Anesthetic experience of a patient with Prune-belly syndrome

Yoon, Ji-Hyun; Ryu, Jaekyu; Kim, Jae-Won; Jeong, Chang-Young; Park, Sun Gyoo

doi:10.4097/kjae.2014.67.s.s94

Cited by 4 publications

(4 citation statements)

References 3 publications

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“…The last is blamed for majority of deaths in prune belly syndrome [18,19]. Yoon underlines that flat diaphragm and lack of abdominal muscles may not only impede coughing, but also may lead to post-operative complications [20]. Grimsby recommends careful anesthesiology preoperative assessment aiming diminishing the risk of respiratory complications, including lethal ones [15].…”

Section: Resultsmentioning

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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Section: Resultsmentioning

confidence: 99%

Orthopedic problems in prune belly syndrome – case report

Gruca¹,

Jasiewicz²

2018

Chir. Narzadow Ruchu Ortop. Pol.

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“…Anesthesia, airway management, and pain relief are challenging in PBS patients. 1,80,84 Respiratory complications are the primary cause of postoperative morbidity. Ineffective cough leads to insufficient clearing of secretions, and micrognathia present in some patients makes intubation difficult.…”

Section: Discussionmentioning

confidence: 99%

“…Ineffective cough leads to insufficient clearing of secretions, and micrognathia present in some patients makes intubation difficult. 1,84 Some PBS patients have Potter facies attributable to oligohydramnios, which affects mask placement and airway maintenance. 84 Anesthetic and analgesic drugs should be administered with caution because impaired kidney function affects their clearance.…”

Section: Discussionmentioning

confidence: 99%

“…1,84 Some PBS patients have Potter facies attributable to oligohydramnios, which affects mask placement and airway maintenance. 84 Anesthetic and analgesic drugs should be administered with caution because impaired kidney function affects their clearance. 27 Opiates should be avoided in particular because they depress respiration and affect renal function, both of which usually are already impaired in PBS patients.…”

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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