Hassim, A.M.

Kihn, R.

English

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8 February 1969 S.A. MEDICAL JOURNAL 155UNILATERAL POLYCYSTIC KIDNEY WITH RENAL HYPOPLASIA*R. KIHN. M.CH. (CAPE TOWN) AND A. M. HASSIM, M.R.C.O.G., Departments of Surgery and Obstetric.l', LU.l'ClkaCentral HospiTal, ZambiaUnilateral polycystic disease of the kidney is rare as isindicated by the paucity of reports in the literature. It isunilateral in only 3% of cases.' In a large series reportedby Bell' only 2 cases were diagnosed between the agesof 1 month and 25 years. The youngest patient in a reportof 85 cases of polycystic kidney by Waiters and Braaschwas 21 months old."CASE REPORTAn African mother of 3 children was admitted to thelabour ward as an unbooked case on 3 January 1966.General examination was normal. On abdominal examina-tion the uterine fundus was consistent with a pregnancyat term. There was no evidence of hydramnios. She was inadvanced labour. A normal vaginal delivery of a healthymale infant weighing 7 lb. 7 oz. ensued. Routine examina-tion of the infant after birth showed abdominal distensiondue to a partly solid mass measuring about 12 cm. across.The mass was limited in mobility and was bulging intothe left flank. The surface was not clearly defined, but ap-peared lobulated and was resonant. No other abnormalitywas detected. The diagnosis of polycystic kidney or Wilms'stumour was considered.Investigations, including full haematological examina-tion, serum chemistry, blood urea, urinalysis and radio-logical chest examination, were within normal limits. Anintravenous pyelogram showed complete absence of ex-cretion of dye on the left side; the right kidney excretednormally. No abnormality was noted in the calyceal sys-tem of the right kidney. The pelvis, right ureter and blad-. der shadows were normal.On 15 January 1966 laparotomy was performed througha left paramedian incision. A lobulated, tensely cysticmass was present in the position of the left kidney. Theleft ureter appeared as a thin, solid cord. The right renalsystem, liver, spleen and the pancreas appeared normal.Transperitoneal nephrectomy of the abnormal organ wasperformed.Recovery was uneventful and mother and baby weredischarged on the 9th postoperative day. When last seenat follow-up examination 21 months after operation, theinfant was progressing very satisfactorily.Pathologist's ReportA reniform mass measuring 10 x 8 x 7 cm. showedmultiple cysts containing clear fluid. There was a completeabsence of normal architecture. Sections showed a fewinfantile glomeruli surrounded by normal and cystictubules of varying sizes and shapes. No communicationbetween the cysts and renal pelvis was detected in any ofthe sections examined. The blood-vessels were normal.The features were consistent with the diagnosis of con-genital polycystic kidney.DISCUSSIONThe pathogenesis of polycystic disease of the kidney re-'Dale received: 14 May 1968.mains obscure. llildebrandt (quoted by Aird)' postulateda faIlure of the collecting tubules, which develop fromthe Wolffian duct, to join the secreting tubules whichoriginate in the metanephros. Although this is the mostacceptable theory today, it does not explain the associa-tion with similar cysts in other organs.'It is now generally believed that the disease, as it oc-curs in adults, is different from that encountered in in-fants. The adult form is transmitted as a Mendelian domi-nant, while the infantile form is transmitted as a recessivetrait. Tubular cysts in adults communicate with the renalpelvis; those in infants rarely do so.··· The connectivetissue in infants may appear embryonic.' The cysts arelined by flattened to cuboidal epithelium and contain clearor brownish fluid with urate, oxalate, cholesterol, sodium,potassium, chloride, urea, albumin and a few red bloodcells.' The infantile type may be associated with othercongenital abnormalities, such as hydrocephalus, spinabifida, cardiac malformations and, rarely, cysts in thelungs, spleen and pancreas.Clinically, the infantile type of disease is more commonin females. Depending on the ize of the kidneys, ob-structed labour may result, or the mass may be di-covered after birth. According to Cooke: 25~~ of childrenare stillborn or die soon after birth, 4% die in the fir tyear and another 1t'Yo succumb in the first 20 years. Renalrickets may develop in the last group.Intravenous pyelography may demonstrate the typical'spider pelvis', or show a non-functioning kidney as in ourcase.In the differential diagnosis Wilms's tumour, hydrone-phrosis and a solitary renal cyst must be considered.When the condition is bilateral it is not amenable tosurgery. Waiters and Braasch" believe that 'adequatevisualization of the pelvis of but one kidney can be re-garded as sufficient evidence of normal renal function towarrant removal of the other kidney'.SUMMARYA case of unilateral polycystic disease of the kidney, diagnosedat birth, is reported.The clinical and pathological features are reviewed. 'ephrec-tomy is considered the treatment of choice in the case pre-sented.We wish to thank Dr S. B. Gore for the report on thespecimen and pathological slides.REFERENCESI. Aird. 1. (1958): A Companion in Surgical Studies, 2nd ed., p. 1124.Edinburgh: E. & S. Livingslone.2. Bell, E T. (1946): Renal Disease, p. 85. Philadelphia: Lea & Febiger.3. Waiters, W. and Braasch, W. F. (1933): Surg. Gynec. ObsleL. 57, 647.4. Ackerman. L. V. (1964): Surgical Pathology, 3rd ed., p. 5 . SILouis: C. V. Mosby.5. Nelson, W. E. (1964): Textbook of Pediatrics, lh cd., p. JlOO. Phila-delphia: W. B. Saunders.6. Cooke. L. (1936): Lancet. I, 312.

Unilateral polycystic kidney with renal hypoplasia

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Unilateral polycystic kidney with renal hypoplasia

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