Three cases of prune belly syndrome at the Lagos State University Teaching Hospital, Ikeja

Solarin, Adaobi; Disu, Elizabeth; Gbelee, Henry Olusegun; Animasahaun, Adeola B; Aremu, Oluwatosin E; Ogbuokiri, Eucharia; Ogunnaike, Gbemisola O

doi:10.4103/1319-2442.225190

Cited by 7 publications

(11 citation statements)

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“…23,44,45,52 On the other hand, the syndrome may affect only one fetus in monozygotic twin pregnancies, suggesting that it is not purely an inherited disorder. 34,35,53,54 PBS has also been posited to be associated with trisomy 2 and deletions in chromosome 6’s long arm. 55,56…”

Section: Discussionmentioning

confidence: 99%

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

Keet

Henry

Tubbs

2020

Journal of Clinical Urology

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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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Section: Discussionmentioning

confidence: 99%

“…Surgical treatment and kidney transplantation are not always available in developing countries, and accordingly, a more conservative management strategy may be dictated. 8,26,34…”

Section: Discussionmentioning

confidence: 99%

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

Keet

Henry

Tubbs

2020

Journal of Clinical Urology

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“…Prune Belly Syndrome (PBS) is a rare congenital disorder composed of anomalies of various organ systems. It is allied with tremendous morbidities as 50% have variable degrees of urinary pathology during their lifetime and 67% develop renal failure 6 . These two case reports describe PBS, one with pectus carinatum and the second case with pectus excavatum.…”

Section: Discussionmentioning

confidence: 99%

“…Also mesenchymal developmental defects have been suggested as the underlying defect 1 . The cause of PBS is unknown 6 . Familial case reports and the higher incidence in males have suggested involvement of an X linked factor and recently mutations in the X linked gene Filamin A were reported 7 .…”

Section: Ethical Considerationsmentioning

confidence: 99%

Two cases of Prune Belly Syndrome from Kagera Region Tanzania

Kessy¹,

Philemon

Hamel

2020

EAHRJ

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Prune Belly Syndrome is a rare congenital disorder with unknown aetiology, consisting of a triad of abdominal muscle wall weakness, undescended testes, and urinary tract abnormalities. We are unaware of any preceding report of Prune Belly Syndrome in Tanzania, and here we describe two cases reported in Kagera region. The first case is a 2 month old boy with the triad of Prune Belly Syndrome along with pectus carinatum who died due to septicaemia. This case posed a diagnostic challenge at birth and during the natal period. Paucity of comprehensive knowledge of congenital malformations at the peripheral health facilities may have also contributed to the diagnostic challenge in the first place. The second case is a neonate who was referred to regional referral hospital where he was diagnosed with Prune Belly Syndrome at the age of four weeks. Because of limited capacity to manage congenital malformations at the regional referral hospital, he was referred to an urologist at the zonal referral hospital. However, inadequacies in supporting systems to the parents compounded care of the neonate with Prune Belly Syndrome. High index of Prune Belly Syndrome suspicion is needed in a resource limited setting in order to timely make diagnosis. There is also a need to strengthen institutional and individual's capacity for prenatal screening to detect congenital anomalies at an early stage of foetus development. Multidisciplinary management approach is necessary in order to improve the quality of life for patients with Prune Belly Syndrome. Psychosocial and medical support systems should be put in place in order to enhance preparedness for patient care in resource limited settings including equipping the referral hospital with different specialists and ensuring availability of basic investigations for patients.

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“…Prune belly syndrome is a rare congenital syndrome of unknown etiology. It consists of three symptoms: total or partial absence of abdominal muscles, cryptorchismus, and genitourinary malformations [1][2][3][4]. Big, wrinkled abdomen attracts attention in the neonate's appearance.…”

Section: Introductionmentioning

confidence: 99%

Orthopedic problems in prune belly syndrome – case report

Gruca¹,

Jasiewicz²

2018

Chir. Narzadow Ruchu Ortop. Pol.

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Prune belly syndrome is a rare congenital syndrome of unknown etiology. It consists of three symptoms: total or partial absence of abdominal muscles, cryptorchismus, and genitourinary malformations. The goal of this study is an analysis of prune belly syndrome patients treated in one hospital. Material: between 2008 and 2017 authors treated 3 patients with prune belly syndrome. A retrospective analysis of medical history of these patients was performed, basing on patients' data and images available in hospital archive. One patient was treated due to club-foot deformity and lower limb length inequality. Other two were treated for scoliosis, of which one received growth sparing technique, and the other posterior spinal fusion. In all cases the goals of treatment were achieved, no serious complications were noted. Conclusion: patients with prune-belly syndrome may also require orthopedic treatment. Multiple congenital anomalies, eg foot, spine or rib cage deformities may necessitate surgical treatment. Despite of higher risk of pulmonary complications, surgery might be uneventful.

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Three cases of prune belly syndrome at the Lagos State University Teaching Hospital, Ikeja

Cited by 7 publications

References 0 publications

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

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

Two cases of Prune Belly Syndrome from Kagera Region Tanzania

Orthopedic problems in prune belly syndrome – case report

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