Orthopaedic Management of Bladder Exstrophy

Author(s):  
R. Özgür Özer

Bladder exstrophy is an embryologic malformation that affects urogenital and skeletal systems. Non-operative treatment of this rare problem is impossible. Urogenital reconstructions can be facilitated by orthopedic procedures. These reconstructions can be performed in a single stage as a complete repair or multi-stage approaches. The goal of the treatment is closure of the bladder and abdominal wall for the achievement of continence, preservation of renal functions, and cosmetic and functional reconstruction of genital organs. Orthopedic procedures are performed to decrease the tension that complicates the bladder and abdominal wall closure by approximating the pubic rami to achieve a secure closure and a low recurrence rate. Surgical interventions consist of the approximation of the pubic rami with different materials such as suture materials and plaque or the application of different osteotomy types such as posterior iliac, anterior pelvic (pubic), diagonal iliac, horizontal iliac and posterior pelvic resection osteotomies. The age of the patient, the amount of pubic diastasis and history of previous operations that the patient has undergone should be considered during the operation planning. Pubic rami can be approximated without performing pelvic osteotomy in patients who are operated within the first 72 hours after birth. But, osteotomy is required in children older than 2 years of age with severe pubic diastasis, concomitant cloacal exstrophy and unsuccessful operation history. The surgical team should have enough knowledge and experience to perform different osteotomy types in case of need to combine anterior and posterior iliac osteotomies. With these multidisciplinary approaches, much more successful outcomes could be achieved.

2017 ◽  
Vol 05 (01) ◽  
pp. e57-e59
Author(s):  
Alejandra Vilanova-Sánchez ◽  
Christina Ching ◽  
Alessandra Gasior ◽  
Karen Diefenbach ◽  
Richard Wood ◽  
...  

AbstractCloacal exstrophy is the most severe type of anorectal malformations that belongs to the bladder–exstrophy–epispadias complex of genitourinary malformations. Interestingly, its variant, the covered cloacal exstrophy, is often missed. The clinical findings of this variant may include an imperforate anus, low lying umbilicus, thick pubic bone, and pubic diastasis but with an intact abdominal wall. We present an interesting case of covered cloacal exstrophy with a side-by-side duplicated bladder and discuss important considerations for the time of colostomy creation in the newborn period.


2019 ◽  
Author(s):  
Timothy S. Baumgartner ◽  
John P. Gearhart

This chapter details the latest surgical advances and outcomes in the modern surgical management of male classic bladder exstrophy to include patient selection for closure, operative considerations, newborn primary bladder and posterior urethral closure, early epispadias repair, bladder neck reconstruction with an antireflux procedure, and postoperative management. It highlights how to achieve the primary objectives of (1) a secure abdominal closure, (2) reconstruction of a functional and cosmetically acceptable penis, and (3) urinary continence with the preservation of renal function. In addition, it addresses the most common pitfalls and challenges encountered when accomplishing each of the major surgical interventions. This review contains 3 figures, 5 tables, and 47 references. Key Words: Congenital defect, Bladder exstrophy, Epispadias, Reconstruction, Urinary Continence, Magnetic Resonance Imaging, Pain management, Pelvic osteotomy


Author(s):  
Andrew H. Shitta ◽  
Mercy W. Isichei ◽  
Ezekiel D. Dung ◽  
Solomon D. Peter ◽  
Michael B. Ode ◽  
...  

Background: Congenital anterior abdominal wall defects (AAWD) is a spectrum of abdominal wall defects that includes omphalocele, gastroschisis, bladder exstrophy, cloacal exstrophy, prune belly syndrome and pentalogy of Cantrell. Early Prenatal diagnosis of AAWD provides opportunity for abnormal karyotypes screening and planned delivery in a specialized centre. Ultrasound can detect these defects during pregnancy. This study aims to evaluate the detection rate of AAWD during routine obstetric ultrasonography in our region.Methods: A retrospective study of all patients that presented with AAWD to our centre from January 2008 to July 2020. Data included patient’s age, sex, birth weight, diagnosis, resuscitation time, outcome, maternal age, parity and antenatal ultrasound scan (USS) records. Antenatal USS before 12 weeks only, were excluded. Data analysed using excel.Results: Of the 140 with AAWD, 84.29% had omphalocele, 10% gastroschisis, 2.14% prune belly syndrome and 0.71% each with bladder exstrophy, cloacal exstrophy and pentalogy of Cantrell. There were 123 booked pregnancies. Majority (112) had antenatal care elsewhere while 11 attended our Centre. Ultrasonography of 108 pregnancies scanned at12 weeks or beyond, had 4 confirmed prenatal diagnosis of AAWD. All done in our centre. Mean gestational age at diagnosis was 24weeks. Outcome was rupture1 (25%) and 25% mortality (prenatally diagnosed) and 51.92% mortality for patients with missed diagnosis.Conclusions: Our obstetric ultrasound detection rate of AAWD is very low. There is a need for improvement in training to improve perinatal care of these defects.


2014 ◽  
Vol 2014 ◽  
pp. 1-9 ◽  
Author(s):  
Brian M. Inouye ◽  
Ali Tourchi ◽  
Heather N. Di Carlo ◽  
Ezekiel E. Young ◽  
John P. Gearhart

The exstrophy-epispadias complex is a rare spectrum of malformations affecting the genitourinary system, anterior abdominal wall, and pelvis. Historically, surgical outcomes were poor in patients with classic bladder exstrophy and cloacal exstrophy, the two more severe presentations. However, modern techniques to repair epispadias, classic bladder exstrophy, and cloacal exstrophy have increased the success of achieving urinary continence, satisfactory cosmesis, and quality of life. Unfortunately, these procedures are not without their own complications. This review provides readers with an overview of the management of the exstrophy-epispadias complex and potential surgical complications.


2021 ◽  
pp. 205-210
Author(s):  
Michael Obladen

Although rare, cleft bladder was known to the ancients: the infants did not die after birth, but grew up incontinent. Combined with abnormal genitalia, the malformation was confused with hermaphroditism, the most loathed congenital anomaly. Three grades were discerned in the 17th century: epispadias, where the urethra opens at the anterior side of the penis; bladder exstrophy with open anterior bladder wall; and cloacal exstrophy with common outlet for stool and urine. Explanations of its origin included obstructed urethra, short umbilical cord, and bursting allantois, bladder, abdominal wall, or cloacal membrane. As the incontinent children suffered from painful inguinal eczema, urine collecting devices were constructed from copper and tin, and from rubber once vulcanization was invented. Correcting operations were attempted from 1852 with limited success. Today, stepwise corrective surgery begins by closing the bony pelvis, bladder, and the abdominal wall, followed by epispadias repair and penis remodelling. No controlled trials or international protocols have come forth, and the long-term prognosis is all but satisfying.


2019 ◽  
Vol 12 (1) ◽  
pp. bcr-2018-226076
Author(s):  
Prashant Kumar ◽  
Rishi Nayyar ◽  
Amlesh Seth ◽  
Deepti Gupta

The exstrophy–epispadias complex represents a spectrum of genitourinary malformations ranging from simple glanular epispadias to an overwhelming multisystem defect, cloacal exstrophy. Neonatal total reconstruction of bladder exstrophy–epispadias complex is the treatment of choice. An adult patient presenting with untreated exstrophy is very rare. Malignant transformation, commonly adenocarcinoma, in such cases is a known complication due to mucosal metaplasia of urothelium. Management in such cases necessitates a radical surgical procedure that often results in a massive defect in the anterior abdominal wall. Providing a cover for such defects is a challenging task for the reconstructive surgeon. Local skin flaps and wide mobilisation of the rectus muscle are the usually employed techniques for closure of such defects. However, these may be inadequate in extremely large defects such as those encountered in our patients. We, hereby, describe our technique of closure of the abdominal wall defect using a pedicled anterolateral thigh flap.


Genes ◽  
2021 ◽  
Vol 12 (8) ◽  
pp. 1149
Author(s):  
Glenda M. Beaman ◽  
Raimondo M. Cervellione ◽  
David Keene ◽  
Heiko Reutter ◽  
William G. Newman

The bladder exstrophy–epispadias complex (BEEC) is an abdominal midline malformation comprising a spectrum of congenital genitourinary abnormalities of the abdominal wall, pelvis, urinary tract, genitalia, anus, and spine. The vast majority of BEEC cases are classified as non-syndromic and the etiology of this malformation is still unknown. This review presents the current knowledge on this multifactorial disorder, including phenotypic and anatomical characterization, epidemiology, proposed developmental mechanisms, existing animal models, and implicated genetic and environmental components.


2020 ◽  
Vol 56 ◽  
pp. 101425
Author(s):  
Mohamed B. Aboushaer ◽  
Noura M. Almutairi

2012 ◽  
Vol 188 (6) ◽  
pp. 2343-2346 ◽  
Author(s):  
Ifeanyi Anusionwu ◽  
Nima Baradaran ◽  
Bruce J. Trock ◽  
Andrew A. Stec ◽  
John P. Gearhart ◽  
...  

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