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Published on: November 20, 2015
Iniencephaly: radiologic and pathomorphologic perinatal observation
Alexandra Korostyshevskaya1, Alyona Gornostaeva1,2, Rem Volkov3
1Institute "International Tomography Center" of the Siberian Branch of the Russian Academy of Sciences, Novosibirsk, Russian Federation.
Insights
Iniencephaly (IE) is a rare neural tube defect causing severe head retroflexion and spinal deformities. This case highlights complex associated anomalies and the role of advanced imaging in diagnosis and management.
Area of Science:
- Medical Imaging
- Radiology
- Fetal Medicine
Background:
- Iniencephaly (IE) is a rare, severe neural tube malformation characterized by extreme retroflexion of the fetal head and spinal deformities.
- It is often associated with other congenital anomalies, leading to a poor fetal prognosis.
Observation:
- A rare case of IE was diagnosed in a 44-year-old pregnant female at 26 weeks gestation via ultrasound, with detailed assessment at 36 weeks using fetal MRI.
- Imaging revealed extensive anomalies including cervical meningocele, occipital bone defect, cervical spina bifida, vertebral malformations, facial deformities, aortic coarctation, and hypoplastic lungs.
Findings:
- The fetal MRI and ultrasound findings confirmed a complex presentation of iniencephaly with multiple systemic malformations.
- Pathological examination post-termination of pregnancy corroborated the detailed radiological diagnoses.
Implications:
- This case emphasizes the critical role of multimodal imaging (ultrasound and MRI) in accurately diagnosing complex neural tube defects like IE.
- Accurate diagnosis through advanced imaging is crucial for informed clinical decision-making and management planning in cases of severe fetal malformations.
Abstract:
Iniencephaly (IE) is a rare neural tube malformation involving severe head retroflexion and deformity of the spine. IE is typically accompanied with other congenital abnormalities and carrying a poor fetal prognosis. This report presents radiological findings in a rare case of IE associated with multiple malformations of the skull, spine, face, heart, and body. A 44-year-old pregnant female underwent an obstetric ultrasound examination on the 26th week of gestation followed by fetal magnetic resonance imaging on the 36th week. Imaging revealed complex developmental anomalies, which led to the diagnosis of IE with a large cervical meningocele, occipital bone defect, spina bifida of the cervical vertebrae, multiple malformed vertebra, deformed face, coarctation of the aortic arch, and hypoplastic lungs. Based on these findings, a decision to terminate pregnancy was made. Pathological examination of the fetus showed close agreement with imaging. The presented case underscores the importance of multimodal imaging for clinical decision making in the management of complex neural tube malformations.
