Difficulty in the Diagnosis of Biliary Atresia Splenic Malformation Syndrome In Utero.
Kurume Med J
; 68(3.4): 265-268, 2023 Sep 25.
Article
en En
| MEDLINE
| ID: mdl-37380446
ABSTRACT
The fetus of a 30-year-old pregnant Japanese woman was diagnosed with absence of inferior vena cava (IVC) and azygos continuation of interrupted IVC without cardiac anomalies at 34 weeks of gestation, and a healthy male neonate weighing 2,910 g was delivered at 37 weeks of gestation. On day 42 after birth, direct bilirubin predominant hyperbilirubinemia and high serum gamma-GTP levels were detected. Computed tomography revealed the presence of a lobulated and accessory spleen, and laparotomy demonstrated type III biliary atresia (BA), confirming the final diagnosis of BA splenic malformation (BASM) syndrome. In retrospect, non-visualization of the gallbladder was missed in utero. The combination of the absence of IVC and BA without cardiac anomalies is far less likely to occur in left isomerism. Although BA remains difficult to detect in utero, special attention should be paid to cases of BA associated with findings of left isomerism, including the absence of IVC, to enable early diagnosis and treatment of BASM.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Anomalías Múltiples
/
Atresia Biliar
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Malformaciones Vasculares
Tipo de estudio:
Diagnostic_studies
/
Screening_studies
Límite:
Adult
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Female
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Humans
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Male
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Newborn
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Pregnancy
Idioma:
En
Revista:
Kurume Med J
Año:
2023
Tipo del documento:
Article