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Early Second Trimester Intervention In A Surviving Infant With Postnatally Diagnosed Urethral Atresia
0022-5347/02/1684-1532/0 THE JOURNAL OF UROLOGY® Copyright © 2002 by AMERICAN UROLOGICAL ASSOCIATION, INC.®
Vol. 168, 1532–1533, October 2002 Printed in U.S.A.
DOI: 10.1097/01.ju.0000030160.46045.b3
EARLY SECOND TRIMESTER INTERVENTION IN A SURVIVING INFANT WITH POSTNATALLY DIAGNOSED URETHRAL ATRESIA C. D. ANTHONY HERNDON
AND
ANTHONY J. CASALE
From the Riley Hospital for Children, Indianapolis, Indiana KEY WORDS: urethral obstruction, bladder outlet obstruction, fetal diseases, urologic surgical procedures, prenatal diagnosis
Indications for prenatal intervention are few, and ideally this approach should be restricted to cases presenting late in the second trimester with oligohydramnios, bilateral ureteral obstruction and favorable urine electrolytes. We report on a patient with urethral atresia who survived renal transplantation following the parents’ decision to intervene prenatally despite an early second trimester presentation and poor urine electrolytes. CASE REPORT
A 37-year-old woman (gravida IV, para II) was referred for screening fetal ultrasound at 14 weeks of gestation. The fetus was identified with a massively dilated bladder and normal amniotic fluid. At 15 weeks of gestation level III ultrasound demonstrated oligohydramnios, bilateral echogenic kidneys with hydronephrosis and the “keyhole” sign indicating profound bladder outlet obstruction (fig. 1). The remaining organ systems were unremarkable. Serial bladder electrolytes were obtained at 15, 16 and 17 weeks of gestation (see table). The parents decided to intervene despite a poor prognosis, and at 18 weeks of gestation a vesico-amniotic shunt was placed with immediate decompression of the bladder and restoration of amniotic fluid. At 30 weeks of gestation oligohydramnios was detected despite a decompressed urinary system, and elective cesarean section was performed at 34 weeks of betamethasone treatment. Peritoneal dialysis was instituted in the first week of life to manage renal insufficiency and oliguria. Since neonatal evaluation suggested bladder outlet obstruction, vesicostomy was performed. At age 14 months a 2 cm. proximal congenital urethral atretic segment was identified and serially dilated allowing closure of the vesicostomy (fig. 2). At age 30 months bilateral reimplantation with ureterocystoplasty was performed in anAccepted for publication May 24, 2002.
ticipation of a living related kidney transplant, which was ultimately successful. At age 36 months the patient is stable with a creatinine of 0.6 mg./dl. and is voiding without difficulty. DISCUSSION
Profound bladder outlet obstruction detected early in the second trimester is considered a lethal condition in the absence of intervention. Institutions that specialize in antenatal intervention report a survival rate of 33% in the presence of poor serial urine electrolytes.1 Our case highlights several interesting points, including age at detection (14 weeks of gestation), age at intervention (15 to 18 weeks of gestation) and neonatal survival despite the postnatal diagnosis. Urethral atresia is a rare congenital abnormality whose antenatal detection and subsequent intervention have rarely been documented.2 To our knowledge this case represents the earliest detection and intervention in a patient with urethral atresia who survived. Uniform guidelines concerning the recommendation of antenatal intervention for urological disorders do not exist. However, there is a general consensus among pediatric urologists not to recommend intervention early in the second trimester for severe obstruction in the presence of bilateral cystic kidneys.3 Despite evidence supporting a bleak outcome, the wishes of the family prevailed, intervention preserved pulmonary development, and the child survived and is
Sodium Chloride Osmolality Wks. (normal less (normal less (normal less Gestation than 100 than 90 mg./ than 200 mg./dl.) dl.) mOsm./l.) 15 16 17
91 114 108
72 91 85
200 267 236
Calcium Protein (normal less (normal less than 8 mg./ than 20 mg./ dl.) dl.) 5.6 7.8 9.2
19.0 56.5 32.3
FIG. 1. Sagittal level III ultrasound at 15 weeks of gestation FIG. 2. Combined antegrade via vesicostomy/retrograde urethroshows classic “keyhole” sign. gram reveals 2 cm. atretic urethral segment (arrows). 1532
INTRAUTERINE INTERVENTION IN URETHRAL ATRESIA
thriving. This is not to diminish the great commitment of resources from the family and the health care system that were needed to support and achieve this success. REFERENCES
1. Johnson, M. P., Bukowski, T. P., Reitleman, C., Isada, N. B., Pryde, P. G. and Evans, M. I.: In utero surgical treatment of fetal obstructive uropathy: a new comprehensive approach to
1533
identify appropriate candidates for vesicoamniotic shunt therapy. Am J Obstet Gynecol, 170: 1770, 1994 2. Freedman, A. L., Bukowski, T. P., Smith, C. A., Evans, M. I., Johnson, M. P. and Gonzalez, R.: Fetal therapy for obstructive uropathy: specific outcomes diagnosis. J Urol, 156: 720, 1996 3. Herndon, C. D. A., Ferrer, F. A., Freedman, A. and McKenna, P. H.: Consensus on the prenatal management of antenatally detected urological abnormalities. J Urol, 164: 1052, 2000
Vol. 168, 1532–1533, October 2002 Printed in U.S.A.
DOI: 10.1097/01.ju.0000030160.46045.b3
EARLY SECOND TRIMESTER INTERVENTION IN A SURVIVING INFANT WITH POSTNATALLY DIAGNOSED URETHRAL ATRESIA C. D. ANTHONY HERNDON
AND
ANTHONY J. CASALE
From the Riley Hospital for Children, Indianapolis, Indiana KEY WORDS: urethral obstruction, bladder outlet obstruction, fetal diseases, urologic surgical procedures, prenatal diagnosis
Indications for prenatal intervention are few, and ideally this approach should be restricted to cases presenting late in the second trimester with oligohydramnios, bilateral ureteral obstruction and favorable urine electrolytes. We report on a patient with urethral atresia who survived renal transplantation following the parents’ decision to intervene prenatally despite an early second trimester presentation and poor urine electrolytes. CASE REPORT
A 37-year-old woman (gravida IV, para II) was referred for screening fetal ultrasound at 14 weeks of gestation. The fetus was identified with a massively dilated bladder and normal amniotic fluid. At 15 weeks of gestation level III ultrasound demonstrated oligohydramnios, bilateral echogenic kidneys with hydronephrosis and the “keyhole” sign indicating profound bladder outlet obstruction (fig. 1). The remaining organ systems were unremarkable. Serial bladder electrolytes were obtained at 15, 16 and 17 weeks of gestation (see table). The parents decided to intervene despite a poor prognosis, and at 18 weeks of gestation a vesico-amniotic shunt was placed with immediate decompression of the bladder and restoration of amniotic fluid. At 30 weeks of gestation oligohydramnios was detected despite a decompressed urinary system, and elective cesarean section was performed at 34 weeks of betamethasone treatment. Peritoneal dialysis was instituted in the first week of life to manage renal insufficiency and oliguria. Since neonatal evaluation suggested bladder outlet obstruction, vesicostomy was performed. At age 14 months a 2 cm. proximal congenital urethral atretic segment was identified and serially dilated allowing closure of the vesicostomy (fig. 2). At age 30 months bilateral reimplantation with ureterocystoplasty was performed in anAccepted for publication May 24, 2002.
ticipation of a living related kidney transplant, which was ultimately successful. At age 36 months the patient is stable with a creatinine of 0.6 mg./dl. and is voiding without difficulty. DISCUSSION
Profound bladder outlet obstruction detected early in the second trimester is considered a lethal condition in the absence of intervention. Institutions that specialize in antenatal intervention report a survival rate of 33% in the presence of poor serial urine electrolytes.1 Our case highlights several interesting points, including age at detection (14 weeks of gestation), age at intervention (15 to 18 weeks of gestation) and neonatal survival despite the postnatal diagnosis. Urethral atresia is a rare congenital abnormality whose antenatal detection and subsequent intervention have rarely been documented.2 To our knowledge this case represents the earliest detection and intervention in a patient with urethral atresia who survived. Uniform guidelines concerning the recommendation of antenatal intervention for urological disorders do not exist. However, there is a general consensus among pediatric urologists not to recommend intervention early in the second trimester for severe obstruction in the presence of bilateral cystic kidneys.3 Despite evidence supporting a bleak outcome, the wishes of the family prevailed, intervention preserved pulmonary development, and the child survived and is
Sodium Chloride Osmolality Wks. (normal less (normal less (normal less Gestation than 100 than 90 mg./ than 200 mg./dl.) dl.) mOsm./l.) 15 16 17
91 114 108
72 91 85
200 267 236
Calcium Protein (normal less (normal less than 8 mg./ than 20 mg./ dl.) dl.) 5.6 7.8 9.2
19.0 56.5 32.3
FIG. 1. Sagittal level III ultrasound at 15 weeks of gestation FIG. 2. Combined antegrade via vesicostomy/retrograde urethroshows classic “keyhole” sign. gram reveals 2 cm. atretic urethral segment (arrows). 1532
INTRAUTERINE INTERVENTION IN URETHRAL ATRESIA
thriving. This is not to diminish the great commitment of resources from the family and the health care system that were needed to support and achieve this success. REFERENCES
1. Johnson, M. P., Bukowski, T. P., Reitleman, C., Isada, N. B., Pryde, P. G. and Evans, M. I.: In utero surgical treatment of fetal obstructive uropathy: a new comprehensive approach to
1533
identify appropriate candidates for vesicoamniotic shunt therapy. Am J Obstet Gynecol, 170: 1770, 1994 2. Freedman, A. L., Bukowski, T. P., Smith, C. A., Evans, M. I., Johnson, M. P. and Gonzalez, R.: Fetal therapy for obstructive uropathy: specific outcomes diagnosis. J Urol, 156: 720, 1996 3. Herndon, C. D. A., Ferrer, F. A., Freedman, A. and McKenna, P. H.: Consensus on the prenatal management of antenatally detected urological abnormalities. J Urol, 164: 1052, 2000