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Management of Prenatally Diagnosed Uropathies Arch Dis Child: first published as 10.1136/adc.64.1_Spec_No.58 on 1 January 1989. Downloaded from Archives of Disease in Childhood, 1989, 64, 58-63 Personal practice Management of prenatally diagnosed uropathies D F M THOMAS AND A C GORDON St James's University Hospital and The General Infirmary, Leeds It is not unrealistic to anticipate that by the turn of more realistic to regard prenatal ultrasound as a the century virtually every child in the United means of screening fetuses for uropathies that will Kingdom with an appreciable urological abnormal- require investigation in postnatal life. ity will have been diagnosed by ultrasound before A recent analysis of about 47 000 pregnancies birth. There is a possibility, however, that our over a five year period in Leeds yielded an incidence ability as clinicians to interpret and utilise informa- of prenatally diagnosed uropathies of 1/570 pregnan- tion derived from prenatal ultrasound will not keep cies. This figure includes those pregnancies that pace with the increasing sophistication and availabil- were terminated and those that subsequently re- ity of the imaging techniques. Current management sulted in neonatal death from pulmonary hypopla- of prenatally diagnosed uropathies is based as much sia. If these non-viable fetuses are excluded from the on empiricism as on science. Therapeutic strategies calculation, we arrive at a figure of one live born and indications for surgery based on experience with neonate with a significant urological abnormality in by copyright. symptomatic conditions in older children are not every 800 live births. Thus the 'pick-up' rate for necessarily relevant to neonates with asymptomatic prenatal diagnosis is now within the incidence range anomalies diagnosed prenatally. previously recorded in large postmortem studies. (For example, one study of 245 000 necropsies Prenatal diagnosis: accuracy and reliability reported an incidence of renal anomalies of 1/650.1) Our figures suggest that prenatal ultrasound diagno- It is important to have realistic expectations of sis is highly effective and is already capable of ultrasound. It can provide only anatomical picking up most important urological abnormalities information-which in this context usually means before birth. dilatation of the urinary tract. As it cannot reliably Although we can rely increasingly on the ability of distinguish between high grade obstruction, low ultrasound to detect abnormal kidneys that are http://adc.bmj.com/ pressure non-obstructive dilatation or reflux, a final dilated, this is not the case with kidneys that are urological diagnosis depends upon the use of cys- small or absent. We should be very reluctant to tography and isotopes, which are only available advise any action or invasive intervention on the postnatally. basis of an ultrasound diagnosis of renal agenesis. Nevertheless, prenatal ultrasound screening can serve two important roles. The first is in identifying Obstetric implications of prenatally diagnosed grossly affected fetuses at a time when termination uropathies on September 29, 2021 by guest. Protected of pregnancy is still an option. The second role lies in its ability to identify fetuses with uropathy that require appropriate investigation in postnatal life. The specialised areas of intrauterine diagnosis and Within this limited framework prenatal ultra- fetal intervention are the joint concern of obstetri- sound is highly effective. With modern real time cians, radiologists, and paediatric specialists. The scanners diagnostic errors are more likely to be due latter are generally better placed to advise on the to inexperience in obtaining and interpreting the likely significance of ultrasound findings and to images rather than any deficiency of the equipment. predict the likely prognosis and course of postnatal Some skilled specialist radiologists are now able to management. Perhaps the most effective way of provide highly detailed anatomical information- ensuring paediatric involvement in obstetric man- particularly in later pregnancy. While this is desir- agement is to set up a combined prenatal counselling able (it enables us to give a more accurate prognosis clinic. In practice, however, it may prove very to the parents) it is not essential. At present it is difficult to reorganise the timetables of the various 58 Arch Dis Child: first published as 10.1136/adc.64.1_Spec_No.58 on 1 January 1989. Downloaded from Management of prenatally diagnosed uropathies 59 consultant specialists whose presence at such a clinic liquor-of which fetal urine is a major constituent. is essential. In its analysis of fetal intervention for obstructive The gestational age at which routine ultrasound uropathy, the International Fetal Surgery Registry scanning is undertaken is something of a comprom- reported neonatal death from pulmonary hypoplasia ise. Most obstetricians settle for the period between in 27 (37%) of 73 fetuses treated in utero. In a 17 and 20 weeks. Scanning before 17 weeks yields further 11 cases the pregnancy was subsequently less detailed anatomical information and a lower terminated. It is highly likely that these fetuses pick up rate for congenital abnormalities. Routine would also have succumbed to pulmonary hypopla- scanning after 20 weeks is more accurate, but leaves sia if the pregnancies had proceeded to term. These less time to acquire additional information on the figures and those published in a recent review of the fetus upon which to base a decision to terminate the literature suggest that intrauterine surgery to drain pregnancy. Scans undertaken between 17 to 20 the urinary tract is unlikely to reduce neonatal weeks can be expected to pick up most of the lethal mortality from pulmonary hypoplasia. urological abnormalities. These scans can also yield There is another major concern about intrauter- a great deal of anatomical information: biparietal ine intervention. Dilatation is not synonymous with diameter and fetal length; they can show longitudi- obstruction. Ultrasound imaging cannot distinguish nal and transverse views of the spine and four between dilatation, which is a consequence of high chamber views of the heart; and provide informa- pressure obstruction (for which it is possible to make tion on the stomach, kidneys, and bladder. At this a theoretical case of intervention) and non- stage in the pregnancy, however, dilatation due to obstructive dilatation or reflux for which interven- reflux or to less severe forms of obstruction may not tion would be inappropriate. Furthermore, fetal yet be evident. intervention has a published procedure related The findings of an appreciable urological abnor- complication rate of 44%4 and a procedure related mality on the initial dating scan should be confirmed mortality rate of anything up to 10%.3 4 The by a second examination-ideally performed by a published data do not show a benefit from fetal by copyright. radiologist with particular expertise and experience intervention that justifies these risks. in prenatal diagnosis. Some form of radiological Fifty nine fetuses with bilateral uropathy have 'second opinion' is highly desirable if termination of been managed in Leeds during the last five years. pregnancy is one of the options under consideration. In no instance has intrauterine drainage been Indeed there is a strong case for referring the attempted. In 15 cases the fetus was severely mother to a regional centre to be scanned by a affected (oligohydramnios, etc, see below) and the radiologist working in conjunction with an obstetri- pregnancy was terminated. A further eight cases cian trained in intervention techniques and a resulted in neonatal death from pulmonary hypo- paediatric urologist (or surgeon). plasia. Thus in this series of fetuses managed Obstetric intervention can take the form of without intrauterine drainage, a total of 23 (39%) intrauterine drainage (fetal surgery), termination of were non-viable as a result of severe urinary tract http://adc.bmj.com/ pregnancy, or induction of premature labour. obstruction. The mortality reported by the Inter- national Fetal Surgery Registry was 59% despite (1) INTRAUTERINE DRAINAGE intervention.3 It would be unwise, however, to Fetal surgery-to drain the obstructed urinary draw any firm conclusions from these figures as tract-is a logical and, at first sight, attractive there may have been important differences between extension of prenatal diagnosis. Unfortunately there the two groups of fetuses. Long term follow up is are several flaws in this concept. essential. Nevertheless our limited experience The first is that the renal dysplasia and pulmonary does not suggest that intrauterine intervention on September 29, 2021 by guest. Protected hypoplasia may already be irreversible by the time offers any great benefit. It is our view that the the urinary tract dilatation is first detected at 17 to continuing use of these procedures can only be 20 weeks. This dilemma is illustrated by the first justified in the context of a formal prospective published case of fetal surgery, which was reported controlled trial. by Harrison and his colleagues in San Francisco.2 In this case a fetus with severe outflow obstruction was (2) TERMINATION OF PREGNANCY operated upon via a hysterotomy at 21 weeks' The most important indicator of poor prognosis is gestation. Despite adequate decompression of the oligohydramnios, but others include 'bright' (dys- urinary tract by cutaneous ureterostomy, the plastic) renal parenchyma, a high concentration of affected
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