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Prenatal sonographic detection of genital malformations
214
INTERNATIONAL
pathic bladder who were still incontinent after bladder neck reconstruction underwent periurethral or pericervical injection. Continence improved in all patients who had a neuropathic bladder and 10 of those with extrophy-epispadias. Bladder capacity increased by 25% in four of the five extrophy patients who were treated to stimulate bladder enhancement. The authors discuss differing possible mechanisms for early and late failure of the technique.-MN. de la Hunt “Congenital” Hydronephrosis: Limitations of Diagnosis by Fetal Ultrasonography. A.M.K. Rickwood, J. P! Hamey, M.O. Jones, eta/. Br J Urol75:529-530, (April), 1995.
Forty-three children born after 1985 who presented clinically with uncomplicated pelviureteric junction obstruction before 1993 were reviewed. Thirty-two had undergone fetal ultrasonography, only five of whom were reported as having minimal or equivocal hydronephrosis, and none of whom were investigated after birth. No abnormality had been reported for any of the seven who were scanned at no later than 30 weeks’ gestation. The age at time of clinical presentation ranged from 3 to 97 months. Twenty-two had symptoms, and in three the abnormality was detected incidentally during investigation of another complaint. Only 14 have undergone surgery: pyeloplasty (12) and nephrectomy (2). This study provides evidence that fetal ultrasonography has limitations in identifying patients who present later with clinical pelviureteric junction obstruction and that the obstruction may develop either late in pregnancy or after birth.--MN. de la Hunt Pyeloplasty Improves Renal Function and Somatic Children With Ureteropelvic Junction Obstruction. and R. Gonzales. J Urol154:218-222, (July), 1995.
Growth in J. Tapia
The systemic effects of pyeloplasty in 38 children with unilateral ureteropelvic junction obstruction were studied. The mean age at the time of surgery was 2.5 years, and 22 patients were I 1 year of age. The mean follow-up period was 18 months. The authors recorded serum creatinine levels, glomerular filtration rate calculated by the Schwartz formula body growth, the degree of dilatation, and the excretory curve of the diuresis renogram. In 36 cases, pyeloplasty was effective in decreasing the degree of ultrasonographic dilatation and the excretory cume of the renogram. Two patients required reoperation. Individual renal function improved significantly in children under 1 year of age with preoperative differential function of less than 45%, but not in older children. The serum creatinine level decreased significantly after pyeloplasty in all children. Likewise, the glomerular filtration rate improved significantly in all children. Preoperatively, 72% of children under 1 year of age were below the 50th percentile for height. The distribution of heights became normal after surgery. Overall, percentile rank for height increased in a significant number of patients of all ages. It is concluded that unilateral hydronephrosis systemically affects body growth and overall renal function. The salutary effect of pyeloplasty goes beyond the affected kidney, and pyeloplasty may be of greater benefit when performed in infancy. The authors believe these parameters must be considered in protocols designed to compare outcomes between the children with hydronephrosis treated surgically and those who are simply observed.George Prenatal Mandell,
W. Holcomb,
Jr
Sonographic Detection of Genital Malformations. B. Bromley, C.A. Peters, ef al. J Ural 153:1994-1996,
J.
(June),
1995. Postnatal clinical and pathological correlation of sonographitally identified genital malformations in 17 fetuses was undertaken
ABSTRACTS
to determine the outcome of these findings. The diagnoses confirmed during autopsy or by postnatal examination and surgery included male (XY) pseudohermaphroditism (2), hypospadias with chordee (3), microphallus (2), cloaca1 anomaly (2), congenital adrenal hyperplasia (3), penoscrotal transposition (2), intraabdominal testes (l), megalourethra (l), and cloaca1 exstrophy variant (1). Additional abnormalities included congenital heart defects, cleft palate, and renal, anorectal, cranial, and cerebral malformations. Four fetuses with a sonographically abnormal-appearing phallus were found to have an endocrine disorder (3 congenital adrenal hyperplasia, 1 panhypopituitarism). Outcomes included two abortions and one neonatal death; the rest of the neonates underwent medical and reconstructive treatment. Prenatal detection of genital abnormalities can be helpful in evaluating the fetuses with severe multisystem anomalies as well as lesions more amenable to correction in the neonatal period. Detection is particularly important in neonates with endocrine disorders and complex genitourinary and anorectal malformations--George W. Holcomb, Jr Enuresis
in Sickle
Cell
Disease.
Griggs, et al. J Urol153:1987-1989,
T.E. Figueroa,
E. Benaim,
XT.
(June), 1995.
The prevalence of enuresis and management options for this condition in sickle cell patients were studied. Ninety-one active patients (6 to 2.1years old) were surveyed for symptoms of primary nocturnal enuresis. Of the 91, 27 (29.6%) had primary nocturnal enuresis. Of those with enuresis, 17 had homozygous sickle cell anemia, five had hemoglobin sickle cell disease, four had sickle cell p+ thalassemia, and one had sickle cell p” thalassemia. Of ten patients who elected to receive intranasal desmopressin acetate, six (60%) had complete or partial resolution of nocturnal enuresis. These data confirm the high prevalence of nocturnal enuresis in patients with sickle cell disease and support the role of desmopressin acetate in their treatment.-George W. Holcomb, Jr Scarring Following Renal Trauma in Children. R. Surana, Khan, and R.J. Fitzgerald. Br J Urol75:663-665, (May), 1995.
A.
Nineteen children with documented renal injury (13 contusions, 4 lacerations, 1 pelviureteric junction disruption, 1 deep laceration) had follow-up for 1 month to 12 years (mean, 5.5 years) with blood pressure measurement and assessment of scarring with ultrasonography and dimercapto-succinic acid (DMSA) scan. Eighteen presented with hematuria and were managed nonoperatively. One with pelviureteric junction disruption presented 3 weeks after injury with abdominal distension and vomiting and underwent nephrectomy after attempted reconstruction. Renal scarring was found in one of 13 who had renal contusion, two of four who had renal laceration, and the one who had rupture and had not had nephrectomy. Transient hypertension was noted in only two. Longterm follow-up with a radionucleide scan was recommended as well as long-term blood-pressure monitoring, although none of the patients had sustained hypertension within the study period.M.N.
de la Hunt
Experience Conjoined
With Twins.
Urol154:563-567,
Urogenital Reconstruction H. Hsu, J. W. Duckett, J.M.
of lschiopagus Templeton, Jr, et al. 5
(August), 1995.
Ischiopagus conjoined twins are joined from the lower chest or abdomen down to the pelvis. The authors reviewed their experience in separating six sets of ischiopagus twins. Their treatment is discussed. In addition, reports on 36 sets of ischiopagus twins are reviewed. If a shared bladder is present, one twin retains it while the other receives a temporary urinary drainage system. Later, the twin without a bladder undergoes reconstruction to create a conti-
INTERNATIONAL
pathic bladder who were still incontinent after bladder neck reconstruction underwent periurethral or pericervical injection. Continence improved in all patients who had a neuropathic bladder and 10 of those with extrophy-epispadias. Bladder capacity increased by 25% in four of the five extrophy patients who were treated to stimulate bladder enhancement. The authors discuss differing possible mechanisms for early and late failure of the technique.-MN. de la Hunt “Congenital” Hydronephrosis: Limitations of Diagnosis by Fetal Ultrasonography. A.M.K. Rickwood, J. P! Hamey, M.O. Jones, eta/. Br J Urol75:529-530, (April), 1995.
Forty-three children born after 1985 who presented clinically with uncomplicated pelviureteric junction obstruction before 1993 were reviewed. Thirty-two had undergone fetal ultrasonography, only five of whom were reported as having minimal or equivocal hydronephrosis, and none of whom were investigated after birth. No abnormality had been reported for any of the seven who were scanned at no later than 30 weeks’ gestation. The age at time of clinical presentation ranged from 3 to 97 months. Twenty-two had symptoms, and in three the abnormality was detected incidentally during investigation of another complaint. Only 14 have undergone surgery: pyeloplasty (12) and nephrectomy (2). This study provides evidence that fetal ultrasonography has limitations in identifying patients who present later with clinical pelviureteric junction obstruction and that the obstruction may develop either late in pregnancy or after birth.--MN. de la Hunt Pyeloplasty Improves Renal Function and Somatic Children With Ureteropelvic Junction Obstruction. and R. Gonzales. J Urol154:218-222, (July), 1995.
Growth in J. Tapia
The systemic effects of pyeloplasty in 38 children with unilateral ureteropelvic junction obstruction were studied. The mean age at the time of surgery was 2.5 years, and 22 patients were I 1 year of age. The mean follow-up period was 18 months. The authors recorded serum creatinine levels, glomerular filtration rate calculated by the Schwartz formula body growth, the degree of dilatation, and the excretory curve of the diuresis renogram. In 36 cases, pyeloplasty was effective in decreasing the degree of ultrasonographic dilatation and the excretory cume of the renogram. Two patients required reoperation. Individual renal function improved significantly in children under 1 year of age with preoperative differential function of less than 45%, but not in older children. The serum creatinine level decreased significantly after pyeloplasty in all children. Likewise, the glomerular filtration rate improved significantly in all children. Preoperatively, 72% of children under 1 year of age were below the 50th percentile for height. The distribution of heights became normal after surgery. Overall, percentile rank for height increased in a significant number of patients of all ages. It is concluded that unilateral hydronephrosis systemically affects body growth and overall renal function. The salutary effect of pyeloplasty goes beyond the affected kidney, and pyeloplasty may be of greater benefit when performed in infancy. The authors believe these parameters must be considered in protocols designed to compare outcomes between the children with hydronephrosis treated surgically and those who are simply observed.George Prenatal Mandell,
W. Holcomb,
Jr
Sonographic Detection of Genital Malformations. B. Bromley, C.A. Peters, ef al. J Ural 153:1994-1996,
J.
(June),
1995. Postnatal clinical and pathological correlation of sonographitally identified genital malformations in 17 fetuses was undertaken
ABSTRACTS
to determine the outcome of these findings. The diagnoses confirmed during autopsy or by postnatal examination and surgery included male (XY) pseudohermaphroditism (2), hypospadias with chordee (3), microphallus (2), cloaca1 anomaly (2), congenital adrenal hyperplasia (3), penoscrotal transposition (2), intraabdominal testes (l), megalourethra (l), and cloaca1 exstrophy variant (1). Additional abnormalities included congenital heart defects, cleft palate, and renal, anorectal, cranial, and cerebral malformations. Four fetuses with a sonographically abnormal-appearing phallus were found to have an endocrine disorder (3 congenital adrenal hyperplasia, 1 panhypopituitarism). Outcomes included two abortions and one neonatal death; the rest of the neonates underwent medical and reconstructive treatment. Prenatal detection of genital abnormalities can be helpful in evaluating the fetuses with severe multisystem anomalies as well as lesions more amenable to correction in the neonatal period. Detection is particularly important in neonates with endocrine disorders and complex genitourinary and anorectal malformations--George W. Holcomb, Jr Enuresis
in Sickle
Cell
Disease.
Griggs, et al. J Urol153:1987-1989,
T.E. Figueroa,
E. Benaim,
XT.
(June), 1995.
The prevalence of enuresis and management options for this condition in sickle cell patients were studied. Ninety-one active patients (6 to 2.1years old) were surveyed for symptoms of primary nocturnal enuresis. Of the 91, 27 (29.6%) had primary nocturnal enuresis. Of those with enuresis, 17 had homozygous sickle cell anemia, five had hemoglobin sickle cell disease, four had sickle cell p+ thalassemia, and one had sickle cell p” thalassemia. Of ten patients who elected to receive intranasal desmopressin acetate, six (60%) had complete or partial resolution of nocturnal enuresis. These data confirm the high prevalence of nocturnal enuresis in patients with sickle cell disease and support the role of desmopressin acetate in their treatment.-George W. Holcomb, Jr Scarring Following Renal Trauma in Children. R. Surana, Khan, and R.J. Fitzgerald. Br J Urol75:663-665, (May), 1995.
A.
Nineteen children with documented renal injury (13 contusions, 4 lacerations, 1 pelviureteric junction disruption, 1 deep laceration) had follow-up for 1 month to 12 years (mean, 5.5 years) with blood pressure measurement and assessment of scarring with ultrasonography and dimercapto-succinic acid (DMSA) scan. Eighteen presented with hematuria and were managed nonoperatively. One with pelviureteric junction disruption presented 3 weeks after injury with abdominal distension and vomiting and underwent nephrectomy after attempted reconstruction. Renal scarring was found in one of 13 who had renal contusion, two of four who had renal laceration, and the one who had rupture and had not had nephrectomy. Transient hypertension was noted in only two. Longterm follow-up with a radionucleide scan was recommended as well as long-term blood-pressure monitoring, although none of the patients had sustained hypertension within the study period.M.N.
de la Hunt
Experience Conjoined
With Twins.
Urol154:563-567,
Urogenital Reconstruction H. Hsu, J. W. Duckett, J.M.
of lschiopagus Templeton, Jr, et al. 5
(August), 1995.
Ischiopagus conjoined twins are joined from the lower chest or abdomen down to the pelvis. The authors reviewed their experience in separating six sets of ischiopagus twins. Their treatment is discussed. In addition, reports on 36 sets of ischiopagus twins are reviewed. If a shared bladder is present, one twin retains it while the other receives a temporary urinary drainage system. Later, the twin without a bladder undergoes reconstruction to create a conti-