A wretched condition

Mapping Intimacies ◽

10.1093/med/9780198854807.003.0029 ◽

2021 ◽

pp. 205-210

Author(s):

Michael Obladen

Keyword(s):

Abdominal Wall ◽

Bladder Wall ◽

Bladder Exstrophy ◽

Corrective Surgery ◽

Cloacal Exstrophy ◽

Bony Pelvis ◽

Cloacal Membrane ◽

Long Term Prognosis ◽

Epispadias Repair

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.

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A case report on continent isolated male epispadias.

The Professional Medical Journal ◽

10.29309/tpmj/2020.27.06.3899 ◽

2020 ◽

Vol 27 (06) ◽

pp. 1320-1322

Author(s):

Rao Nouman Ali ◽

Khalid Hussain ◽

Hassam Khalid ◽

Zain Yasin ◽

Attiq-ur- Rehman

Keyword(s):

Wide Spectrum ◽

Bladder Exstrophy ◽

Dorsal Surface ◽

Premature Rupture ◽

Cloacal Exstrophy ◽

Rare Congenital Anomaly ◽

Repair Technique ◽

Cloacal Membrane ◽

Medial Migration ◽

Epispadias Repair

Epispadias is a rare congenital anomaly in which the urethral opening is on the dorsal surface of penis. Its incidence is 1 in 117000 newborn males and 1 in 484000 in newborn females. Its etiology is considered as because of failure of medial migration of mesenchyme between the ectodermal and endodermal layers of cloacal membrane due to premature rupture of cloacal membrane. Epispadias often presents as exstrophy epispadias complex, a wide spectrum of abnormalities that consist of classic bladder exstrophy, Epispadias and cloacal exstrophy. This case was not a part of exstrophy epispadias complex and it was an isolated continent epispadias which is extremely rare and it was managed with Cantwell Ransley epispadias repair technique.

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Orthopaedic Management of Bladder Exstrophy

Turkish Association of Pediatric Surgeons ◽

10.5222/jtaps.2021.25986 ◽

2021 ◽

Author(s):

R. Özgür Özer

Keyword(s):

Abdominal Wall ◽

Bladder Exstrophy ◽

Pelvic Osteotomy ◽

Orthopedic Procedures ◽

Suture Materials ◽

Cloacal Exstrophy ◽

Surgical Interventions ◽

Pubic Rami ◽

Multi Stage ◽

Pubic Diastasis

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.

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Prenatal ultrasound diagnosis of anterior abdominal wall defects in sub Saharan Africa; simple but often missed

International Journal of Research in Medical Sciences ◽

10.18203/2320-6012.ijrms20213069 ◽

2021 ◽

Vol 9 (8) ◽

pp. 2252

Author(s):

Andrew H. Shitta ◽

Mercy W. Isichei ◽

Ezekiel D. Dung ◽

Solomon D. Peter ◽

Michael B. Ode ◽

...

Keyword(s):

Prenatal Diagnosis ◽

Abdominal Wall ◽

Detection Rate ◽

Anterior Abdominal Wall ◽

Bladder Exstrophy ◽

Prune Belly Syndrome ◽

Abdominal Wall Defects ◽

Cloacal Exstrophy ◽

Pentalogy Of Cantrell ◽

Prune Belly

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.

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Modern Management of the Exstrophy-Epispadias Complex

Surgery Research and Practice ◽

10.1155/2014/587064 ◽

2014 ◽

Vol 2014 ◽

pp. 1-9 ◽

Cited By ~ 8

Author(s):

Brian M. Inouye ◽

Ali Tourchi ◽

Heather N. Di Carlo ◽

Ezekiel E. Young ◽

John P. Gearhart

Keyword(s):

Quality Of Life ◽

Abdominal Wall ◽

Surgical Outcomes ◽

Surgical Complications ◽

Anterior Abdominal Wall ◽

Bladder Exstrophy ◽

Urinary Continence ◽

Cloacal Exstrophy ◽

Genitourinary System

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.

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Bladder Exstrophy from Childhood into Adult Life

Journal of the Royal Society of Medicine ◽

10.1177/014107689608900112 ◽

1996 ◽

Vol 89 (1) ◽

pp. 39P-46P ◽

Cited By ~ 27

Author(s):

Jacob Ben-Chaim ◽

Steven G Docimo ◽

Robert D Jeffs ◽

John P Gearhart

Keyword(s):

Urinary Tract ◽

Adult Life ◽

Bladder Exstrophy ◽

Cloacal Exstrophy ◽

Female Ratio ◽

Live Births ◽

Cloacal Membrane ◽

Male To Female ◽

Exstrophy Of The Bladder

Exstrophy of the bladder is rare and the incidence of bladder exstrophy is calculated to be from 1 per 30 000 to 50 000 live births with male to female ratio ranging from 1.5-5 to 1 1-4 It was found that persistence or overgrowth of the cloacal membrane on the lower anterior abdominal area, prevents normal mesenchymal ingrowth. This causes divergence of the lower abdominal muscular structures and forces the genital ridges to fuse caudal to the cloacal membrane. The stage of ingrowth of the urorectal septum at the time of rupture determines whether one will produce an exstrophic urinary tract alone (classic bladder exstrophy or epispadias) or cloacal exstrophy with the hindgut interposed between the hemibladders5,6.

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Management of cavernous abdominal wall defects post radical cystectomy in adult exstrophy complex

BMJ Case Reports ◽

10.1136/bcr-2018-226076 ◽

2019 ◽

Vol 12 (1) ◽

pp. bcr-2018-226076

Author(s):

Prashant Kumar ◽

Rishi Nayyar ◽

Amlesh Seth ◽

Deepti Gupta

Keyword(s):

Abdominal Wall ◽

Abdominal Wall Defect ◽

Bladder Exstrophy ◽

Rectus Muscle ◽

Anterolateral Thigh Flap ◽

Abdominal Wall Defects ◽

Cloacal Exstrophy ◽

Local Skin ◽

Anterolateral Thigh ◽

Total Reconstruction

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.

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