Management of high-risk gestational trophoblastic disease.
Department of Obstetrics and Gynecology, Northwestern University Medical School, Chicago, Illinois 60611, USA. firstname.lastname@example.org
Multimodality therapy with combination chemotherapy employing etoposide, high-dose methotrexate, actinomycin D, cyclophosphamide and vincristine (EMA-CO), and adjuvant radiotherapy and surgery, when indicated, has resulted in cure rates of 80-90% in patients with high-risk metastatic gestational trophoblastic tumors. However, approximately 25-30% of high-risk patients will have an incomplete response to first-time chemotherapy or will relapse from remission. Most of these patients will have a clinicopathologic diagnosis of choriocarcinoma, metastases to sites other than the lung and vagina, more than eight metastases and/or failed inappropriate previous chemotherapy, resulting in very high World Health Organization scores. Salvage chemotherapy with cisplatin/etoposide, usually in conjunction with bleomycin or ifosfamide, as well as surgical resection of sites of resistant disease in selected patients, will result in a cure in most patients. New technology, such as the use of colony-stimulating factors to prevent treatment delays and dose reductions or high-dose chemotherapy with or without autologous bone marrow transplantation or peripheral blood stem cell support, may play an important role in the future management of patients who develop drug resistance.
Spontaneous rupture of a primigravid uterus secondary to placenta percreta. A case report.
Imseis HM. Murtha AP. Alexander KA. Barnett BD.
Department of Obstetrics and Gynecology, Duke University Medical Center, Durham, North Carolina.
BACKGROUND: Uterine rupture secondary to placenta percreta has been observed in multiparous patients. These cases are typically associated with a prior history of uterine trauma or infection: hysterotomy, myomectomy, cornual resection, dilatation and curettage, manual removal of the placenta or endometritis. Spontaneous rupture of the primigravid uterus without a history of trauma or infection is an exceedingly rare occurrence. This case represents the second reported in the medical literature and the first to result in a live-born infant. CASE: A 23-year-old, African American primigravida at 26 weeks' gestation presented with acute-onset abdominal pain, severe hypotension, tachycardia and fetal heart rate decelerations. Blood product replacement was initiated, and an emergency laparotomy was performed for a presumptive diagnosis of intraabdominal hemorrhage. A significant hemoperitoneum was encountered, with the fetus floating freely in the peritoneal cavity. The uterus had a fundal rupture with a clinically apparent placenta percreta that necessitated performing a total abdominal hysterectomy. The patient recovered uneventfully, and the infant survived without significant morbidity. CONCLUSION: Spontaneous rupture of the primigravid uterus can occur in the absence of a history of uterine trauma or infection. If a gravid woman presents with hypotension, abdominal pain and fetal distress, the differential diagnosis should include rupture of the uterus, regardless of parity or gynecologic history. Rapid diagnosis, blood product replacement and emergency laparotomy are the key steps in successful management.
Periovarian peritoneal adhesions in women with endometriosis. Structural patterns.
Jirasek JE. Henzl MR. Uher J.
Institute of the Care of Mother and Child, Prague, Czech Republic.
OBJECTIVE: To draw attention to the structural features of adhesions associated with pelvic endometriosis since they are less well studied than endometriosis proper. STUDY DESIGN: Sixty-two samples of periovarian adhesions were laparoscopically obtained from 24 infertile women 26-38 years of age and were prepared for detailed histologic analyses. RESULTS: Macroscopically, the adhesions were either velamentous or cordlike and grossly were free of endometriosis. Most adhesions were attached to the connective tissue of the ovarian tunica albuginea; in two cases they were attached to the corpus luteum. Upon microscopic analysis, velamentous adhesions consisted of fibrous sheets of collagen connective tissue, with the surface lined with single-layered coelomic epithelium. The cord-like adhesions consisted of "hyalinized" fibrous tissue and were either avascular or vascularized. Irregular cystic or tubular structures that could be regarded as endometriosis were found in four patients. Hyaline cartilage within the tissue of the adhesion was an unexpected finding in one patient. CONCLUSION: Microscopic analysis permitted grouping of the adhesions in the following way: (1) Connective tissue adhesions (23 patients) with the following subcategories: (A) fibrous, either avascular or encompassing degenerating blood vessels (6 patients); (B) vascularized, containing granulomatous tissue (12 patients); (C) vascularized with stromoglandular endometrioid cysts or tubules (4 patients); (D) rare types, such as that encompassing hyaline cartilage (1 patient). (2) Fibrin adhesions (1 patient). From the clinical point of view, the presence of endometrial tissue within adhesions raises the question of whether there is a need for removal, rather than just lysis, of adhesions to avoid persistent pain.
Endometriosis. Current issues in diagnosis and medical management.
Leuven Institute for Fertility and Embryology, Belgium.
OBJECTIVE: To explore the hypothesis that endometriosis is a disease not just because it exists but because it is functionally active. STUDY DESIGN: Qualitative research analyzing the morphologic appearances of endometriosis and the clinical effect of medical therapies. RESULTS: Analysis of the appearances of symptomatic endometriosis demonstrates that the ectopic endometriumlike tissue mimics eutopic endometrium but with loss of polarization. Ectopic implants resembling superficial endometrium are hemorrhagic and associated with adhesion and pseudocyst or endometrioma formation. Ectopic implants resembling basal or junction zone endometrium are associated with nodular adenomyotic lesions in the posterior fornix and pelvic supportive structures. They are characterized by smooth muscle hyperplasia and T-lymphocyte aggregates. CONCLUSION: Medical therapy has been shown to be very efficient in reducing pelvic pain as soon as amenorrhea is created and maintained. Regression, but not elimination, of the implant is obtained by medical therapy. Clinical data support the hypothesis that the efficacy of medical therapy is largely achieved by preventing cyclic bleeding in the implants.
Screening semen donors for hereditary diseases. The Fairfax cryobank experience.
Bick D. Fugger EF. Pool SH. Hazelrigg WB. Yadvish KN. Spence WC. Maddalena A. Howard-Peebles PN. Schulman JD.
Genetics & IVF Institute, Fairfax, Virginia 22031, USA.
OBJECTIVE: To study the carrier frequency of hereditary diseases in potential semen donors with no family history of a genetic disease. STUDY DESIGN: Carrier screening was performed on potential semen donors for chromosomal abnormalities, cystic fibrosis, alpha-1-antitrypsin deficiency, hemoglobinopathies, Tay-Sachs disease, Gaucher disease, Canavan disease, and hereditary breast and ovarian cancer (the BRCA1 185delAG mutation). The screening regimen used for each donor was dictated by his ethnic background. RESULTS: Among 361 individuals screened for chromosomal abnormalities, 1 carried an inversion, and 4 were possible mosaics. Fifteen of 407 potential donors carried cystic fibrosis, 18 of 209 carried alpha-1-antitrypsin deficiency, and 2 of 74 carried a hemoglobinopathy. No carriers of Tay-Sachs disease (56 screened), Gaucher disease (32 screened), Canavan disease (22 screened) or the BRCA1 185delAG mutation (22 screened) were found. CONCLUSION: Screening semen donors for a number of genetic diseases that are passed silently from generation to generation is warranted since family history alone cannot identify them.