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Case Report
Renal complication due to prune belly syndrome: A case report
1 Medical Sciences Course, Health Sciences School, Faculdade Ceres (FACERES), São José do Rio Preto, SP, Brazil
2 Neurosurgeon, Member of the Brazilian Society of Neurosurgery, Santa Mônica Hospital, Goiânia, GO, Brazil
3 Medical Sciences Course, Health Sciences School, Metropolitan University of Santos, Santos, SP, Brazil
4 Neurosurgeon, PhD in Neurosurgery, Member of the Brazilian Society of Neurosurgery, Professor at Santa Mônica Hospital, Goiânia, GO, Brazil
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Pedro Nogarotto Cembraneli
Praça Angelo Testa, nº 40, Jardim das Nações, 12080-050 Taubaté, SP,
Brazil
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Article ID: 101097Z01PC2020
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How to cite this article
Cembraneli PN, Cavalcante JBDF, Cavalcante RBDF, Ambrogi G, Cavalcante JEDS. Renal complication due to prune belly syndrome: A case report. Int J Case Rep Images 2020;11:101097Z01PC2020.ABSTRACT
Introduction: Prune belly syndrome (PBS) is a congenital disorder characterized by injury or hypoplasia of the abdominal wall musculature, severe urinary tract abnormalities, and bilateral cryptorchidism in males. It has an estimated incidence of 1 in 40,000 live births. Life expectancy is low, and most affected individuals do not survive for more than one year. About 30% of survivors develop chronic renal failure and require a kidney transplant.
Case Report: A male patient, delivered by cesarean section at 39 weeks of gestation, presented with respiratory failure, anuria, flaccid abdomen, bilateral cryptorchidism, and heartbeat with absence of abdominal muscle movements at delivery. Ultrasound of kidneys and urinary tract showed bilateral obstructive hydronephrosis, dilated ureters with tortuous aspect, and bladder partially full with normal wall thickness, suggesting low obstruction. In addition, polycystic kidneys with reduction in renal parenchyma were observed bilaterally. Based on the signs and imaging exam, the patient was diagnosed with PBS. Peritoneal dialysis and ureterostomy were performed. At two years of age, ureteroplasty and orchiopexy were performed, and spontaneous urethral diuresis occurred. After the surgical procedure, the patient underwent hemodialysis for one year before kidney transplant.
Conclusion: Prune belly syndrome is a rare, complex disease with high morbidity and mortality rates. Therefore, early diagnosis before birth during a routine fetal ultrasound or recognition at birth, as well as choice of the most suitable treatment requires greater knowledge of PBS and its complications.
Keywords: Abdominal wall, Cryptorchidism, Prune belly syndrome, Renal insufficiency, Urinary tract
SUPPORTING INFORMATION
Author Contributions
Pedro Nogarotto Cembraneli - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Julia Brasileiro de Faria Cavalcante - Substantial contributions to conception and design, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Renata Brasileiro de Faria Cavalcante - Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Gabriel Ambrogi - Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
José Edison da Silva Cavalcante - Acquisition of data, Revising it critically for important intellectual content, Final approval of the version to be published
Guaranter of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2020 Pedro Nogarotto Cembraneli et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
