Laparoscopic morcellation of didelphic uterus with cervical and renal aplasia.

This is a case report (and review of the literature) of a 12-year and 10-month-old girl with a rare congenital anomaly of uterus didelphys, unilateral cervix aplasia, and ipsilateral renal aplasia. She had severe dysmenorrhea from the first menses. In an effort to preserve fertility, a cervical fistula was made that closed over. A laparoscopic hemi-hysterectomy was done successfully and rapidly with laparoscopic morcellation. Because no ureter was present, it was not necessary to trace it. For this congenital anomaly, laparoscopic morcellation of the obstructed hemiuterus is the preferred treatment either as a primary procedure or as a secondary procedure following failure of a surgical cervical fistula for the young patient.

Prepubertal unilateral fibrous hyperplasia of the labium majus: report of eight cases and review of the literature.

OBJECTIVE: To report and propose a consensus term for eight cases of a newly recognized, asymptomatic, rapidly growing unilateral labium majus mass without palpable borders in prepubertal girls, appearing to be temporally associated with the physiologic increase of adrenal hormones. METHODS: Histologic examination, special stains, and immunohistochemistry were performed on all cases. In our personal cases, electron microscopy and chromosomal analysis were also performed, together with pelvic magnetic resonance imaging (MRI), inguinal exploration, vaginoscopy with biopsies, and adrenal hormone levels. RESULTS: Of the eight cases, seven were Ashkenazi Jewish girls from the same area in New York City. The unilateral masses were asymptomatic, soft, without palpable borders. The overlying skin had a slightly tan peau d'orange surface. The masses could not be completely excised because they extended into the contiguous pelvic floor. Histologically, the masses were composed of bland hypocellular fibrous tissue extending into the deep subcutaneous tissue. The masses blended into the surrounding tissue and adjacent pelvic floor as shown by MRI preoperatively and postoperatively. Residual tissue did not progress after incomplete resection. CONCLUSION: These fibrous lesions develop in months at the time of physiologic increase in adrenal hormone secretion just before puberty and subsequently appear to stop growing. The surgeon should not attempt a complete removal but simply excise sufficient tissue for a reasonable cosmetic result and to confirm the diagnosis. The lesions reflect fibroblastic hyperplasia, which is possibly hormone-driven. The ethnic and geographic clustering of cases raises consideration of environmental exposures or genetic predisposition.

Uterus transplantation.

Until recently, only life and death situations warranted organ transplantation. Nonvital transplantation, to further a patient s wishes and goals, was not considered justified. It can be argued, however, that this distinction is not morally significant. Patients with kidney failure, for example, can be kept alive by dialysis. But their quality of life would be greatly enhanced by kidney transplant, which is thus considered a justified procedure. So a spectrum of rationales may justify transplantation. Transplantation of the uterus would relieve the anguish of women who greatly desire to conceive a child. Some women do not have a uterus. In some cases this is due to a congenital absence (Rokitansky s syndrome). In other cases, surgical removal of the uterus was required to repair an obstetrical rupture. With a transplanted uterus, many of these women could have the opportunity to become pregnant as a result of nonvital organ transplant. While other organ transplant donations most often come from cadavers and less often from living donors (kidney or partial liver), the donor source for a uterus may be an otherwise healthy living patient who requires uterus removal as a standard care procedure. Furthermore, it should be possible to remove the transplanted uterus from the recipient after successful pregnancies, so the patient would not be subjected to lifelong antirejection medications. Since animal uterus transplantation has been done successfully, human uterus transplantation might be considered for select cases. One such case has been reported.

Cultured bilayered skin allograft for vaginal construction.

OBJECTIVE: This is the first report of live human cultured bilayered skin allograft (taken from another person) (LHCBSA) to line a dissected space to create a vagina. CASE: A 19-year-old with Mayer-Rokitansky-Küster-Hauser syndrome (MRKH syndrome) of vaginal and uterine agenesis had a space dissected and lined with LHCBSA. Although the lining devitalized within 2 weeks, there was a rapid ingrowth of vaginal mucosal cells from the vaginal dimple with an excellent long-term result. CONCLUSION: This is the first report that LHCBSA is able to stimulate vaginal mucosal cell growth for a neovagina. It is possible that it might stimulate other surface tissue lining to cover adjacent raw areas such as bladder or esophagus.

Experimental uterus transplantation.

BACKGROUND: Uterus transplantation (UTx) is developed in animal models as a future method to treat uterine factor infertility. METHODS: All published studies in the area of UTx research were identified. Aspects relating to surgery, cold-ischemia/reperfusion, rejection, immunosuppression, pregnancy, ethics and institutional requirements were examined. RESULTS: Uterus retrieval surgery has been solved in animals, including primates. Studies on cold-ischemia/reperfusion indicate an ischemic tolerance of >24 h. The transplantation procedure, with vascular anastomosis, has not been fully developed in animal models, indicated by frequent thrombosis formation. Pregnancies have only been reported in syngenic/auto-UTx animal models. Several ethical issues in relation to UTx, and requirements for a team that would be suitable to undertake human UTx, exist. CONCLUSION: Much research on UTx has been performed in appropriate animal models. Several aspects of the procedure have been optimized but some remain to be solved. It is predicted that the research will soon reach a stage that could merit introduction of human UTx as an experimental procedure.

Successful pregnancy following surgery in the obstructed uterus in a uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: case report and literature review.

SYNOPSIS: After surgical correction of unilateral distal vaginal agenesis, the obstructed didelphic uterus was able to harbor 2 subsequent pregnancies. BACKGROUND: There was a congenital anomaly syndrome of uterus didelphys, unilateral distal vaginal aplasia, and ipsilateral renal aplasia. Intuition suggests that later pregnancy would be in the unobstructed uterus. OBJECTIVE: The purpose is to report pregnancy occurring twice in a previously obstructed didelphic uterus after surgical correction. CASE: A girl aged 13 years, 8 months presented with the syndrome of didelphic uterus, upper right hematocolpos, and right renal aplasia. Right vaginal marsupialization was done. Subsequently, she had 2 pregnancies in the right didelphic uterus. CONCLUSION: Pregnancy occurred in the obstructed uterus despite a large hematometra, extensive right pelvic peritoneal endometriosis, and hematocolpos. The surgeon should make every effort to preserve the obstructed uterus.

Squamous papilloma with hyperpigmentation in the skin graft of the neovagina in Rokitansky syndrome: literature review of benign and malignant lesions of the neovagina.

BACKGROUND: It is rare for a benign or malignant neoplasm to develop in a neovagina. CASE: This is the first report of a squamous papilloma with hyperpigmentation which developed in the neovagina 12 years after a McIndoe procedure was done with a split-thickness skin graft from the patient's buttock. The patient had congenital absence of the vagina and uterus (Rokitansky syndrome, Mayer-Rokitansky-Küster-Hauser syndrome, MRKH syndrome). It presented as post-coital blood spotting for 3 months. CONCLUSION: As a routine, all cases of vaginal construction regardless of the reason for the surgery or the lining of the neovagina should have a continuous annual examination and careful inspection of the entire vagina. Biopsy of any unusual finding should be done. Despite the dark color of the vaginal lesion, bleeding and rapid appearance our patient had a benign tumor. Radiation therapy to the neovagina and prolonged treatment with podophyllin and cauterization should be avoided.

Prepubertal distal longitudinal vaginal folds.

OBJECTIVE: The purpose of this paper is to further describe prepubertal distal longitudinal vaginal folds and its natural history. DESIGN: Biopsy of the folds and follow-up through puberty. RESULTS: Histology of the folds shows normal vaginal mucosa over loose fibrous connective tissue without a firm fibrous core. The folds disappear in puberty. CONCLUSIONS: Prepubertal distal longitudinal folds are common, and disappear probably by spreading out during puberty with the physiologic estrogenic widening and lengthening of the vagina. This can occur because the folds do not have a dense fibrous core. The folds may be confused with the vaginal rhabdomyosarcoma or sexual molestation if the fold reaches the hymen, and forms a "bump". The folds are benign.

Stage I ovarian carcinoma: different clinical pathologic patterns.

OBJECTIVE: To analyze clinicopathologic patterns of early ovarian carcinoma. DESIGN: Retrospective chart and histopathology review. SETTING: Mount Sinai School of Medicine, New York and the Centre Jean Perrin, Clermont Ferrand, France. PATIENT(S): Seventy-six consecutive cases of Fédération Internationale de Gynécologie et d'Obstétrique stage I ovarian carcinoma. INTERVENTION(S): Surgical staging. MAIN OUTCOME MEASURE(S): Symptomatology, pathology, and histology analysis. RESULT(S): Twenty-two cases (29%) were serous papillary carcinomas and 54 were nonserous carcinomas (71%) (40 endometrioid, 10 clear cell, and 4 mixed endometrioid and clear cell carcinomas). Ninety-eight percent of ovarian endometriosis, 95% of endometrial carcinomas, and 83% of endometrial polyps and hyperplasias were associated with nonserous carcinomas. Most patients with serous papillary carcinoma presented with asymptomatic pelvic masses; patients with nonserous carcinomas presented with pelvic pain or abnormal vaginal bleeding with or without pelvic mass. CONCLUSION(S): Over two thirds of stage I ovarian carcinomas were nonserous, and were diagnosed because of associated symptoms: pelvic pain with endometriosis and/or adnexal masses, or vaginal bleeding from endometrial pathology. Serous papillary carcinomas were often asymptomatic and diagnosed during follow-up evaluations in breast cancer patients. Stage I ovarian carcinoma has different clinical and pathologic patterns than advanced ovarian carcinoma. The risk of ovarian and endometrial malignancy should be taken into consideration during evaluation of patients with endometriosis and breast cancer histories.

Mesenteric fibromatosis masquerading as an ovarian neoplasm twenty years after Chernobyl radiation exposure.

BACKGROUND: Since their initial description in 1832, desmoid tumors have been reported to occur in virtually every part of the body. Intra-abdominal desmoid tumors, or mesenteric fibromatosis, are often associated with Familial Adenomatous Polyposis mutation in a syndrome known as Gardner's Syndrome. Although sporadic cases of desmoid tumors do occur, unlike Gardner's Syndrome, they predominantly occur extra-abdominally. CASE: Case report of a 61-year-old female who presented with two months of abdominal pain, progressive lower abdominal distension and a 10-15 pound weight gain accompanied by one week of urinary hesitancy and frequency. Patient underwent a diagnostic workup for an ovarian neoplasm, but was found at surgery to have mesentric fibromatosis. CONCLUSION: Although uncommon, mesentric fibromatosis must be considered in the differential diagnosis when evaluating a patient with an abdominal mass of unknown origin.