Weissman A, Jakobi P, Zaidise I, Drugan A
Department of Obstetrics and Gynecology, Rambam Medical Center, Technion-Faculty of Medicine, Haifa, Israel.
J Reprod Med. 1993 Nov;38(11):890-6.
Neurofibromatosis is one of the most frequent genetic diseases in humans. Pregnancy in neurofibromatosis patients is, however, less common. Most current information on pregnancy and neurofibromatosis is derived from case reports, which may not reflect the true situation. In the past 15 years only two series of pregnant neurofibromatosis patients were reported in the English-language literature. We present our experience with 34 pregnancies in nine neurofibromatosis patients who delivered at our medical center. While fertility does not seem to be impaired in neurofibromatosis, these patients experience a higher-than-expected rate of first-trimester spontaneous abortions (20.7%), stillbirths (8.7%) and intrauterine growth retardation (13.0%). A high rate of cesarean section (26%) was also observed in our series. We conclude that pregnant neurofibromatosis patients constitute a high-risk group, in danger of developing life-threatening complications. However, with proper antenatal care, most pregnant neurofibromatosis patients can deliver safety if the pregnancy continues beyond the first trimester.
神经纤维瘤病是人类最常见的遗传性疾病之一。然而，神经纤维瘤病患者怀孕的情况较少见。目前关于怀孕与神经纤维瘤病的大多数信息来自病例报告，这些报告可能无法反映真实情况。在过去15年里，英文文献中仅报道了两例患有神经纤维瘤病的孕妇病例系列。我们介绍了在我们医疗中心分娩的9例神经纤维瘤病患者的34次妊娠经历。虽然神经纤维瘤病患者的生育能力似乎未受损害，但这些患者的孕早期自然流产率（20.7%）、死产率（8.7%）和胎儿宫内生长迟缓率（13.0%）均高于预期。我们的病例系列中还观察到较高的剖宫产率（26%）。我们得出结论，怀孕的神经纤维瘤病患者构成高危人群，有发生危及生命并发症的风险。然而，通过适当的产前护理，如果妊娠持续超过孕早期，大多数怀孕的神经纤维瘤病患者可以安全分娩。
