Sirenomelia (Mermaid Syndrome)

Defrin Defrin, Calvindra Leenesa, Marcella Marcella

Abstract


Objective: To report a rare fetal abnormalities case
Method: Case report
Case:  Reported case of a 37-year-old patient came to the emergency room at RSIA Siti Hawa Padang with complaints of inpartu signs in the last 8 hours before entering the hospital. After the examination, the diagnosis was G3P2A0L3 35-36 weeks of preterm parturient active phase of first stage + twice previous CS + breech presentation. Then, the patient was planned for an emergency CS at 07.50 am. Born babies with genitalia form was not identified, BW: 2030 grams, BH: 30 cm, Scoring Apgar: 3/5, there are congenital abnormalities in the lower extremity of the baby like mermaid form. After observing the baby’s room, the baby was declared dead at 09.50 am.
Conclusion: Sirenomelia is a rare congenital defect that has a fatal impact, characterized by varying degrees of fusion in the lower limbs, thoracolumbar spinal anomalies, sacrococcygeal agenesis, genitourinary and anorectal atresia. Because of the poor prognosis, management of sirenomelia will be very difficult with unexpected results.

Sirenomelia (Sindroma Mermaid)

Abstrak
Tujuan: Melaporkan sebuah kasus jarang tentang kelainan janin
Metode: Laporan kasus
Kasus: Pasien 37 tahun datang sendiri ke IGD RSIA Siti Hawa Padang dengan keluhan nyeri pinggang menjalar ke ari-ari semakin lama semakin kuat dan sering sejak 8 jam sebelum masuk rumah sakit. Setelah dilakukan pemeriksaan ditegakkan diagnosis G3P2A0H3 parturien preterm 35-36 minggu kala I fase aktif + bekas SC 2x + letak sungsang. Kemudian, pasien direncanakan untuk dilakukan SC emergensi pukul 07.50 WIB. Dilahirkan bayi dengan JK : tidak teridentifikasi, BB : 2030 gram, PB : 30 cm, A/S : 3/5, terdapat kelainan kongenital pada ekstermitas bawah bayi. Setelah dilakukan observasi di ruang bayi, kemudian bayi dinyatakan meninggal pukul 09.50 WIB.
Kesimpulan: Sirenomelia adalah cacat bawaan yang jarang dan menimbulkan dampak yang fatal, ditandai dengan berbagai tingkat fusi pada tungkai bawah, anomali tulang belakang thoracolumbar, agenesis sacrococcygeal, genitourinari dan atresia anorektal. Karena memiliki prognosis yang buruk, pengelolaan sirenomelia akan menjadi sangat sulit dengan hasil yang tidak terduga.

Kata kunci : Kelainan janin, Sirenomelia, Mermaid syndrome.




Keywords


Fetal abnormalities, Sirenomelia, Mermaid syndrome.

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References


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DOI: http://dx.doi.org/10.24198/obgynia/v4n2.243

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