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- -
ANOMALIES ASSOCIATED WITH MYELODYSPLASIA KARL
J. KREDER,
M.D.
PAUL R. YOUNG, M.D. GORDON WORLEY, M.D. GEORGE
D. WEBSTER,
M.B.,
F.R.C.S.
From the Division of Urology, Department Medical Center, Durham, North Carolina
of Surgery,
Duke
University
ABSTRACTThe records of 181 patients with myelodysplasia were reviewed. The prevalence of associated genitourinary anomalies as well as cardiac, facial, anal, and tethered cord were determined. The prevalence ofcongenital malformations associated with myelodysplasia were less in this series than in previous reports. The association of myelodysplasia with other congenital anomalies has long been recognized; it was first described by Morgagni in 1761.’ Many subsequent reports describing associated genitourinary anomalies have been based on autopsy data, in which case one might expect a higher prevalence of associated anomalies than would be identified in a large cohort of myelodysplastic patients who were alive and we11.2 4 Herein we report on 181 myelodysplastic children who were evaluated at Duke University Medical Center between 1981 and 1989. The prevalence of associated genitourinary anomalies including horseshoe kidney, crossed ectopia, congenital renal absence, and cryptorchidism were noted. The prevalence of several other associated congenital anomalies (cardiac, facial, anal, and tethered cord) were also noted and are reported. Material
and Methods
A total of 181 patients, 96 female and 85 male, with myelomeningocele were evaluated at Duke University Medical Center between 1981 and 1989. The routine assessment of these patients included history, physical examination, determination of hematocrit and serum electrolytes, urinalysis, and urine culture. All patients had a complete urodynamic evaluation including fluoroscopic examination of the bladder and outlet to characterize their neurogenic bladder
248
as well as excretory urography and renal ultrasonography to identify associated renal anomalies. The presence of undescended testes, facial abnormalities, and anal anomalies were noted during the physical examination. Cardiac malformations were diagnosed by echocardiography. Magnetic resonance imaging and, prior to its development, myelography were used to make the diagnosis of tethered cord. Results The spinal cord lesions in this series of 181 patients occurred at all levels (Table I) and did not appear to have any predictive value in determining whether or not other congenital malformations might be present. In this series of patients, no anal abnormalities were noted on physical examination. Cardiac anomalies were diagnosed by echocardiography in 3 patients and included 1 with patent ductus arteriosus, 1 with a small atria1 septal TABIJ: I.
SPine Cervical Thoracic
No. of Patients 3 20
Lumbar Lumbosacral Sacral
UROI,OGY
Level of spinal lesion
38 103 17
/ hlARCI1 I!)92 / VOLUME
Percent 2 11 21 57 9
XXXIX. NUhfRER G
TAHI.P:II.
Renal mmnulirs ~~ -.4nomalies (‘X )-----~------ -~ Cross-Fused linilat. Conq~r~. Renal Ectopia Absent Kidncs!
Horseshoe Kidney
Series DmlC
21181 (1.1)
11181 (0.6)
9181
(I.11
Roberts”
Sheffield* Edin.l’Dublin* f;orhes* ’ Gaum ef al. t’* Wilcock and Emer\.*4 Schwidde~” General population KI
- -
ANOMALIES ASSOCIATED WITH MYELODYSPLASIA KARL
J. KREDER,
M.D.
PAUL R. YOUNG, M.D. GORDON WORLEY, M.D. GEORGE
D. WEBSTER,
M.B.,
F.R.C.S.
From the Division of Urology, Department Medical Center, Durham, North Carolina
of Surgery,
Duke
University
ABSTRACTThe records of 181 patients with myelodysplasia were reviewed. The prevalence of associated genitourinary anomalies as well as cardiac, facial, anal, and tethered cord were determined. The prevalence ofcongenital malformations associated with myelodysplasia were less in this series than in previous reports. The association of myelodysplasia with other congenital anomalies has long been recognized; it was first described by Morgagni in 1761.’ Many subsequent reports describing associated genitourinary anomalies have been based on autopsy data, in which case one might expect a higher prevalence of associated anomalies than would be identified in a large cohort of myelodysplastic patients who were alive and we11.2 4 Herein we report on 181 myelodysplastic children who were evaluated at Duke University Medical Center between 1981 and 1989. The prevalence of associated genitourinary anomalies including horseshoe kidney, crossed ectopia, congenital renal absence, and cryptorchidism were noted. The prevalence of several other associated congenital anomalies (cardiac, facial, anal, and tethered cord) were also noted and are reported. Material
and Methods
A total of 181 patients, 96 female and 85 male, with myelomeningocele were evaluated at Duke University Medical Center between 1981 and 1989. The routine assessment of these patients included history, physical examination, determination of hematocrit and serum electrolytes, urinalysis, and urine culture. All patients had a complete urodynamic evaluation including fluoroscopic examination of the bladder and outlet to characterize their neurogenic bladder
248
as well as excretory urography and renal ultrasonography to identify associated renal anomalies. The presence of undescended testes, facial abnormalities, and anal anomalies were noted during the physical examination. Cardiac malformations were diagnosed by echocardiography. Magnetic resonance imaging and, prior to its development, myelography were used to make the diagnosis of tethered cord. Results The spinal cord lesions in this series of 181 patients occurred at all levels (Table I) and did not appear to have any predictive value in determining whether or not other congenital malformations might be present. In this series of patients, no anal abnormalities were noted on physical examination. Cardiac anomalies were diagnosed by echocardiography in 3 patients and included 1 with patent ductus arteriosus, 1 with a small atria1 septal TABIJ: I.
SPine Cervical Thoracic
No. of Patients 3 20
Lumbar Lumbosacral Sacral
UROI,OGY
Level of spinal lesion
38 103 17
/ hlARCI1 I!)92 / VOLUME
Percent 2 11 21 57 9
XXXIX. NUhfRER G
TAHI.P:II.
Renal mmnulirs ~~ -.4nomalies (‘X )-----~------ -~ Cross-Fused linilat. Conq~r~. Renal Ectopia Absent Kidncs!
Horseshoe Kidney
Series DmlC
21181 (1.1)
11181 (0.6)
9181
(I.11
Roberts”
Sheffield* Edin.l’Dublin* f;orhes* ’ Gaum ef al. t’* Wilcock and Emer\.*4 Schwidde~” General population KI
