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PubMed1998

Further delineation of the Opitz G/BBB syndrome: report of an infant with complex congenital heart disease and bladder exstrophy, and review of the literature.

The combination of complex congenital heart disease (double outlet right ventricle with pulmonary atresia, malalignment ventriculoseptal defect, right-sided aortic arch with left ductus arteriosus) and bladder exstrophy occurred in an infant with Opitz syndrome. Neither of these defects has previously been reported in association with Opitz syndrome. These malformations, which are midline defects, further characterize this syndrome as an impairment in midline development. The spectrum of congenital heart disease and genitourinary anomalies seen in Opitz syndrome is reviewed.

Abnormalities, Multiple↗
PubMedDate not supplied

Association of bladder exstrophy with congenital pathology of the hip and lumbosacral spine: a long-term follow-up study of 13 patients.

The authors evaluated, clinically and radiographically, 13 of 28 patients with bladder exstrophy treated at their institution between 1964 and 1982. All had been treated with bilateral iliac osteotomies and pelvic rami reapproximation to assist in urologic repair. At skeletal maturity, the diastasis had partially recurred and the patients had short stature and were living normal lives. Mild acetabular dysplasia and other pelvic abnormalities were common, as were abnormal radiographic findings in the lumbosacral spine. Despite these findings, most did not affect function. These results support the need for pelvic reconstruction for urologic repair, as well as the need for periodic radiographic evaluation of the pelvis and lumbosacral spine. If present, these have the potential to adversely affect function as an adult.

Abnormalities, Multiple↗
PubMed1993

Cosmetic reconstruction of the mons veneris and lower abdominal wall by skin expansion as the last stage of the surgical treatment of bladder exstrophy: a report of three cases.

The results of the correction of the medial scar depression and diastasis of the pubic hair of the mons veneris in three postpuberal female patients operated on for bladder exstrophy are reported. The reconstructive surgery is carried out using skin expanders. The expanded skin is utilized to create two dermoadipose flaps to fill the medial depression, to outline again the public hair areas, and to allow sutures without tension.

Abdominal Muscles↗
PubMed1980

[Bladder exstrophy. Transformation from an external diversion of the urine to an internal diversion].

It is possible to transform an external diversion (transcolic cutaneous ureterostomy) into an internal one (uretero-sigmoido-sigmoidostomy) in cases of bladder exstrophy which have not been reconstructed. This must not be done at too early an age (not before 7) and only under certain conditions : good renal function, normal upper urinary tract, no sigmoido-ureteral reflux good anal sphincter. Such an operation radically changes the life of the exstrophic who was previously handicapped by a collecting apparatus. One must assure a careful long term follow-up. The major details of technique and the results of 20 cases are described.

Adolescent↗
PubMed1999

Normal urodynamics in patients with bladder exstrophy: are they achievable?

PURPOSE: Urodynamic study was performed in patients with exstrophy to determine the effect of bladder neck reconstruction and the ability to achieve normal urodynamics following surgery. MATERIALS AND METHODS: A total of 30 exstrophy cases at different stages of reconstruction were retrospectively reviewed in terms of bladder capacity, compliance, stability and presence of detrusor contractions following urodynamic study. RESULTS: Bladder capacity increased from a third predicted volume for age to half after reconstruction. Approximately 80% of patients had compliant and stable bladders before bladder neck reconstruction. Following bladder neck reconstruction approximately half of the patients maintained normal compliance with a smaller number maintaining normal stability. A quarter of patients maintained normal filling dynamics following bladder neck reconstruction, and 19% maintained normal filling and voiding dynamics after reconstruction. CONCLUSIONS: The majority of closed exstrophy bladders have normal filling dynamics before bladder neck reconstruction. Compliance and stability are impaired following bladder neck reconstruction. Approximately 25% of patients with exstrophy may maintain normal detrusor function following reconstruction. However, less invasive alternatives to the Young-Dees-Leadbetter bladder neck reconstruction should be sought.

Adolescent↗
PubMed1985

Family-focused services for children with rare disorders, exemplified by bladder exstrophy. A Norwegian national health program.

Families of children with rare disorders have many experiences in common, as well as special problems related to the specific disorder. This paper presents Frambu Health Centre and the family-focused services offered to patients with bladder exstrophy during the first information and treatment course arranged for this group at the Centre. Main areas of concern as reported by the families are presented.

Adolescent↗
PubMed2003

Fetal bony pelvis in the bladder exstrophy complex: normal potential for growth?

OBJECTIVES: To provide the first look at the bony histologic features of fetuses with the exstrophy complex, specifically evaluating the endochondral ossification, stage of development, and microscopic potential for normal growth. METHODS: Three fetuses between 28 and 30 weeks of gestation, one with classic bladder exstrophy, one with cloacal exstrophy, and one control, were obtained from France. The bony pelves were dissected and preserved in formalin, and multiple representative sections were sliced from all pelvic areas: pubis, ischium, ilium, and sacrum. These slices were sequentially processed as slides, stained with hematoxylin-eosin, and evaluated microscopically for histologic changes, developmental stage, and degree of endochondral ossification. RESULTS: All slides from the three specimens showed cartilage analogue with endochondral ossification. Histologically the exstrophy specimens were identical to the control and appeared completely normal; bone development was occurring at an expected rate with the potential for continued normal growth. CONCLUSIONS: These new findings illustrate that fetal bone in the exstrophy complex displays normal microscopic growth patterns and unhindered endochondral ossification at 28 weeks of gestation, well beyond the embryologic period. With no evident microscopic bony defect, the gross bony anomalies in exstrophy should be surgically correctable, leading us to conclude that early reapproximation of the physiologic shape of the pelvis could lead to more normal gross bone growth, decreased shortage of bone, and a more appropriate distribution of the mechanical and developmental forces on a closed, normally functioning pelvic ring.

Abortion, Legal↗
PubMed2006

Aesthetic aspects of bladder exstrophy: results of puboplasty.

PURPOSE: At times children born with bladder exstrophy-epispadias complex must undergo several operations and the resultant scar tissue on the abdomen can be quite disfiguring. Long-term followup in these patients reveals that many have a poor self-image, and the aesthetic aspects of the genitalia and lower abdomen acquire greater significance with age. We present our retrospective case series of the experience of 1 surgeon during 27 years. MATERIALS AND METHODS: Our database includes 116 patients born with exstrophy-epispadias complex. Primary reconstruction was performed in 62 infants and neonates, while 54 children and young adults underwent initial surgery elsewhere. Secondary puboplasty was performed in 88 of the 116 patients. Various techniques were used, ranging from simple excision and longitudinal closure in 12 cases, Z-plasty and pubic contouring in 23, the use of axial pattern inguinal skin flaps in 50 and tissue expanders in 3, when necessary. Long-term followup data were available on 76 patients. The parent and, when appropriate, the patient determined satisfaction with the cosmetic appearance. RESULTS: Patient satisfaction with the cosmetic and functional outcomes of surgery was high. Of the 76 patients 73 (96%) were satisfied following puboplasty. Complications included wound infection and keloid formation in 4% and 10% of cases, respectively. CONCLUSIONS: Achievement of excellent aesthetic results is possible with secondary puboplasty. Simple closure is associated with a higher incidence of keloid formation compared to the Z-plasty closure technique. Axial pattern skin flaps and Z-plasty techniques yield superior cosmetic results for contouring the mons pubis in patients with exstrophy-epispadias complex.

Adolescent↗
PubMed1988

Anatomic basis for the orthopedic treatment of bladder exstrophy: anatomic study of the sacrosciatic ligaments in the newborn.

Anatomic study of the pelvis of 30 fetuses and newborn children, yielded a precise relationship between the sacrotuberous ligaments and size of the gluteal region. At birth, the distance between the posterior superior iliac spine and the ischium is 40 mm. Closure of the anterior defect in bladder exstrophy sometimes requires an orthopedic surgical procedure. Bilateral posterior iliac osteotomy and section of these ligaments can be easily done by a single medial posterior approach herein described.

Bladder Exstrophy↗