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CASE OF DIAGNOSIS OF SIRENOMELIA SYNDROME IN NORTH OSSETIA – ALANIA: EXPERIENCE, PROBLEMS AND DIFFICULTIES IN DIAGNOSIS

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Sirenomelia is a rare congenital malformation with lesions of the lower spine and pelvic organs in combination with the fusion of the lower extremities. The cause of sirenomelia is unknown. The aim of this work was to describe the clinical case of sirenomelia in a newborn child from dichorionic diamniotic twins. The child was found to have complete fusion of the lower extremities, agenesis of the external genitalia, anus atresia, and other gastrointestinal and urogenital anomalies which is typical for sirenomelia. In addition, there were tetralogy of Fallot, esophageal atresia with lower tracheoesophageal fistula, and cystic renal dysplasia. A retrospective analysis of data of the monitoring program for congenital malformations in North Ossetia – Alania from 2000 to 2019 was carried out. During the study period, 4 cases of sirenomelia were registered. During the study period, 156,925 children were born in North Ossetia – Alania, thus, the prevalence of sirenomelia syndrome was 1:39,231 newborns.
Title: CASE OF DIAGNOSIS OF SIRENOMELIA SYNDROME IN NORTH OSSETIA – ALANIA: EXPERIENCE, PROBLEMS AND DIFFICULTIES IN DIAGNOSIS
Description:
Sirenomelia is a rare congenital malformation with lesions of the lower spine and pelvic organs in combination with the fusion of the lower extremities.
The cause of sirenomelia is unknown.
The aim of this work was to describe the clinical case of sirenomelia in a newborn child from dichorionic diamniotic twins.
The child was found to have complete fusion of the lower extremities, agenesis of the external genitalia, anus atresia, and other gastrointestinal and urogenital anomalies which is typical for sirenomelia.
In addition, there were tetralogy of Fallot, esophageal atresia with lower tracheoesophageal fistula, and cystic renal dysplasia.
A retrospective analysis of data of the monitoring program for congenital malformations in North Ossetia – Alania from 2000 to 2019 was carried out.
During the study period, 4 cases of sirenomelia were registered.
During the study period, 156,925 children were born in North Ossetia – Alania, thus, the prevalence of sirenomelia syndrome was 1:39,231 newborns.

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