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| IMAGES IN NEPHROLOGY |
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| Year : 2012 | Volume
: 22
| Issue : 6 | Page : 482-483 |
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Story behind the wrinkle
R Jha1, D Gude2, S Sinha3
1 Department of Nephrology, Medwin Hospital, Nampally, Hyderabad, Andhra Pradesh, India
2 Department of Internal Medicine, Medwin Hospital, Nampally, Hyderabad, Andhra Pradesh, India
3 Department of Urology, Medwin Hospital, Nampally, Hyderabad, Andhra Pradesh, India
| Date of Web Publication | 14-Jan-2013 |
Correspondence Address:
R Jha
Consultant, Department of Nephrology, Medwin Hospital, Nampally, Hyderabad, Andhra Pradesh - 500 001
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0971-4065.106053
How to cite this article:
Jha R, Gude D, Sinha S. Story behind the wrinkle. Indian J Nephrol 2012;22:482-3 |
A 16-year-old boy born of a consanguineous marriage presented for evaluation of recurrent urinary tract infections (UTI) from the age of 2 years. His urine stream was slow and he had sense of incomplete bladder emptying, increased urinary frequency, and nocturia three to four times per night. Examination revealed a marfanoid body habitus, and absent testes with minimal secondary sexual characteristics. The lax and wrinkled abdomen wall depicted the absence of abdominal muscles [Figure 1]. Ultrasonography confirmed bilateral cryptorchidism and bilateral hydronephrosis. His serum creatinine was 2.3 mg/dl. A plain X-ray abdomen at the age of 2 years [Figure 2] revealed absent abdominal musculature and visceroptosis. Micturating cystourethrogram at the age of 8 years showed bilateral grade IV vesicoureteric reflex, with kidney and ureter shifted to extreme right due to the lax abdominal wall [Figure 3] and no other genitourinary abnormalities. A diagnosis of prune belly syndrome was confirmed. | Figure 1: Lax, wrinkled abdominal wall of a patient with prune belly syndrome
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 | Figure 2: A plain X-ray abdomen done the age of 2 years depicting absent abdominal musculature and the resultant visceroptosis
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 | Figure 3: Micturating cystourethrogram done at the age of 8 years showing bilateral vesicouretric reflex (grade IV) with kidney and ureter (dilated) shifted to extreme right due to the lax abdominal wall
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He presented to us at the age of 16 years. Urodynamic study showed acontractile large capacity (1,200 ml) bladder with normal compliance and safe end-fill pressure during storage phase and abdominal strain pattern during voiding phase together with a large residual urine volume. He was started on clean intermittent self-catheterization (CISC) with low-dose ciprofloxacin (100 mg) for 6 weeks and standard care for chronic kidney disease (CKD). He subsequently underwent laparoscopic bilateral orchidopexy. At a follow-up of 7 years, he is doing well (stable serum creatinine at 2.2 mg/dl) with excellent somatic growth. There were no further hospitalizations for urosepsis despite no prophylaxis.
Prune belly syndrome (also called Eagle-Barrett syndrome More Details/Triad syndrome) is characterized by the triad of deficient abdominal musculature, urinary tract anomalies, and cryptorchidism. [1] It has an incidence of about 1 in 50,000 live births with 95% being male. [2] As depicted in our case, the skin of the anterior abdominal wall is wrinkled, thin, and lax, giving it a prune-like appearance. Apart from absent abdominal musculature (complete in 30%, partial in 70%) [3] and cryptorchidism (in about 95%), renal hypoplasia, dysplasia, ureteral dilatation, megacystis, dilated prostatic urethra, and prostatic hypoplasia are commonly seen.
CISC plays a salutary role in dealing with such congenital anomalies in preventing recurrences of UTI and the progression of CKD, as exemplified in our case.
| Acknowledgments | |  |
We thank our colleagues and staff of Nephrology and Internal Medicine.
| References | | |
| 1. | Bogart MM, Arnold HE, Greer KE. Prune-belly syndrome in two children and review of the literature. Pediatr Dermatol 2006;23:342-5. 
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| 2. | Herman TE, Siegel MJ. Prune belly syndrome. J Perinatol 2009;29:69-71.
[PUBMED] |
| 3. | Reinberg Y, Shapiro E, Manivel JC, Manley CB, Pettinato G, Gonzalez R. Prune belly syndrome in females: A triad of abdominal musculature deficiency and anomalies of the urinary and genital systems. J Pediatr 1991;118:395-8.
[PUBMED] |
[Figure 1], [Figure 2], [Figure 3]
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