Dental treatment of patients with prune belly syndrome

Background: Prune belly syndrome (PBS), also known as Eagle-Barrett syndrome (EGBRS), is a rare congenital disease characterized by deficiency or absence of abdominal wall muscles, urological abnormalities, and bilateral cryptorchidism.

Types of studies reviewed: A review of literature was done using four search engines (PubMed, Google Scholar, Scopus, Science Direct) and keywords (individually and in combinations): prune belly syndrome, PBS, Eagle-Barrett syndrome, dental manifestation, clinical manifestation, and psychological aspects. The search was run with no language restrictions and covered the 1965-2021 time period.

Results: The search yielded a large number of articles. The vast majority were dealing with a variety of treatments. PBS is a multisystem disease with a variable spectrum ranging from mild cases to infant mortality. Comorbidities of PBS (63% gastrointestinal, 65% orthopedic, and 49% cardiopulmonary) present challenges for treatment. PBS affects quality of life of patients and caregivers. We selected and summarized published information that is relevant to oral health and dental care.

Conclusions and practical implications: Providing information to dental practitioners will improve their understanding of PBS. It will help them to better treat patients with PBS and it will encourage more dental providers to welcome patients with PBS into their dental clinics.