Masó ZME, Borrero TCM, González NX

Language: Spanish
References: 15
Page:
PDF size: 263.82 Kb.

ABSTRACT

Introduction: Prune-Belly syndrome (PBS) or "prune abdomen" syndrome, also known as Eagle Barrett syndrome, is a form of fetal obstructive uropathy (UOF). Its incidence is estimated between 1/40,000 and 1/50,000 live births.
Case presentation: it is a case of Prune-Belly syndrome in a 36-day-old infant, out-of-hospital delivery. Clinically, she presented a distended abdomen with wrinkled or prune skin, bilateral cryptorchidism, signs and symptoms of respiratory failure. Ultrasonography revealed the absence of musculature in the abdominal wall, megacyst, cystic dilatation of the ureters, kidneys with cystic and dysplastic changes.
Discussion: regarding survival, numerous studies have found that the percentage of mortality in the first months of life is 27%. It is characterized by a classic triad: congenital absence of the muscles of the abdominal wall; urinary tract abnormalities and bilateral cryptorchidism, described in the reviewed literature, which coincides with the clinical characteristics of the case presented, with a male-female ratio of 20: 1. The highest risk has been described in twins and African Americans.
Conclusions: it is characterized by a classic triad: congenital absence of the muscles of the abdominal wall; urinary tract abnormalities and bilateral cryptorchidism. It is more common in males. The diagnosis of PBS can be made during pregnancy through obstetric ultrasound. Mortality in the first months of life is high.

REFERENCES

1. Marrero Abreu S, Diffur Duverger RE, Torres León L. Síndrome de Prune Belly. Presentación de un caso. REMIJ. 2017; 18(2):1-6.

2. Arias Ulloa R, Arroyo Torres JA, Pedroza Soto FA. Síndrome de Prune Belly: Reporte de un caso. Lux Médica. 2018; 13(39): 45-51.

3. Pachajoa H. De las bases embriológicas a la clínica en el síndrome de Prune Belly. Revista Cubana de Obstetricia y Ginecología. 2016;42(2): 254-259.

4. Meza YCR, Pérez OR. Síndrome Prune Belly: sobrevida de un paciente con insuficiencia renal. Pediatría. 2017;50(3): 78-81.

5. Portillo AC. Síndrome prune belly (vientre en ciruela): reporte de un caso. Rev Nac (Itauguá). 2015; 7(1): 46-9.

6. Ferrer Montoya R, Vázquez García R, Madiedo Pérez D. Síndrome de Prune Belly. Revisión Bibliográfica. Multimed. 2016; 20(4):793.

7. Fernández N, Ortiz AM, Iregui JD, Estrada A, Rojas A, Pérez Niño J. Follow up of Patients with Eagle-Barett (Prune Belly) Syndrome Treated with Single-Stage Abdominoplasty and Total Genitourinary Tract Reconstruction. Rev Urol colombiana [Internet]. 2019. [citado 24/11/2020]; Disponible en: https://www.thieme-connect.com/products/ejournals/pdf/10.1055/s-0039-1688965.pdf.

8. Blandón Paternina HJ, Díaz Jiménez IM, Ruiz Barrios JP. Síndrome de Prune Belly, Reporte de un caso y revisión de la literatura. Ciencia, Tecnología e Innovación en Salud. 2018; 3:54-9.

9. León Hernández MA, Acosta León J, Cárdenas Camarena L. Síndrome de Prune Belly: cirugía para mejoría estética y reconstrucción abdominal. Cir. Plást. Iberolatinoam. 2015; 41(2): 175-17.

10. Silva Hermann DH, Casavilca C, Padilla R. Síndrome de abdomen en ciruela pasa y dextrocardia, asociación infrecuente. Rev Med Hered. 2015; 26: 42-46.

11. Hernández Sarmiento R, Gelvez Nieto JC, Vergara Méndez D, Malo Rodríguez G, Gastelbondo Amaya R, González Chaparro LE, et al. Síndrome de Prune Belly en una paciente adolescente. Rev Mex Pediatr. 2020; 87(6): 227-231.

12. Arias Vargas R, Herrera Watson G, Lobo Prada T. Síndrome de Prune Belly. Rev Médica Sinergia. 2020; 5(11):1-9.

13. Sierra K, Mendoza Y, Rivera L, Cabrera Lozada C, Chuquiralao C, Rodriguez C, et al. Diagnóstico ecográfico del Síndrome Brune Belly. Reporte de caso. Rev. Latin. Perinat. 2018; 21(3): 168-71.

14. Durán Álvarez S. Seudo síndrome prune belly. Rev cubana Pediatr. 2020; 92: e990.

15. Ibadin MO, Ademola AA, Ofovwe GE. Familial prune belly syndrome in a Nigerian family. Saudi J Kidney DisTranspl. 2012; 23(2):338-42.