scholarly journals Respiratory support in the absence of abdominal muscles: A case study of ventilatory management in prune belly syndrome

2020 ◽  
Author(s):  
Heleen J.C.L. Apostel ◽  
Els L.I.M. Duval ◽  
Jozef De Dooy ◽  
Philippe G. Jorens ◽  
Tom Schepens
Keyword(s):  
Respiratory Support ◽  
Abdominal Muscles ◽  
Prune Belly Syndrome ◽  
Prune Belly ◽  
Ventilatory Management

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

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.

scholarly journals A rare case report of prune belly syndrome with malnutrition

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.

scholarly journals Prune belly syndrome - A case report

2012 ◽  
Vol 7 (3) ◽  
pp. 48-52
Author(s):  
D Rupshikha ◽  
MJ Phukon ◽  
GR Kusre ◽  
N Satyanarayana ◽  
P Sunitha
Keyword(s):  
Intestinal Tract ◽  
Undescended Testis ◽  
Abdominal Muscles ◽  
Prune Belly Syndrome ◽  
Medical Sciences ◽  
College Hospital ◽  
Medical College ◽  
Persistent Cloaca ◽  
Prune Belly ◽  
Gastro Intestinal Tract

Prune Belly Syndrome (PBS) is a rare congenital anomaly. A male baby was received from the Department of Paediatrics, Assam Medical College hospital which died on the second day of birth. It was a diagnosed case of Prune Belly Syndrome (PBS) due to its flabby abdominal wall ballooning out in flanks and skin creases radiating laterally and downwards from the umbilicus - "Prune" like appearance. It was observed that the baby had congenital absence of abdominal muscles and undescended testis. On dissection, associated defects of urinary tract and gastro intestinal tract, persistent cloaca and preductalcoarctation of aorta were noted. The aetiology of this case owes for an overall mesenchymal defect. DOI: http://dx.doi.org/10.3126/jcmsn.v7i3.6709 Journal of College of Medical Sciences-Nepal, 2011, Vol-7, No-3, 48-52  

Case Study on Management of Prune Belly Syndrome (Triad Syndrome)

2017 ◽  
Vol 16 (06) ◽  
pp. 26-29
Author(s):  
Dr. Rajnish Kumar ◽  
Dr. Nameer Faiz ◽  
Dr.Amarendra Kumar
Keyword(s):  
Prune Belly Syndrome ◽  
Prune Belly

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

2019 ◽  
Vol 29 (01) ◽  
pp. 014-020
Author(s):  
Nicolás Fernández ◽  
Ana María Ortiz ◽  
Juan David Iregui ◽  
Andrea Estrada ◽  
Angeline Rojas ◽  
...  
Keyword(s):  
Renal Function ◽  
Urinary Tract ◽  
Abdominal Wall ◽  
Management Approach ◽  
Abdominal Muscles ◽  
Prune Belly Syndrome ◽  
Urologic Surgery ◽  
Prune Belly ◽  
Bowel Movements

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.

scholarly journals A Rare Case Report of Prune Belly Syndrome

2013 ◽  
Vol 5 (2) ◽  
pp. 83-84
Author(s):  
Pooja Singhania ◽  
Deepti Shrivastava ◽  
Anuradha Kakani ◽  
Juhee Papalkar
Keyword(s):  
Case Report ◽  
Rare Case ◽  
Unknown Etiology ◽  
Abdominal Muscles ◽  
Prune Belly Syndrome ◽  
Fetal Therapy ◽  
Prune Belly ◽  
High Incidence ◽  
Rare Case Report ◽  
Characteristic Features

ABSTRACT Prune belly syndrome is a congenital abnormality of unknown etiology with characteristic features: Deficient development of abdominal muscles that causes the skin of the abdomen to wrinkle like a prune, cryptorchidism, abnormalities of the urinary tract. It is associated with high incidence of fetal and neonatal mortality. It can be diagnosed at 16 weeks during routine ultrasound and can be managed by fetal therapy. In rural setup women are neither visiting for routine anomaly scan nor affording for fetal therapy, hence termination of pregnancy remains the only option. How to cite this article Papalkar J, Shrivastava D, Kakani A, Singhania P. A Rare Case Report of Prune Belly Syndrome. J South Asian Feder Obst Gynae 2013;5(2):83-84.

scholarly journals The Prune Belly syndrome: urological aspects and long-term outcomes of a rare disease

2012 ◽  
Vol 4 (2) ◽  
pp. 20 ◽  
Author(s):  
Vahudin Zugor ◽  
Günter E. Schott ◽  
Apostolos P. Labanaris
Keyword(s):  
Renal Dysplasia ◽  
Abdominal Muscles ◽  
Prune Belly Syndrome ◽  
Renal Surgery ◽  
Prune Belly ◽  
Bilateral Cryptorchidism ◽  
Renal Malformations ◽  
Congenital Aplasia ◽  
The Individual

Prune-Belly syndrome is a disorder characterized by the following triad of symptoms: deficiency of the abdominal muscles, malformations of the urinary tract and bilateral cryptorchidism. This study included a total of 16 patients. The findings included clinical characteristics, diagnostics, therapy and long-term clinical outcomes. All patients were asked to complete a questionnaire and, in some cases, were given further examination. All patients were diagnosed with congenital aplasia of the abdominal wall and a variety of urogenital malformations. Cryptorchidism was present in 11 patients (68.8%), malformations of the prostate in 3 (18.8%), urethral malformations in 8 (50%) and mega-ureter in 14 patients (87.5%). A mega-bladder was observed in 13 patients (81.3%). Distinctive renal malformations, such as renal dysplasia, in 3 patients (18.8%) and hydronephrosis in 9 patients (56.3%), respectively. Abdominoplasty was performed on 4 patients (25%). Urethral surgery was performed in 10 patients (62.5%). Seven patients (43.8%) required ureter surgery, most of which involved re-implantation of the ureter and, in some cases, additional ureter modeling. Renal surgery was performed on 5 patients. Four patients with non-functioning kidneys with hydronephrosis underwent a nephrectomy and one patient pyeloplasty. We demonstrate that successful treatment is possible even in cases of serious and complex malformations, such as those of the Prune-Belly syndrome. Treatment must be tailored to the individual patient. The severity of the renal dysplasia is the main prognostic factor.

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