Background: Prune belly syndrome (PBS) is a congenital anomaly that consists of a triad of abdominal wall defect, bilateral cryptorchidism, and urinary tract dilation. The disease is of varying severity. This study aims to highlight the challenges and peculiarities in the management of PBS in a resource-poor setting. Materials and Methods: This is a ten-year retrospective study conducted at the University of Port Harcourt Teaching Hospital. Ethical approval for the study was sought and gotten from the hospital’s ethical committee. The information gotten included history, duration of symptoms, examination findings, age of the patient, category of disease, and intraoperative findings. The data from the folders were collected and evaluated. Frequencies, percentages, the mean and standard deviation were used to summarize the data as appropriate. Results: Fifteen patients were included in the study. The hospital incidence of PBS was 112/100,000, twelve males and three females. The age range was from 1 day to 15 years, mean age was 14 months ± 2.3 months. Most patients presented between 3 months and 2 years and 11 months. Twelve patients had category three PBS and five patients had associated anomalies. Eleven male patients died after 5 years of follow-up from progressive renal deterioration. The female patient fared better than the males. Conclusion: PBS is rare, most patients with the condition present late. The most common cause of mortality was progressive renal deterioration.
References
[1]
Arlen, A.M., Nawaf, C. and Kirsch, A.J. (2019) Prune Belly Syndrome: Current Perspectives. Pediatric Health, Medicine and Therapeutics, 10, 75. https://doi.org/10.2147/PHMT.S188014
[2]
Wong, D.G., Arevalo, M.K., Passoni, N.M., et al. (2019) Phenotypic Severity Scoring System and Categorisation for Prune Belly Syndrome: Application to a Pilot Cohort of 50 Living Patients. BJU International, 123, 130-139. https://doi.org/10.1111/bju.14524
[3]
Woodhouse, C.R., Ransley, P.G. and Innes-Williams, D. (1983) Prune Belly Syndrome—Report of 47 Cases. The Journal of Urology, 130, 631. https://doi.org/10.1016/S0022-5347(17)51375-0
[4]
Moreno, A.E., Montenegro, M.A., Santos, P.A., et al. (2022) Pregnant Patient with Prune Belly Syndrome: Case Report. Einstein (São Paulo), 20, eRC6903. https://doi.org/10.31744/einstein_journal/2022RC6903
Cembraneli, P.N., Cavalcante, J.B., Cavalcante, R.B., et al. (2020) Renal Complication Due to Prune Belly Syndrome: A Case Report. International Journal of Case Reports and Images, 11, 1-5. https://doi.org/10.5348/101097Z01PC2020CR
[7]
Lopes, R.I., Tavares, A., Dénes, F.T. and Cocuzza, M. (2020) Gonadal Function and Reproductive System Anatomy in Postpubertal Prune-Belly Syndrome Patients. Urology, 145, 292-296. https://doi.org/10.1016/j.urology.2020.04.124
[8]
Iqbal, N.S., Jascur, T.A., Harrison, S.M., et al. (2020) Prune Belly Syndrome in Surviving Males Can Be Caused by Hemizygous Missense Mutations in the X-Linked Filamin A Gene. BMC Medical Genetics, 21, Article No. 38. https://doi.org/10.1186/s12881-020-0973-x
[9]
Quilici, G., Tolarova, M.M., Quilici, M. and Quilici, D.L. (2022) Dental Treatment of Patients with Prune Belly Syndrome. Special Care in Dentistry. https://doi.org/10.1111/scd.12728
[10]
Keet, K., Henry, B.M. and Tubbs, R.S. (2021) Prune-Belly Syndrome in Africa: An Analysis and Systematic Review of Cases, Aetiology, Treatment, and Outcomes. Journal of Clinical Urology, 14, 369-384. https://doi.org/10.1177/2051415820903196
[11]
Aloni, M.N., Mujinga, V., Tady, B.M. and Nkidiaka, E.D. (2015) The First Description of Prune Belly Syndrome in Central Africa. Pediatrics & Neonatology, 56, 355-356. https://doi.org/10.1016/j.pedneo.2015.01.006
[12]
Leahy, S.D., Kala, U. and Petersen, K.L. (2018) Prune Belly Syndrome: A South African Perspective. African Journal of Nephrology, 21, 39-44. https://doi.org/10.21804/21-1-2957
[13]
Ekwunife, O.H., Ugwu, J.O. and Modekwe, V. (2014) Prune Belly Syndrome: Early Management Outcome of Nine Consecutive Cases. Nigerian Journal of Clinical Practice, 17, 425-430. https://doi.org/10.4103/1119-3077.134012
[14]
Ibadin, M.O., Ademola, A.A. and Ofovwe, G.E. (2012) Familial Prune Belly Syndrome in a Nigerian Family. Saudi Journal of Kidney Diseases and Transplantation, 23, 338.
[15]
Salako, A.A., Takure, A.O., Olajide, A.O., et al. (2009) Prune Belly Syndrome in an Adult Nigerian: Case Report. African Journal of Medicine and Medical Sciences, 38, 357-360.
[16]
Okeniyi, J., Ogunlesi, T., Dedeke, O., Oyelami, O. and Oyedeji, G. (2004) Prune Belly Syndrome in a Nigerian Child. International Journal of Pediatrics and Neonatology, 5, 1-4.
[17]
Solarin, A.U., Disu, E.A., Gbelee, H.O., et al. (2018) Three Cases of Prune Belly Syndrome at the Lagos State University Teaching Hospital, Ikeja. Saudi Journal of Kidney Diseases and Transplantation, 29, 178-184. https://doi.org/10.4103/1319-2442.225190
[18]
Fette, A. (2015) Associated Rare Anomalies in Prune Belly Syndrome: A Case Report. Journal of Pediatric Surgery Case Reports, 3, 65-71. https://doi.org/10.1016/j.epsc.2014.12.007
[19]
Fagbamigbe, A.F. and Idemudia, E.S. (2017) Wealth and Antenatal Care Utilization in Nigeria: Policy Implications. Health Care for Women International, 38, 17-37. https://doi.org/10.1080/07399332.2016.1225743
[20]
Ekholuenetale, M., Benebo, F.O. and Idebolo, A.F. (2020) Individual-, Household-, and Community-Level Factors Associated with Eight or More Antenatal Care Contacts in Nigeria: Evidence from Demographic and Health Survey. PLOS ONE, 15, e0239855. https://doi.org/10.1371/journal.pone.0239855
[21]
Jude, E., Houeninvo, H.G. and Sossou, G.A. (2022) Education, Health and Economic Growth in African Countries. Development, 2015, 93-101.
[22]
Jaca, A., Malinga, T., Iwu-Jaja, C.J., et al. (2022) Strengthening the Health System as a Strategy to Achieving a Universal Health Coverage in Underprivileged Communities in Africa: A Scoping Review. International Journal of Environmental Research and Public Health, 19, 587. https://doi.org/10.3390/ijerph19010587
[23]
Routh, J.C., Huang, L., Retik, A.B. and Nelson, C.P. (2010) Contemporary Epidemiology and Characterization of Newborn Males with Prune Belly Syndrome. Urology, 76, 44-48. https://doi.org/10.1016/j.urology.2009.12.072
Kondo, F., Matsumoto, F., Suenaga, S., et al. (2022) Bladder Cancer in a Long-Term Survivor of the Prune Belly Syndrome. Urology, 161, 93-95. https://doi.org/10.1016/j.urology.2021.11.028
[26]
Achour, R., Bennour, W., Ksibi, I., et al. (2018) Prune Belly Syndrome: Approaches to Its Diagnosis and Management. Intractable & Rare Diseases Research, 7, 271-274. https://doi.org/10.5582/irdr.2018.01094
[27]
Zugor, V., Schott, G.E. and Labanaris, A.P. (2012) The Prune Belly Syndrome: Urological Aspects and Long-Term Outcomes of a Rare Disease. Pediatric Reports, 4, e20. https://doi.org/10.4081/pr.2012.e20
[28]
Fusaro, F., Zanon, G.F., Ferreli, A.M., et al. (2004) Renal Transplantation in Prune-Belly Syndrome. Transplant International, 17, 549-552. https://doi.org/10.1111/j.1432-2277.2004.tb00486.x
[29]
Seidel, N.E., Arlen, A.M., Smith, E.A. and Kirsch, A.J. (2015) Clinical Manifestations and Management of Prune-Belly Syndrome in a Large Contemporary Pediatric Population. Urology, 85, 211-215. https://doi.org/10.1016/j.urology.2014.09.029
