Vázquez CEE, Torres PG, Grether GP, Malfavón FM

Language: Spanish
References: 15
Page: 142-147
PDF size: 303.78 Kb.

ABSTRACT

Prune-Belly syndrome is a congenital disease defined by a classic triad that includes abdominal muscle abnormalities, urinary tract abnormalities, and bilateral cryptorchidism in men. The objective of this article is to document a case diagnosed at birth, which presented diagnosis of lower urinary obstruction at 17 weeks of gestation secondary to fetal megabladder of 5.7 × 5.8 × 5.2 cm and received treatment with laser ablation of urethral valves. At the time of birth, he presented clinical findings compatible with the Prune-Belly syndrome as well as a left clubfoot with nail aplasia of the 2nd to 5th ipsilateral toe. Some patients present extra-genitourinary manifestations at the cardiopulmonary, gastrointestinal, skeletal or neurological level, however, to our knowledge, no case with skin abnormalities such as nail aplasia has been published. Although it is a syndrome that reports a very low mortality, its consequences are diverse for the health and quality of life of infants. The etiology, symptoms, signs of suspicion, known treatments and prognosis of the product will be reviewed.

REFERENCES

1. Routh JC, Huang L, Retik AB, Nelson CP. Contemporary epidemiology and characterization of newborn males with prune belly syndrome. Urology. 2010; 76 (1): 44-48.

2. Arlen AM, Nawaf C, Kirsch AJ. Prune belly syndrome: current perspectives. Pediatr Health Med Ther. 2019; 10: 75-81.

3. Woods AG, Brandon DH. Prune belly syndrome: a focused physical assessment. Adv Neonatal Care. 2007; 7 (3): 132-143.

4. Domínguez LM, Álvarez FÁO. Manejo de las anomalías renales del tracto urinario detectadas por ecografía prenatal. Uropatías obstructivas. Protoc Diagn Ter Pediatr. 2014; (1): 225-239.

5. Seidel NE, Arlen AM, Smith EA, Kirsch AJ. Clinical manifestations and management of prune-belly syndrome in a large contemporary pediatric population. Urology. 2015; 85 (1): 211-215.

6. Farrugia MK. Fetal bladder outflow obstruction: interventions, outcomes and management uncertainties. Early Hum Dev. 2020; 150: 105189.

7. Grimsby GM, Harrison SM, Granberg CF, Bernstein IH, Baker LA. Impact and frequency of extra-genitourinary manifestations of prune belly syndrome. J Pediatr Urol. 2015; 11 (5): 280.e1-280.e6.

8. Chen L, Cai A, Wang X, Wang B, Li J. Two- and three-dimensional prenatal sonographic diagnosis of prune-belly syndrome. J Clin Ultrasound. 2009; 38 (5): 279-282.

9. Achour R, Bennour W, Ksibi I, Cheour M, Hamila T, Hmid RB et al. Prune belly syndrome: approaches to its diagnosis and management. Intractable Rare Dis Res. 2018; 7 (4): 271-274.

10. Montoya-Chinchilla R, Guirao-Piñera MJ, Nortes-Cano L. Valvas ureterales: revisión de la literatura y descripción de 4 nuevos casos. An Pediatría. 2014; 80 (1): 51-54.

11. Alvarado G, Garcia S, Garrido R. Valvas ureterales congénitas. Informe de dos casos. Acta Pediatr Mex. 2009; 30 (3): 133-136.

12. Singh SK, Wadhwa P. Ablation of diaphragmatic annular ureteral valve with holmium laser. Int Urol Nephrol. 2006; 38 (1): 157-159.

13. Zugor V, Schott GE, Labanaris AP. The Prune Belly syndrome: urological aspects and long-term outcomes of a rare disease. Pediatr Rep. 2012; 4 (2): e20.

14. Diao B, Diallo Y, Fall PA, Ngom G, Fall B, Ndoye AK et al. Syndrome de Prune Belly: aspects épidémiologiques, cliniques et thérapeutiques. Prog Urol. 2008; 18 (7): 470-474.

15. Hassett S, Smith GHH, Holland AJA. Prune belly syndrome. Pediatr Surg Int. 2012; 28 (3): 219-228.