• Archives of Plastic Surgery
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• 제47권3호
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• pp.272-276
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• 2020

Anorectal malformation or imperforate anus is a congenital anomaly of rectum and anus. Mullerian duct anomalies are abnormal development of uterus, cervix, and vagina. Imperforate anus with double uterus is extremely rare and cannot explain by normal embryologic development. Moreover, guideline in treatment is inconclusive. We report an extremely rare case of a young adult female who presented with recurrent pelvic inflammatory disease caused by rectovaginal fistula in congenital imperforate anus and didelphys uterus, and successfully neoanal reconstruction with gracilis muscle flap. Aims for treatment are closed rectovaginal fistula, and anal sphincter reconstruction. To our best knowledge, the imperforate anus with double uterus is extremely rare anomaly. Furthermore, successfully anal sphincter reconstruction with functional gracilis muscle in the imperforate anus with double uterus has never been reported in English literature.

• Childhood Kidney Diseases
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• 제11권2호
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• pp.299-305
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• 2007

HWW(Herlyn-Werner-Wunderlich) 증후군은 중복자궁, 일측성 폐쇄질 및 동측 신장 무형성을 보이는 비뇨생식기계의 선천성 기형으로 매우 드문 질환 중 하나이다. 대부분 초경 이후 발생하는 월경통이나 복강내 종물등으로 발견되지만, 본 증례는 소아에서 반복되는 요로 감염과 현미경적 혈뇨로 인해 진단된 경우이다. 복부 초음파 검사에서 일측 신무형성이나 중복 자궁의 소경이 보일 때는 이러한 뮬러관 기형의 가능성을 염두에 두어야 보다 빠른 진단이 가능하고, 환자의 고통 또한 중여줄 수 있을 것이다. 특히 반복되는 요로 감염시에도 단순한 감염 치료보다 정밀검사를 항상 염두에 두어야 할 것이다.

• 대한핵의학회지
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• 제14권1호
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• pp.37-44
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• 1980

The usefulness of single, random measurements of serum FSH and LH in the diagnosis of primary amenorrhea by radioimmunoassay was investigated. The 16 patients were divided into 3 groups by the level of serum FSH and LH. The first group with increased level of serum FSH and LH is five patients, all of these are related to the acquired or congenital abnormality of the ovary. Further studies indicated include buccal smear, chromosome analysis, gynecography and laparosocopy. The second group with normal serum FSH and LH is nine patients, four patients of these are related to the developmental anomaly of the Mullerian duct and five patients are undo etermined origin. Further studies indicated include laparoscopy and gynecography. The third group with decreased serum FSH and normal or decreased serum LH is two patients, one of these is related to the pituitary function, isolated FSH deficiency, the other is undetermined origin. Further studies indicated include the pituitary function test, LH-RH stimulation test, skull radiography. Determination of serum FSH and LH levels does not permit a specific etiologic diagnosis of primary amenorrhea. However the serum levels of FSH and LH can be used to differentiate the principal area of the investigation and can be of assistance in choosing more specific testing procedures.

• Archives of Plastic Surgery
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• 제38권5호
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• pp.616-620
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• 2011

Purpose: Mayer-Rokitansky-Kuster syndrome (MRK) is second common cause of primary amenorrhea. It is a syndrome of vaginal aplasia and Mullerian duct anomaly. Vaginal aplasia varies from agenesis of whole vagina to aplasia of upper 2/3. For reconstructing vagina, various methods are introduced. Gracilis myocutaneous flap was the first attempt in that the flap is used in vaginal reconstruction. Various flap-based vaginal reconstruction methods have been introduced. Modified Singapore flap (pedicled neurovascular pudendal thigh fasciocutaneous flap) is one of those methods that used posterior labial artery as pedicle, and pudendal nerve branch as sensory root. As its donor lies on inguinal crease that is easily hidden and there are benefits on sexual intercourse by early sensory recovery, authors think that modified Singapore flap is effective for young MRK syndrome patients. Methods: Eight patients underwent surgery between 2008 and 2010. The flap was designed on both groin area with external pudendal artery branch as a pedicle. All flaps were fixated in pelvic cavity with absorbable suture, and additional compression on neovaginal wall was supplied by polyvinyl alcohol sponge ($Merocel^{(R)}$). Results: All patients were successfully reconstructed without flap related complications such as congestion or partial flap loss. The average size of the flap (each side) was 69.34 $cm^2$. Polyvinyl alcohol sponge ($Merocel^{(R)}$) was inserted into neovagina for 5 days on every patient. One case of rectal laceration was occurred while making pelvic pocket by OBGY team. Other complications such as lumen narrowing, wound contracture or vaginal prolapsed were not reported during 8 months of follow up. Conclusion: Modified Singapore flap is a sensate fasciocutaneous flap that is thinner than other myocutaneous flap such as VRAM, and more durable over skin graft. Therefore this is a good choice for vaginal reconstruction in MRK syndrome. And known complications of Modified Singapore flap could be reduced with careful procedure and mild compression techniques.