md-medicaldata

Authors

 

Ivana Stanišić¹, Stefan Ivić¹, Tanja Lakić^1,2, Vladan Trivunović¹, Goran Đenadić³, Jovan Ugarković<sup>4

1</sup>Centar za patologiju i histologiju, Univerzitetski klinički centar Vojvodine, Srbija
²Medicinski fakultet, Univerzitet u Novom Sadu, katedra za patologiju
³Služba za patološko-anatomsku dijagnostiku, Opšta bolnica “Dr Laza K. Lazarević”, Šabac, Srbija
⁴Klinika za ginekologiju i akušerstvo, Univerzitetski klinički centar Vojvodine

 

UDK: 618.33

The paper was received / Rad primljen: 15.11.2022.

Accepted / Rad prihvaćen: 15.12.2022.

 

Correspondence to:

Ivana Stanišić
Centar za patologiju i histologiju,
Univerzitetski klinički centar Vojvodine
Hajduk Veljkova 1
21000 Novi Sad
e-mail: tilivana92@gmail.com

 

 

Abstract

 

 

Sirenomelia is an extremely rare congenital fetal malformation involving the partial or complete fusion of the lower extremities and it is associated with anomalies of other organs. We present the case of a 25-year-old pregnant woman in whom anhydramnios was identified during a regular prenatal ultrasound examination in the 20th week of gestation. The fetus is vital, the heart action is registered, and the fetal kidneys are altered, the urinary bladder is not present, thus raising the suspicion of Potter’s syndrome. At the patient’s personal request and by the decision of the Ethics Committee, feticide was performed and the pregnancy ended in the 24th week of gestation. At the autopsy of the fetus, multiple anomalies of the upper and lower extremities (sirenomelia) and almost all organ systems, primarily of the genitourinary tract, were observed. Genitourinary tract malformations: aplasia of the right kidney, multicystic altered left kidney and absence of urinary bladder. The etiology of sirenomelia has not yet been elucidated, and therefore every case of this truly rare anomaly requires attention and creates the possibility for further research.

 

Key words:

oligohydramnios; sirenomelia; fused limbs

 

 

 

Sažetak

Sirenomelija je veoma retka malformacija koja se manifestuje u vidu parcijalne ili kompletne fuzije donjih ekstremiteta i najčešće je udružena sa anomalijama drugih organa. Predstavljamo slučaj trudnice stare 25 godina kod koje je na redovnom prenatalnom ultrazvučnom pregledu u 20-toj nedelji gestacije identifikovan anhidramnion. Plod je vitalan, srčana radnja se registruje, a fetalni bubrezi su izmenjeni, mokraćna bešike nije prisutna, s toga je postavljena sumnja na Poterov sindrom. Na lični zahtev pacijentkinje i odlukom Etičkog odbora izvršen je feticid i trudnoća je završena u 24-toj nedelji gestacije. Na obdukciji ploda uočene su multiple anomalije gornjih i donjih ektremiteta (sirenomelija) i gotovo svih organskih sistema prvenstveno genitourinarnog trakta u vidu aplazije desnog bubrega, multicistično izmenjenog levog bubrega i nepostojanja mokraćne bešike. Etilogija sirenomelije još uvek nije rasvetljena i zato svaki slučaj ove zaista retke anomalije zahteva pažnju i stvara mogućnost za dalja istraživanja.

 

Ključne reči:

oligohidramnion; sirenomelija; fuzija donjih udova

 

 

 

 

References:

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PDF: 04-Stanišić I. et al MD-Medical Data 2022;14(3) 073-075.pdf