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Prune Belly Syndrome Associated With Exomphalos and Anorectal Agenesis
882
PEDIATRIC UROLOGY
lateralized to 1 kidney, 5 had renovascular hypertension, 2 had renal vein thrombosis, 1 had an angiomatous malformation and 1 had glomerulonephritis. Fourteen patients underwent nephrectomy and 2 underwent partial nephrectomy. Of these patients 11 were normotensive. Postoperatively, the authors note that if hypertension develops, is persistent and is associated with a minimally functioning kidney and a normal contralateral kidney, nephrectomy (partial or total) will be therapeutic irrespective of the renal vein renins. The authors conclude that the risk for hypertension remains for years in the presence of scarred kidneys, borderline hypertension may not be progressive, nephrectomy is curative in the presence of a normal contralateral kidney and nephrectomy, although of some benefit in the short-term, is not helpful in the long-term in the presence of multiple scars in the contralateral kidney. R. K. 5 tables, 5 references
Editorial comment. This is a large series of children with hypertension secondary to surgically treated (usually unilateral) renal disease. To these I might add 2 personal cases of multicystic kidney in whom hypertension reverted to normal after surgical removal. Only 3 other such cases are reported in the literature but in an informal poll of pediatric urologists approximately 15 per cent indicated that they had seen hypertension in conjunction with a multicystic kidney. We must determine if these cases are being underreported because many are electing to follow multicystic kidneys, since the diagnosis usually can be made with precision by ultrasonography and renal scan. L. R. K.
PREOPERATIVE AND POSTOPERATIVE THERAPY Anatomical Considerations Relevant to Fecal Continence
Department of Pediatric Surgery, Schneider Children's Hospital of Long Island Jewish Medical Center, New Hyde Park, New York and State University of New York at Stony Brook, Stony Brook, New York
A. PENA,
Semi. Surg. Oncol., 3: 141-145, 1987 The author presents a surgeon'f1 guide to the anatomical findings he considers relevant to the maintenance of fecal continence based on his extensive experience in children. The approach is somewhat different from the typical anatomist in that he recognizes how operative anatomy frequently varies from textbook description. Several practical suggestions to maintain fecal continence postoperatively are made, including use of a posterior sagittal approach and of a muscle stimulator intraoperatively. Finally, the value of meticulous reapproximation of anatomical structures is emphasized. M. J. S. 3 figures, 16 references
PEDIATRIC UROLOGY Prune Belly Syndrome Associated With Exomphalos and Anorectal Agenesis
J.
WALKER, A. I. PROKURAT AND I. M. IRVING, Department of Paediatric Surgery, University of Liverpool, Alder Hey Children's Hospital, Liverpool, England
J. Ped. Surg., 22: 215-217 (Mar.) 1987
A neonate with the prune belly syndrome associated with exomphalos and anorectal agenesis is described. Significant pulmonary problems ensued, confirming the susceptibility of prune belly syndrome patients to respiratory problems. The authors have seen 13 patients with the prune belly syndrome, of whom 4 had exomphalos and 5 had anorectal agenesis. The coexistence of these anomalies suggests a disturbance of early embryogenesis as opposed to theories of obstruction or muscular weakness. The authors also note that gastroschisis has been reported in the prune belly syndrome, and they suggest that there may be a unifying theory of embryogenesis of gastroschisis and exomphalos. R. K. 2 figures, 1 table, 15 references
Editorial comment. We have not encountered a boy with the prune belly syndrome and an imperforate anus but we have noted chest deformity, at least the flared costal margins, in almost all patients. Pectus excavation also is common and megalourethra occasionally is associated. In our experience most of the valves seen in conjunction with the prune belly syndrome are of the type 4 variety and they seem to be loose urethral mucosa from the prostatic urethra. The mucosa slides down into the region of the external sphincter during voiding and this may cause severe obstruction. This has been postulated to be the mechanism in utero that causes the bladder to become dilated and hydronephrosis usually also occurs. The bladder becomes so large that migration of somatic musculature is blocked in the central region of the lower abdomen resulting in the prune belly syndrome (Straub, E. and Spranger, J.: Kidney Int., 20: 695, 1981). L. R. K.
Urinary Bladder Innervation in Children D. M. KULLENDORFF, M. ELMER AND P. ALM, Departments of
Pediatric Surgery, Physiology and Pathology, University of Lund, Lund, Sweden
J. Ped. Surg., 22: 240-242 (Mar.) 1987 Tissue specimens were obtained from the trigone, fundus of the bladder and distal ureter of children undergoing an antireflux operation. These muscle strips were studied with histochemical and immunohistochemical methods as well as by electrical nerve stimulation in an organ bath. Cholinergic nerves were found and the transmitter causing contraction of the detrusor was acetylcholine. No contractile a-adrenoceptors could be found but /3-receptor-mediated relaxation was noted. lmipramine had no anticholinergic effects on the bladder at doses that are used clinically, which suggests that imipramine, widely used for enuresis, acts on the central nervous system and not on the bladder. Terodiline, an anticholinergic and calcium antagonist, inhibited all activity, which suggests that this drug would be suitable for the treatment of enuretic children with small functional capacity bladders. R. K. 20 references
Editorial comment. Cussen measured the size of ureteral muscle cells years ago and he found that the cell size in dilated ureters with reflux was increased as much as 3-fold. This finding did not approach the hypertrophy of the ureteral muscle cells seen in obstruction where the cell size actually could become 40 times normal. One can distinguish reflux megaureter from obstructive megaureter with biopsy, in most instances, if cell size is measured. There currently is hope that bladder biopsies eventually may
PEDIATRIC UROLOGY
lateralized to 1 kidney, 5 had renovascular hypertension, 2 had renal vein thrombosis, 1 had an angiomatous malformation and 1 had glomerulonephritis. Fourteen patients underwent nephrectomy and 2 underwent partial nephrectomy. Of these patients 11 were normotensive. Postoperatively, the authors note that if hypertension develops, is persistent and is associated with a minimally functioning kidney and a normal contralateral kidney, nephrectomy (partial or total) will be therapeutic irrespective of the renal vein renins. The authors conclude that the risk for hypertension remains for years in the presence of scarred kidneys, borderline hypertension may not be progressive, nephrectomy is curative in the presence of a normal contralateral kidney and nephrectomy, although of some benefit in the short-term, is not helpful in the long-term in the presence of multiple scars in the contralateral kidney. R. K. 5 tables, 5 references
Editorial comment. This is a large series of children with hypertension secondary to surgically treated (usually unilateral) renal disease. To these I might add 2 personal cases of multicystic kidney in whom hypertension reverted to normal after surgical removal. Only 3 other such cases are reported in the literature but in an informal poll of pediatric urologists approximately 15 per cent indicated that they had seen hypertension in conjunction with a multicystic kidney. We must determine if these cases are being underreported because many are electing to follow multicystic kidneys, since the diagnosis usually can be made with precision by ultrasonography and renal scan. L. R. K.
PREOPERATIVE AND POSTOPERATIVE THERAPY Anatomical Considerations Relevant to Fecal Continence
Department of Pediatric Surgery, Schneider Children's Hospital of Long Island Jewish Medical Center, New Hyde Park, New York and State University of New York at Stony Brook, Stony Brook, New York
A. PENA,
Semi. Surg. Oncol., 3: 141-145, 1987 The author presents a surgeon'f1 guide to the anatomical findings he considers relevant to the maintenance of fecal continence based on his extensive experience in children. The approach is somewhat different from the typical anatomist in that he recognizes how operative anatomy frequently varies from textbook description. Several practical suggestions to maintain fecal continence postoperatively are made, including use of a posterior sagittal approach and of a muscle stimulator intraoperatively. Finally, the value of meticulous reapproximation of anatomical structures is emphasized. M. J. S. 3 figures, 16 references
PEDIATRIC UROLOGY Prune Belly Syndrome Associated With Exomphalos and Anorectal Agenesis
J.
WALKER, A. I. PROKURAT AND I. M. IRVING, Department of Paediatric Surgery, University of Liverpool, Alder Hey Children's Hospital, Liverpool, England
J. Ped. Surg., 22: 215-217 (Mar.) 1987
A neonate with the prune belly syndrome associated with exomphalos and anorectal agenesis is described. Significant pulmonary problems ensued, confirming the susceptibility of prune belly syndrome patients to respiratory problems. The authors have seen 13 patients with the prune belly syndrome, of whom 4 had exomphalos and 5 had anorectal agenesis. The coexistence of these anomalies suggests a disturbance of early embryogenesis as opposed to theories of obstruction or muscular weakness. The authors also note that gastroschisis has been reported in the prune belly syndrome, and they suggest that there may be a unifying theory of embryogenesis of gastroschisis and exomphalos. R. K. 2 figures, 1 table, 15 references
Editorial comment. We have not encountered a boy with the prune belly syndrome and an imperforate anus but we have noted chest deformity, at least the flared costal margins, in almost all patients. Pectus excavation also is common and megalourethra occasionally is associated. In our experience most of the valves seen in conjunction with the prune belly syndrome are of the type 4 variety and they seem to be loose urethral mucosa from the prostatic urethra. The mucosa slides down into the region of the external sphincter during voiding and this may cause severe obstruction. This has been postulated to be the mechanism in utero that causes the bladder to become dilated and hydronephrosis usually also occurs. The bladder becomes so large that migration of somatic musculature is blocked in the central region of the lower abdomen resulting in the prune belly syndrome (Straub, E. and Spranger, J.: Kidney Int., 20: 695, 1981). L. R. K.
Urinary Bladder Innervation in Children D. M. KULLENDORFF, M. ELMER AND P. ALM, Departments of
Pediatric Surgery, Physiology and Pathology, University of Lund, Lund, Sweden
J. Ped. Surg., 22: 240-242 (Mar.) 1987 Tissue specimens were obtained from the trigone, fundus of the bladder and distal ureter of children undergoing an antireflux operation. These muscle strips were studied with histochemical and immunohistochemical methods as well as by electrical nerve stimulation in an organ bath. Cholinergic nerves were found and the transmitter causing contraction of the detrusor was acetylcholine. No contractile a-adrenoceptors could be found but /3-receptor-mediated relaxation was noted. lmipramine had no anticholinergic effects on the bladder at doses that are used clinically, which suggests that imipramine, widely used for enuresis, acts on the central nervous system and not on the bladder. Terodiline, an anticholinergic and calcium antagonist, inhibited all activity, which suggests that this drug would be suitable for the treatment of enuretic children with small functional capacity bladders. R. K. 20 references
Editorial comment. Cussen measured the size of ureteral muscle cells years ago and he found that the cell size in dilated ureters with reflux was increased as much as 3-fold. This finding did not approach the hypertrophy of the ureteral muscle cells seen in obstruction where the cell size actually could become 40 times normal. One can distinguish reflux megaureter from obstructive megaureter with biopsy, in most instances, if cell size is measured. There currently is hope that bladder biopsies eventually may