Monday, February 10, 2014

Dr. John P. Gearhart closes 100th Cloacal Exstrophy at Johns Hopkins

Cloacal exstrophy is one of the most severe congenital anomalies compatible with life. Fortunately, cloacal exstrophy is rare, occurring in 1 of 200,000 to 400,000 live births and can be diagnosed reliably prenatally.[1]   Historically, surgical reconstruction of cloacal exstrophy was considered futile and untreated neonates died from prematurity, short bowel syndrome, sepsis or renal and central nervous system abnormalities.  Over the past few decades, improvements in surgical technique and anesthesia have improved the outcomes of children born with cloacal exstrophy so that nearly 100% of children survive into adulthood.  Modem treatment of cloacal exstrophy includes a number of goals that were previously unobtainable, namely obtaining secure abdominal and bladder closure; preserving renal function; achieving satisfactory continence; obtaining functional, cosmetically pleasing genitalia, and preventing the short bowel syndrome.[2-5]

John P. Gearhart, MD
The Brady Urological Institute and Department of Urology at Johns Hopkins Hospital has a long history of treating children with bladder- and cloacal-exstrophy.  In 1975, Robert Douglas Jeffs, FRCS, Chief of Pediatric Urology at the University of Toronto Sick Children's Hospital, came to the Johns Hopkins Children's Center as the first Professor of Pediatric Urology.  He pioneered the modern techniques of exstrophy closure with osteotomies and improved long-term renal function.  John P. Gearhart, MD, Professor and Director of Pediatric Urology trained under and succeeded Dr. Jeffs in 1996. Tremendous improvements have been made in the treatment of children with the exstrophy spectrum during the last 10 years.  These include improvements in the understanding of differences in collagen, smooth muscle, neural innervation and bony anatomy at the cellular level in children with exstrophy as well as clinical improvements involving osteotomies, pain control and immobilization and continence surgeries have increased success rates and outcomes. [6]

In the last year, Dr. Gearhart led the team that closed the 100th child with cloacal exstrophy at the Johns Hopkins Children's Center.  This represents the world's largest experience with cloacal exstrophy and two lifetimes of dedication and work to this disease and the children afflicted by it.  See the video, "A Milestone in Pediatric Urology" below.




The outcomes for these 100 children were recently presented at the American Academy of Pediatrics (AAP) Annual Meeting in Orlando, Florida.  Sixty patients had complete follow-up at a median of 9 years (range 1-19 years); 26 patients failed their initial closure and 34 had a successful primary closure.  Children with the highest likelihood of successful primary closure had smaller diastasis of their pelvis, were closed with both osteotomies and an external fixator; and had a delay between osteotomy and abdominal closure.

For more information, please visit Division of Pediatric Urology at the Johns Hopkins Children's Center.



[1] Meizner, I. and Bar-Ziv, J.: In utero prenatal ultrasonic diagnosis of a rare case of cloacal exstrophy. J. Clin. Ultrasound, 13: 500, 1985. 
[2] Gearhart JP, Mathews R. Exstrophy-epispadias complex. In: Wein AJ, Kavoussi LR, Partin AW, Peters CA, Novick AC, editors. Campbell-Walsh Urology. 10th ed. Philadelphia: Elsevier; 2012. p. 3497-553.
[3] Gearhart, J. P. and Jeffs, R. D.: Techniques to create urinary continence in the cloacal exstrophy patient. J. Urol., part 2, 146: 616,1991. 
[4] Mathews R, Jeffs RD, Reiner WG, Docimo SG, Gearhart JP. Cloacal exstrophy--improving the quality of life: the Johns Hopkins experience. The Journal of urology. 1998 Dec;160(6 Pt 2):2452-6.
[5] Lund DP, Hendren WH. Cloacal exstrophy: a 25-year experience with 50 cases. Journal of pediatric surgery. 2001 Jan;36(1):68-75.
[6] The Exstrophy-Epispadias-Cloacal Exstrophy Spectrum: A New Appraisal. John P. Gearhart, MD (Guest Editor), Cenk S.N. Buyukunal, MD (Guest Editor).  Seminars in Pediatric Surgery, Volume 20, Issue 2, May 2011, Pages 61.

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  2. I love dr Heather my son James neal was born with this my son will be 9 on Halloween I'm so thankful and bless...I just couldn't believe it...still more surgery to go but I leave it in their hands...thanks so much stronger gear Hart and your team...see y'all soon...riva.

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