Unilateral macrocystic dysplasia and contralateral agenesis in a monoamniotic twin


Authors: Ostatníková M.;  P. Doležal ;  M. Gábor ;  J. Záhumenský
Authors‘ workplace: II. gynekologicko-pôrodnícka klinika LF UK a UN Bratislava, Slovenská republika
Published in: Ceska Gynekol 2022; 87(4): 278-281
Category: Case Report
doi: 10.48095/cccg2022278

Overview

Objective: We present a case report of a congenital malformation of the uropoetic tract in one of the monoamniotic twins. Case report: A 24-year-old primigravida with male monochorionic monoamniotic twins was dia­gnosed with congenital malformation in fetus A at 24 weeks of gestation. Ultrasound verified macrocystic dysplasia and contralateral renal agenesis. Planned caesarean section was performed after the observational management of the patient in the 34th gestational week. In fetus B, a physiological finding was confirmed on the postpartum ultrasonography. In fetus A, CT examination of the abdomen confirmed the finding of left kidney agenesis and polycystic degeneration of the right kidney. Exitus letalis was stated on the newborn‘s 5th day. Conclusion: The occurrence of the described combination of congenital malformation in monoamniotic twins is rare. When dysplasia significantly affects the function of the parenchyma, renal agenesis with multicystic dysplasia of the other kidney is a condition incompatible with life. For the intrauterine survival of the affected fetus, the normal renal function of the twin was important and thus the normal volume of amniotic fluid was maintained. As a result, the fetus did not develop extrarenal symptoms of the Potter sequence in the described case – especially pulmonary hypoplasia and the newborn was able to ventilate spontaneously. The death was caused by the consequences of renal failure associated with anuria.

Keywords:

monoamniotic twins – macrocystic renal dysplasia – renal agenesis


Sources

1. Šípek A, Gregor V, Horáček J et al. Incidence vrozených vad u dětí z jednočetných a dvojčetných gravidit v České republice – aktuální data. Ceska Gynekol 2009; 74 (5): 369–382.

2. Arulkumaran S, Ledger W, Denny L et al. Oxford textbook of obstetrics and gynaecology. New product. Oxford: Oxford University Press 2019.

3. Shub A, Walker SP. Planned early delivery versus expectant management for monoamniotic twins. Cochrane Database Syst Rev 2015; 2015 (4): CD008820. doi: 10.1002/14651858.CD008820.pub2.

4. Glinianaia SV, Rankin J, Khalil A et al. Prevalence, antenatal management and perinatal outcome of monochorionic monoamniotic twin pregnancy: a collaborative multicenter study in England, 2000–2013. Ultrasound Obstet Gynecol 2019; 53 (2): 184–192. doi: 10.1002/uog.19114.

5. Huber C, Shazly SA, Blumenfeld YJ et al. Update on the prenatal dia­gnosis and outcomes of fetal bilateral renal agenesis. Obstet Gynecol Surv 2019; 74 (5): 298–302. doi: 10.1097/OGX. 0000000000000670.

6. Bhandari J, Thada PK, Sergent SR. Potter syndrome. 2021 [online]. Available from: https: //www.ncbi.nlm.nih.gov/books/NBK560 858/.

7. Hsu PY, Yu CH, Lin K et al. Prenatal dia­gnosis of fetal multicystic dysplastic kidney in the era of three-dimensional ultrasound: 10-year experience. Taiwan J Obstet Gynecol 2012; 51 (4): 596–602. doi: 10.1016/j.tjog.2012.09.015.

8. Cilento Jr BG, Benacerraf BR, Mandell J. Prenatal and postnatal findings in monochorionic, monoamniotic twins discordant for bilateral renal agenesis-dysgenesis (perinatal lethal renal disease). J Urol 1994; 151 (4): 1034–1035. doi: 10.1016/s0022-5347 (17) 35169-8.

9. Klinger G, Merlob P, Aloni D et al. Normal pulmonary function in a monoamniotic twin discordant for bilateral renal agenesis: report and review. Am J Med Genet 1997; 73 (1): 76–79.

10. McNamara MF, McCurdy CM, Reed KL et al. The relation between pulmonary hypoplasia and amniotic fluid volume: lessons learned from discordant urinary tract anomalies in monoamniotic twins. Obstet Gynecol 1995; 85 (5 Pt 2): 867–869. doi: 10.1016/0029-7844 (94) 00249-d.

11. Perez-Brayfield MR, Kirsch AJ, Smith EA. Monoamniotic twin discordant for bilateral renal agenesis with normal pulmonary function. Urology 2004; 64 (3): 589. doi: 10.1016/j.urology.2003.10.053.

Labels
Paediatric gynaecology Gynaecology and obstetrics Reproduction medicine
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