Boczar Maria, Sawicka Ewa, Piwowar Włodzimierz
Klinika Chirurgii Dzieci i Młodziezy, Instytut Matki i Dziecka w Warszawie, 01-211 Warszawa.
Med Wieku Rozwoj. 2009 Oct-Dec;13(4):260-9.
Myelomeningocoele is a serious congenital deformity requiring multidisciplinary medical care, and parent' s aware cooperation. The accessibility and popularity of foetal ultrasonography enables early prenatal diagnosis which makes possible optimal management during pregnancy and treatment after birth.
Evaluation of the treatment of newborns with myelomeningocoele in the last 20 years in relation to the changes in diagnostics and operative procedures.
The evaluation was performed in 114 newborns, operated on because of myelomeningocoele between 1986-1992 (group I) and 1999-2005 (group II). Prenatal diagnosis, the term and kind of labour, anatomical description of deformity, the level of spinal cord damage were analyzed. Evaluation also included: the term and kind of operation, the presence of active hydrocephalus and its association with the anatomical picture of deformity, the strategy of shunt implantation and the number of deaths during the first hospitalization. Orthopaedic and urological problems were not evaluated.
During twenty years, reduction in the number of patients with myelomeningocoele was not observed, despite the introduction of the programme of women' s folic acid prophylaxis. In two analyzed periods there were 8 new cases treated each year. The number of prenatal diagnoses increased: in group I prenatal diagnosis was made only in 8 cases (14%), in group II in 25 cases (44%), but in most cases diagnosis was established late (in the last 3 months of pregnancy). Analysis of different anatomical forms of the deformity showed, that the number of thoracic myelomeningocoeles decreased from 32 cases in group I to 18 cases in group II. In both groups operation was performed during 24 hours following admission of the newborn to the Institute. The surgical technique has not change: large skin defects were closed with the use of rhomboidal muscle and skin flap (Limberg' s plasty). The number of complications in wound healing decreased from 26.4% to 7%. Active hydrocephalus was confirmed in 49 of 57 patients in group I (85%) and in 46 of 57 patients in group II (80%). Patients without active hydrocephalus (group I - 8 children, group II - 11 children) presented lumbo-sacral or sacral localization of defect. In the years 1999-2005 (group II), contemporary with the operation of myelomeningocoele, Rickham reservoir was implanted in 11 newborns with massive hydrocephalus. The shunt was added after the confirmation of active hydrocephalus and after a negative result of cerebrospinal fluid culture. Decrease of deaths during the first hospitalization was observed (8 in group I, 2 in group II). The principal cause of death in both groups was inborn infection.
脊髓脊膜膨出是一种严重的先天性畸形,需要多学科医疗护理以及家长的积极配合。胎儿超声检查的可及性和普及性使得早期产前诊断成为可能,从而能够在孕期进行最佳管理并在出生后进行治疗。
评估过去20年中脊髓脊膜膨出新生儿的治疗情况,以及诊断和手术程序的变化。
对1986 - 1992年(第一组)和1999 - 2005年(第二组)因脊髓脊膜膨出接受手术的114例新生儿进行评估。分析产前诊断、分娩孕周和方式、畸形的解剖描述、脊髓损伤水平。评估还包括:手术孕周和方式、活动性脑积水的存在及其与畸形解剖图像的关联、分流植入策略以及首次住院期间的死亡人数。未评估骨科和泌尿外科问题。
二十年间,尽管实施了妇女叶酸预防计划,但脊髓脊膜膨出患者数量并未减少。在两个分析时间段内,每年均有8例新病例接受治疗。产前诊断数量增加:第一组仅8例(14%)进行了产前诊断,第二组为