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Exstrophy–Epispadias Complex, Pediatric

Timothy S. Baumgartner, FS, MC, Lt Col, USAF and John P. Gearhart, MD Reviewed 10/2018
 


BASICS

DESCRIPTION

The exstrophy–epispadias complex is a rare spectrum of multisystem birth defects involving the genitourinary and gastrointestinal tracts, musculoskeletal system, pelvic floor, and bo...

DIAGNOSIS

PHYSICAL EXAM

  • Bladder exstrophy

    • All cases have pubic diastasis (mean of 4.8 cm) caused by outward rotation of the innominate bones and iliac wings.

    • The exstrophied bladder and posterior urethra...

TREATMENT

SURGERY/OTHER PROCEDURES

Goals: Provide urinary continence with a compliant bladder, preserve renal function, and surgically reconstruct the phallus for cosmesis. 
  • Modern staged repair of blad...

ONGOING CARE

PROGNOSIS

Current treatments preserve the bladder in nearly every patient and allow for continence through the urethra, providing an excellent overall prognosis. There can be favorable lon...

ADDITIONAL READING

  • Ebert AK, Reutter H, Ludwig M, et al. The exstrophy-epispadias complex. Orphanet J Rare Dis.  2009;4:23. [View Abstract on OvidInsights]

  • Friedlander DA, Di Carlo HN, Sponseller ...

CODES

ICD9

  • 753.5 Exstrophy of urinary bladder

  • 752.62 Epispadias

  • 751.5 Other anomalies of intestine

  • 751.2 Atresia and stenosis of large intestine, rectum, and anal canal

ICD10

  • Q64.0 Epispadias

  • Q64.10 Exstroph...

FAQ

  • Q: Will a mother be able to hold and nurse a baby with bladder exstrophy after birth?

  • A: Yes. A mother will be able to nurse and hold her baby immediately after birth. Once the child undergoes init...

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