Freedman A L, Bukowski T P, Smith C A, Evans M I, Johnson M P, Gonzalez R
Department of Pediatric Urology, Children's Hospital of Michigan, Detroit 48201-2196, USA.
J Urol. 1996 Aug;156(2 Pt 2):720-3; discussion 723-4.
Attempts to evaluate prenatal vesico-amniotic shunt therapy have been hampered by inconsistencies in patient selection, treatment and termination criteria, and outcomes measurement. Outcomes have generally been measured against those of patients with postnatally detected posterior urethral valves. The purpose of this report was to evaluate the influence of the underlying diagnosis on the clinical outcomes of fetuses undergoing evaluation for prenatal intervention for suspected obstructive uropathy. Furthermore, specific outcomes diagnosis was compared to the published natural history of these disorders to begin to establish a basis for measuring the efficacy of prenatal intervention.
We retrospectively reviewed the outcomes of 55 consecutive patients undergoing prenatal evaluation using structured outcome measures stratified by specific diagnoses to provide a comparison to the reported natural history for each underlying disorder.
All fetuses had early onset of oligohydramnios/anhydramnios representing the worst end of the spectrum. Compared to postnatally diagnosed patients, prenatally diagnosed patients with posterior urethral valves had lower survival (60 versus 93%) but similar postnatal renal failure rates (31 versus 33%). Cases of prenatally detected but untreated posterior urethral valves had a 44% renal failure rate. In fetuses with the prune-belly syndrome survival (86 versus 72%) and renal function rates (17 versus 27% renal failure) compared favorably with the postnatal experience, although 55% of the cases had significant urethral obstruction. All patients with urethral atresia died.
The underlying etiology of obstruction appears to have a marked influence on clinical outcome independently of treatment. When evaluated by specific diagnosis, intervention appears to provide outcomes in these high risk fetuses that are comparable to those for disease detected postnatally. Interpretation of series that do not distinguish cases by onset, severity and specific pathological process is problematic. Greater standardization of patient selection, treatment and outcome measurement, including the use of specific diagnoses, is necessary to allow an accurate assessment of the efficacy and proper role of fetal therapy.
患者选择、治疗及终止标准和结果测量方面的不一致阻碍了对产前膀胱羊膜分流疗法的评估。结果通常是与出生后检测出后尿道瓣膜的患者的结果进行对比。本报告的目的是评估潜在诊断对因疑似梗阻性泌尿系统疾病接受产前干预评估的胎儿临床结果的影响。此外,将具体结果诊断与这些疾病已公布的自然病史进行比较,以便开始为衡量产前干预的疗效奠定基础。
我们回顾性分析了55例连续接受产前评估患者的结果,采用按特定诊断分层的结构化结果测量方法,以便与每种潜在疾病报告的自然病史进行比较。
所有胎儿均早期出现羊水过少/无羊水,代表了该谱系的最严重结局。与出生后诊断的患者相比,产前诊断为后尿道瓣膜的患者存活率较低(分别为60%和93%),但出生后肾衰竭发生率相似(分别为31%和33%)。产前检测但未治疗的后尿道瓣膜病例的肾衰竭发生率为44%。患有Prune-Belly综合征的胎儿的存活率(分别为86%和72%)和肾功能率(肾衰竭发生率分别为17%和27%)与出生后的情况相比更有利,尽管55%的病例存在明显的尿道梗阻。所有尿道闭锁患者均死亡。
梗阻的潜在病因似乎独立于治疗对临床结果有显著影响。通过特定诊断进行评估时,干预似乎能为这些高危胎儿提供与出生后检测出疾病的胎儿相当的结果。对未按发病、严重程度和特定病理过程区分病例的系列研究进行解读存在问题。为了准确评估胎儿治疗的疗效和恰当作用,患者选择、治疗和结果测量需要更大程度的标准化,包括使用特定诊断。