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The Presentation and Management of Neonatal Obstructive Uropathies J Postgrad Med J: first published as 10.1136/pgmj.48.562.486 on 1 August 1972. Downloaded from Postgraduate Medical Journal (August 1972) 48, 486 -492. The presentation and management of neonatal obstructive uropathies J. H. JOHNSTON F.R.C.S. Alder Hey Children's Hospital, Liverpool Presentation urinary, alimentary and genital tracts share a The existence of a congenital urinary obstruction common evacuatory channel is similarly frequently may be suggested when routine examination of the associated with upper urinary tract lesions. newborn infant reveals such signs as enlargement of one or both kidneys, distension of the bladder or (3) Anomalies of the female genital tract slow, dribbling micturition. The paediatrician should Congenital absence of the vagina is associated also be alerted to the possibility of obstructive uro- with urinary tract anomalies in some 500 of cases pathy when there are present non-urological con- (Bryan, Nigro & Counsellor, 1949). A similar high genital anomalies which often secondarily involve incidence occurs with such conditions as duplication the urinary tract or which are known commonly to ofthe vagina and uterus but these may not be obvious co-exist with congenital urinary tract lesions. clinically in the young child. Secondary involvement, with obstruction, of the urinary tract occurs with space-occupying masses in Deficient abdominal mucsculature (4) by copyright. the pelvis such as hydrometrocolpos or a large intra- Urinary tract anomalies of various degrees of pelvic component of a sacrococcygeal teratoma. are The pelvic tumour, by displacing the bladder, leads severity always present. to chronic urinary retention and upper tract dilata- Cardiac anomalies tion. A congenital retroperitoneal teratoma may dis- (5) place laterally, and obstruct, one or both ureters. Mehrizi (1962) found that 23% of 279 cases of Examples of congenital anomalies which fre- malformation of the urinary tract had congenital quently coincide with congenital urological lesions heart disease. Ventricular septal defect was the most include the following. common lesion; of eighty-nine examples, twenty- two had urinary tract pathology. (1) Anomalies of the umbilicus and umbilical cord http://pmj.bmj.com/ A giant umbilical cord results from the accumula- (6) Limb deformities tion of urine within it as a consequence of a uracho- Hanke & Weicher (1965) reported that twenty-five umbilical fistula. The condition may be an isolated of 100 children with phocomelia due to thalidomide anomaly but the possibility ofan infravesical obstruc- embryopathy had urinary tract abnormalities. In tion must be investigated. Absence of one umbilical the Marfan syndrome, arachnodactyly is commonly artery is strongly suggestive of serious pathology in associated with cardiac and urinary anomalies; the alimentary and urinary tracts; Faierman (1960) Loughridge (1959) recorded eight cases with the on September 30, 2021 by guest. Protected found it to be present in 82% of cases of single um- latter. bilical artery. The baby with an exomphalos is very likely to have a urinary anomaly (McKeown, (7) Exstrophy McMahon & Record, 1953). The more severe forms of exstrophy, particularly the cloacal type, carry a 50/0 incidence of upper (2) Abnormalities of the rectum and anus urinary tract pathology (Soper & Kilger, 1964). In the Alder Hey series (Johnston, 1969) urinary tract pathology was present in 51% of cases of high (8) Gonadal dysgenesis rectal anomaly and in 430o of cases with a low, anal Turner's syndrome, which is suggested when the lesion; these did not include examples of cloacal newborn infant exhibits neck webbing, a low hair- exstrophy. The corresponding figures in Smith's line on the neck and oedema of the extremities, was (1968) series were 38% and 24%. Persistence of the associated with urinary lesions in 72% of cases in original cloacal state in the female in which the the series of Jeune et al. (1963). Postgrad Med J: first published as 10.1136/pgmj.48.562.486 on 1 August 1972. Downloaded from Neonatal obstructive uropathies 487 In a child with one of the above congenital I_ _··· anomalies urographic investigation of the urinary .:.J tract is advisable even in the absence of symptoms .1. suggestive of urologic disease. Hypospadias, especi- ally in its more severe forms, and undescent of the C. lieI- testis both a incidence of .clcl carry higher-than-average *1-.I rlcU* coincident urinary tract pathology but this is not 11*1 sufficiently high to warrant routine urological investi- ---·· ··uurrrr*llrLUI gation. Ascites in the newborn infant, particularly II, when associated with absent or abnormal voiding _ II, should arouse suspicion of the extravasation of urine wmr into the peritoneal cavity as a consequence of a C3 C. "- severe urinary obstruction; because of dialysis across . the peritoneal membrane the urea content of the _... ..._ ,1. ascitic fluid is generally much lower than that of the --F. _II 111 urine. o 11. Pathology . FIG. 1. Cystic, dysplastic, functionless (aplastic) kidney Urinary obstruction which originates post-natally from newborn boy with posterior urethral valves. characteristically leads to dilatation of the urinary tract above the obstructing lesion and to progressive Very often the first therapeutic needs of such thinning and destruction of the renal parenchyma as seriously ill babies are the correction of severe a result of back-pressure. Obstructions active during oligaemic dehydration and of acidaemia by the prenatal life may produce similar effects but often appropriate intravenous infusions and the control other pathological features exist. Prenatally ob- of infection by chemotherapy. Pending the results structed ureters in addition to being dilated are often of urine, and possibly of blood, culture, the most by copyright. lengthened and tortuous and on occasions the result- effective drug is likely to be ampicillin which can be ing ureteral angulations may cause secondary ob- given by intramuscular injection in a dosage of 50 structions, especially at the pelvi-ureteric junction. mg/kg body weight daily in four divided doses. The kidneys may show, instead of, or in addition to, Peritoneal dialysis may be needed when the clinical hydronephrotic atrophy, parenchymal changes indi- or biochemical response to intravenous therapy is cative of failure of maturation; cysts of various sizes unsatisfactory and, in particular, in the presence of may be visible macroscopically, and on histological dangerous hypernatraemia or, less often, of hyper- examination primitive glomeruli and tubules and kalaemia. Commercially available packs (Dialafex, nests of cartilage may be seen. The degree and extent Allen and Hanbury; Dianeal, Baxter) are suitable for of such developmental parenchymal dysplasia vary the newborn. Up to 50 ml fluid/lb body weight is http://pmj.bmj.com/ according to the severity of the obstruction and instilled into the peritoneal cavity at each cycle and probably also according to its time of onset in foetal siphoned out after ~-j hr. life. Withsevere obstructions of early onset the entire When the child's condition has sufficiently im- parenchyma may be grossly dysplastic and the kid- proved, the appropriate urological investigations ney is then functionless (Fig. 1). Lesser degrees of can be performed. In the presence of severely im- dysplasia may, however, be found in kidneys show- paired renal function, intravenous urography re- useful functional ing activity. quires a relatively high dose of contrast medium and on September 30, 2021 by guest. Protected delayed exposures for several hours after injection; Clinical features and general management prior dehydration of the infant by withholding feeds The most severe effects of urinary obstruction in is unnecessary and may be dangerous. Endoscopy, the newborn infant are seen when the obstructive with or without retrograde pyelography, and cysto- lesion is infravesical so that both kidneys are graphy with micturating or expression cysto- affected or when upper tract disease is bilateral or urethrography are commonly needed for diagnosis involves a solitary, or solitary functioning kidney. and full assessment. Such infants generally present with manifestations of azotaemia, acidaemia and, frequently, of urinary Posterior urethral valves infection, often with complicating septicaemia. In the male, the most severe form of congenital Anorexia, vomiting and diarrhoea are usually the lower urinary tract obstruction is that caused by initial symptoms; convulsions may occur and jaun- posterior urethral valves. In the commonest variety dice and a bleeding diathesis may develop as a result of this anomaly (Type 1 of Young & McKay, 1929) of septicaemia. the mucosal valve cusps diverge downwards and Postgrad Med J: first published as 10.1136/pgmj.48.562.486 on 1 August 1972. Downloaded from 488 J. H. Johnston laterally from the lower end of the verumontanum neous ureterostomy. Through a small incision in the and meet on the anterior wall of the urethra at a flank the ureter is brought to the surface in con- lower level. As the name implies, the valves cause a tinuity, opened and sutured to the skin edges. The one-way obstruction; there is no hindrance to retro- method provides free, tubeless urinary drainage and, grade urethral instrumentation but the cusps fall in addition, allows the defunctioned ureter below together during micturition and obstruct the flow of the stoma to regain its muscular tone and contrac- urine through the urethra. Since the obstruction
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