Follow up of Patients with Eagle-Barett (Prune Belly) Syndrome Treated with Single-Stage Abdominoplasty and Total Genitourinary Tract Reconstruction

Abstract Introduction The Prune Belly Syndrome (PBS) is the deficiency or congenital hypoplasia of the abdominal muscles accompanied with disorders of the urinary tract. The surgical treatment for the uropathy of the PBS seeks to correct the anatomical defects that induce damage to the urinary tract, including the improvement of the bladder emptying. The correction of the abdominal wall defect must be considered as an important part of the treatment. Objective To describe of the most relevant clinic characteristics and outcomes of a group of eight patients with PBS managed with abdominoplasty and reconstructive urologic surgery. Methods Retrospective review of the medical charts of patients with PBS at Hospital Universitario San Ignacio, Hospital Militar Central of Bogotá, Clinica Infantil Colsubsidio and Fundación Oftalmológica Ardila Lulle of Bucaramanga, (Colombia) that were managed with reconstructive surgery of the urinary tract and Monfort technique abdominoplasty (between 2006 and 2016) by one of the authors (JPN). The evaluated variables included: phenotypic appearance, renal function, bowel movements, and urinary tract infection (UTI) episodes. These variables as well as the abdominal wall aspect, were evaluated postoperatively. Results There were 11 patients identified with PBS between 2006 and 2016. Eight (8) of them underwent reconstructive urologic surgery, orchidopexy and Monfort technique abdominoplasty. After the procedures, none of the patients presented deterioration of the renal function, and they reported a better quality of the stools according to the Bristol scale; there were also less episodes of UTI. The phenotypic aspect of the abdominal wall was qualified as satisfactory in all cases. Conclusions Reconstructive urologic surgery and abdominoplasty in patients with PBS is an excellent choice of management that reduces the number of UTIs, improves bowel movements according to the Bristol scale, and preserves the renal function. This management approach also improves the phenotypic aspect of the abdominal wall. All of this leads to higher survival rate and better quality of life.

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Prune-belly syndrome in Africa: An analysis and systematic review of cases, etiology, treatment, and outcomes

Journal of Clinical Urology ◽

10.1177/2051415820903196 ◽

2020 ◽

pp. 205141582090319

Author(s):

Kerri Keet ◽

Brandon Michael Henry ◽

R Shane Tubbs

Keyword(s):

Health Care ◽

Urinary Tract ◽

Access To Health Care ◽

Cultural Beliefs ◽

Developed Countries ◽

Abdominal Muscles ◽

Prune Belly Syndrome ◽

Associated Anomalies ◽

Prune Belly ◽

Access To Health

Background: Prune-belly syndrome is a rare congenital disorder characterized by a spectrum of three anomalies: bilateral undescended testes, dilated urinary tract, and anterior abdominal muscle deficiency. Objectives: In developing countries, inadequate access to health care may affect treatment and outcomes of prune-belly syndrome. This study’s goal was to review the anatomical features, etiology, genetics, management, and outcomes of cases in Africa. Methods: PubMed was searched to identify case reports and case studies describing prune-belly syndrome in Africa. Data collected from each study included the number of cases, age at diagnosis, sex, description of the abdominal muscles, testes, and urinary tract, as well as associated anomalies, management, and long-term outcomes. Results: A total of 16 publications that reported 58 cases in African countries were included. The prevalence of female patients (15.5%) was higher than in developed countries (3%). The abdominal muscles were deficient in all cases, and bilateral cryptorchidism was present in nearly all males (96%). Distension of the bladder was common, with normal anatomy reported in only one case. Bilateral hydroureters and hydronephrosis also were present in the majority of cases. Only six cases (10.3%) had no associated anomalies, such as musculoskeletal or cardiovascular. Karyotyping was performed in only three cases (5.2%) because of limited hospital facilities. Six parents (10.3%) declined treatment for their children, 12 cases (20.7%) were managed conservatively, and 25 (43.1%) received surgical intervention. Patients’ mortality rate was higher than in developed countries. Conclusion: Diagnosis and treatment of prune-belly syndrome remains a challenge in Africa, in which multiple factors, such as access to health care and cultural beliefs, affect mortality rates and outcomes. Patient education and support groups may improve compliance with treatment. Level of evidence: Not applicable for this multicenter audit.

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A rare case report of prune belly syndrome with malnutrition

Oxford Medical Case Reports ◽

10.1093/omcr/omab017 ◽

2021 ◽

Vol 2021 (4) ◽

Author(s):

Turyalai Hakimi ◽

Mohammad Akbar Ibrahimi

Keyword(s):

Urinary Tract ◽

Family Medicine ◽

Rural Areas ◽

Valsalva Maneuver ◽

Unknown Etiology ◽

Abdominal Muscles ◽

Prune Belly Syndrome ◽

Prune Belly ◽

Tract Infections ◽

Urinary Tract Malformations

ABSTRACT Prune belly syndrome (PBS) is a rare congenital malformation of unknown etiology characterized by a triad of deficient abdominal wall musculature, undescended testicles and urinary tract malformations. Most of the patients have pulmonary, cardiac, skeletal and gastrointestinal tract anomalies. Lack of abdominal muscles leads to constipation due to inability to perform Valsalva maneuver, which helps push the stool out of the rectum during the defecation. Additionally, frequent respiratory tract infections, persisting constipation and urinary tract infections lead to the development of malnutrition in children. We report this case to raise the awareness of low socioeconomic and low-resource medical settings that malnutrition could be existed or caused by PBS. We also encourage the expansion of pediatric surgery and family medicine training to increase the number of specialist (family medicine) to report and refer PBS in earlier phase, while working in rural areas and remote provinces.

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Pathogenesis of the Prune-Belly Syndrome: A Functional Urethral Obstruction Caused by Prostatic Hypoplasia

PEDIATRICS ◽

10.1542/peds.73.4.470 ◽

1984 ◽

Vol 73 (4) ◽

pp. 470-475 ◽

Cited By ~ 1

Author(s):

Philippe Moerman ◽

Jean-Pierre Fryns ◽

Paul Goddeeris ◽

Joseph M Lauweryns

Keyword(s):

Urinary Tract ◽

Abdominal Wall ◽

Abdominal Distension ◽

Abdominal Muscle ◽

Urethral Obstruction ◽

Prune Belly Syndrome ◽

Urinary Tract Abnormalities ◽

Prune Belly ◽

Urogenital Anomalies

Abdominal muscle deficiency, urinary tract abnormalities, and cryptorchidism are the three major features of the prune-belly syndrome, also referred to as triad syndrome or Eagle-Barrett syndrome. The etiology is unclear and the pathogenesis a subject of continuing debate. Clinical and pathologic experience with seven cases of prune-belly syndrome is reviewed. Findings indicate that the urogenital anomalies can be attributed to a functional urethral obstruction which in turn is the result of prostatic hypoplasia. The histology of the abdominal wall is that of atrophy—ie, the degeneration of already formed muscle—and not of primitive muscle. This observation supports the theory that the abdominal muscle hypoplasia is a nonspecific lesion, resulting from fetal abdominal distension of various causes. Transient fetal ascites may be an important feature of the prunebelly syndrome.

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Current Management of the Dilated Urinary Tract in Prune Belly Syndrome

Urologic Clinics of North America ◽

10.1016/s0094-0143(21)00899-5 ◽

1990 ◽

Vol 17 (2) ◽

pp. 407-418 ◽

Cited By ~ 2

Author(s):

John R. Woodard ◽

Ira Zucker

Keyword(s):

Urinary Tract ◽

Prune Belly Syndrome ◽

Current Management ◽

Prune Belly

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Total Abdominal Wall Reconstruction in the Prune Belly Syndrome

The Journal of Urology ◽

10.1016/s0022-5347(17)44842-7 ◽

1986 ◽

Vol 136 (1 Part 2) ◽

pp. 282-285 ◽

Cited By ~ 31

Author(s):

Richard M. Ehrlich ◽

Malcolm A. Lesavoy ◽

Richard N. Fine

Keyword(s):

Abdominal Wall ◽

Abdominal Wall Reconstruction ◽

Prune Belly Syndrome ◽

Prune Belly

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Surgical Management of the Urinary Tract for the Prune-Belly (Triad) Syndrome

10.2310/uro.11043 ◽

2019 ◽

Author(s):

David B. Joseph

Keyword(s):

Urinary Tract ◽

Prune Belly Syndrome ◽

Ureteral Reconstruction ◽

Prune Belly ◽

Short And Long Term ◽

Tract Infections ◽

Vascular Preservation ◽

Urinary Reconstruction

Urinary reconstruction is tempting based on the impressive abnormal findings that are revealed on imaging. The abnormal appearance of the urinary system by itself is not enough to warrant reconstruction. Reconstruction should only be undertaken when there is clear clinical evidence that stagnant urine leads to urinary tract infections and/or obstruction that is associated with renal compromise. This chapter describes temporary and permanent upper and lower urinary reconstructions. Particular consideration is given to the pathophysiology of prune belly syndrome and the disproportionate dilation and dysfunction of the distal ureter when undertaking ureteral remodeling. The techniques of ureteral folding and formal excisional ureteral tapering are described stressing the importance of vascular preservation. The role of reduction cystoplasty is placed in perspective of short- and long-term benefits. This review contains 18 references. Key Words: Eagle-Barrett syndrome, megacystis, megaureter, prune-belly syndrome, tapered ureteral reimplant, triad syndrome, ureteral reconstruction, urinary diversion, bladder reduction.

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Abdominal Wall Reconstruction in the Prune Belly Syndrome

The Journal of Urology ◽

10.1016/s0022-5347(17)52848-7 ◽

1982 ◽

Vol 128 (1) ◽

pp. 226-226

Author(s):

J. Randolph ◽

C. Cavett ◽

G. Eng

Keyword(s):

Abdominal Wall ◽

Abdominal Wall Reconstruction ◽

Prune Belly Syndrome ◽

Prune Belly

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The Mechanism of Prune Belly Syndrome Development: A Sonographic Sequential Assessment

Journal of Diagnostic Medical Sonography ◽

10.1177/8756479320951195 ◽

2020 ◽

Vol 36 (6) ◽

pp. 594-603

Author(s):

Raham Bacha ◽

Syed Amir Gilani ◽

Iqra Manzoor ◽

Iftikhar Ahmad ◽

Syed Faisal Hanan Shah

Keyword(s):

Abdominal Wall ◽

Anterior Abdominal Wall ◽

Outlet Obstruction ◽

Normal Karyotype ◽

Abdominal Distention ◽

Ductus Venosus ◽

Prune Belly Syndrome ◽

Prune Belly ◽

Antenatal Sonography ◽

Urinary Tract Malformations

Prune belly syndrome is a rare disorder characterized by the absence of anterior abdominal wall muscles, bilateral cryptorchidism, and urinary tract malformations. The aim of this case study was to illustrate the developmental sequelae of prune belly syndrome, acquired through a series of antenatal sonography. A 20-year-old woman visited a diagnostic medical sonography center for an obstetrical sonogram. Her fetus was diagnosed with megacystis at 15 weeks’ gestation. The mother returned for repeated appointment and to observe fetal changes during the development of prune belly syndrome. First, the megacystis, hydroureter, and hydronephrosis were noted followed by hazy ascitic and amniotic fluid. The hemodynamics were altered in the umbilical artery, middle cerebral artery, and ductus venosus. Subsequently, the pressure was neutralized, but it was observed after birth that the newborn had cryptorchidism, a distended abdomen with wrinkled wall, and absent right kidney. In conclusion, prune belly syndrome could be caused by megacystis due to bladder outlet obstruction in otherwise normal karyotype fetuses. Megacystis leads to hydroureter, hydronephrosis, and abdominal distention. The persistent abdominal distention gives rise to the underdevelopment of anterior abdominal wall muscles and cryptorchidism.

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Prune-belly syndrome

Paediatrica Indonesiana ◽

10.14238/pi49.5.2009.304-8 ◽

2009 ◽

Vol 49 (5) ◽

pp. 304

Author(s):

Yenny Yenny ◽

Kusuma P. A ◽

Damanik M. P.

Keyword(s):

Congenital Anomaly ◽

Prognostic Factors ◽

Urinary Tract ◽

Pulmonary Hypoplasia ◽

Clinical Findings ◽

Prune Belly Syndrome ◽

Live Births ◽

Prune Belly ◽

Clinical Variants ◽

Urinary Tract Anomalies

Prune-belly syndrome, also known as Eagle-Barretsyndrome, is a congenital anomaly comprisingthree clinical findings: deficient abdominalmusculature, urinary tract anomalies, andbilateral cryptorchidism. Other clinical findings involvingrespiratory, skeletal, digestion and cardiovascular systemmay also accompany the syndrome. The incidence isapproximately 1 : 30,000 to 40,000 live births and 95%of cases occur in boys. Pulmonary hypoplasia and kidneyfailure are important prognostic factors that contributeto 60% of mortality rate. Treatment includes surgicalcorrection of the abdominal wall and urinary tract,orchidopexy and other supportive managements.l-4 Wereport 4 cases on typical Prune-belly syndrome, togetherwith other clinical variants.

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