures as pathogenetically primary features: lumbosacral vertebrae, kidneys, ureters, uterus/fallopian tubes, vagina, bladder, urethra, adrenals, gonads, anorectum, external genitalia, and umbilical arteries. An embryologic mechanism is proposed which explains this non-random association as arising secondary to disruption of structures derived from the lower portion of the primitive intra-embryonic mesoderm. The Lower Mesodermal Defects Sequence appears to be a rather common (and under-recognized) cause of stillbirth and immediate neonatal death. © 1994 Wiley-Liss, Inc.     

An Unusual Cause of Anuria and Hydronephrosis in a 12-Year-Old Girl

Abstract

A 12-year-old girl was admitted for abdominal pain and signs of acute kidney injury. Physical examination showed abdominal distension and a tumefaction in the lower abdomen. Laboratory and clinical findings were consistent with acute kidney injury. Abdominal ultrasonography showed an oval mass, which corresponded with hematometrocolpos, and right-sided hydronephrosis. Catheterization followed by cruciate hymenectomy was performed. The patient recovered completely. Imperforate hymen is an obstructive anomaly of the female reproductive tract of unknown etiology that can cause a variety of symptoms. It is a rare, but possible cause of acute kidney injury. Patient history data and genital examination findings are sufficient to establish the diagnosis. Abdominal ultrasonography is the most useful diagnostic modality.     

Mimics of malignancy caused by concurrent imperforate hymen and transverse vaginal septum: an instructive case and review of the literature

The coexistence of imperforate hymen and vaginal septum is rare and their ability to mimic malignant manifestations have not been frequently reported. This current case report describes a 13-year-old girl that presented with cyclic abdominal pain for 6 months. She was found to have a huge mass via abdominal plain film X-ray and sonography, with inexplicably high levels of serum carcinoembryonic antigen, cancer antigen (CA)-19-9 and CA-125. Pelvic computed tomography imaging disclosed two huge cystic lesions in the uterine and upper vaginal cavities. Surgical intervention conformed the diagnosis of a concurrent imperforate hymen and transverse vaginal septum, echoing the imaging findings of haematocolpometra. Her tumour marker levels gradually returned to normal after surgery. This rare case of concomitant imperforate hymen and transverse vaginal septum highlights that haematocolpometra, a benign disease that might mimic malignancy, should be taken into consideration in any adolescent females with an abdominal mass and amenorrhoea to ensure an early diagnosis and timely appropriate management.     

Multicenter retrospective comparative study of laparoscopically assisted and conventional anorectoplasty for male infants with rectoprostatic urethral fistula

Background

Laparoscopically assisted anorectoplasty (LAARP) was expected to achieve better fecal continence than conventional procedures. However, the issue of which approach is better remains controversial. We compared outcomes between the conventional procedure and LAARP in male infants with rectoprostatic urethral fistula.

Methods

Institutes belonging to the Japanese Study Group of Anorectal Anomalies (JSGA) were invited to participate. Subjects were male infants with rectoprostatic urethral fistula treated by the conventional approach (abdominoperineal pull-through and PSARP) or LAARP between 2000 and 2006. Medical charts and operative records were reviewed retrospectively.

Results

Eighty-one patients (conventional: 36, LAARP: 45) were enrolled from 15 centers. In both groups, the mean Kelly score was 5. The total score of the scoring system was newly developed by the Japanese Study Group of Anorectal Anomalies. Follow-up Project (5–15 points) was 10.7 and 12.1 in the conventional group and the LAARP group, respectively (p = 0.07). The incidence of failed rectoanal anastomosis, mucosal prolapse, and anal stenosis was comparable in both groups. Posterior urethral diverticula were detected on cystourethrograms in 7% and 11% (p = 1.0) and on MRI in 0% and 34% (p = 0.02) of the conventional and the LAARP groups, respectively. Overall, 94% of diverticula were asymptomatic.

Conclusions

Fecal continence and complication rates after LAARP were comparable to those observed after the conventional method. Posterior urethral diverticula were detected more frequently after LAARP.     

Unique Case of Imperforate Hymen

BackgroundImperforate hymen typically presents in adolescence with pain, hematocolpometra and primary amenorrhea. This case documents a previously unreported etiology for an atypical presentation with a history of recent menstruation.CaseA female adolescent presented with symptoms of urinary retention and leg pain. She reported a history of irregular, painful menses. Clinical examination revealed a pelvic mass and imperforate hymen. Sonography was consistent with hematocolpometra. Before a planned hymenectomy, the patient began to pass dark blood through a fistulous opening in her vulva. Hymenectomy resulted in complete resolution of the pain and hematocolpometra.Summary and ConclusionIdentification of the fistulous tract explained the patient's history of menstrual bleeding despite an imperforate hymen. Spontaneous rupture of hematocolpometra through a fistulous tract to the vulva is a previously unreported atypical presentation of imperforate hymen in a “menstruating” adolescent with pain and a pelvic mass.     

女性生殖道畸形与子宫内膜异位症

女性生殖道畸形的发生是由于性染色体异常或胚胎在发育过程中出现发育停滞或发育异常所致,主要包括阴道和子宫的畸形,常合并有泌尿系统的异常.无孔处女膜、阴道闭锁、阴道横隔等梗阻性生殖道畸形患者由于经血外流受阻,增加了子宫内膜逆流入盆腔种植的机会,发生子宫内膜异住症的几率明显增高.但也不乏残角子宫、单角子宫以及子宫中隔等非梗阻性生殖道畸形发生子宫内膜异位症的报道,其主要发病机制也是经血逆流.生殖道畸形综合征并发子宫内膜异位症的发病机制可能与体腔上皮化生有关.