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Antenatal diagnosis of iniencephaly: sonographic and MR correlation: a case report.

Pungavkar SA, Sainani NI, Karnik AS, Mohanty PH, Lawande MA, Patkar DP, Sinha S - Korean J Radiol (2007 Jul-Aug)

Bottom Line: However, the details of the fetal brain and spinal cord may not be adequately delineated on US.We found MR imaging to be superior for depicting central nervous system abnormalities.MR imaging has evolved as an imaging modality and it is complementary to fetal US, yet US remains the screening modality of choice.

View Article: PubMed Central - PubMed

Affiliation: Department of MRI, Mammography and BMD, Balabhai Nanavati Hospital & Research Centre, S.V. Road, Vile Parle (West), Mumbai, INDIA. drsonap@yahoo.co.in

ABSTRACT
Iniencephaly is an uncommon and fatal neural tube defect involving the occiput and inion, this occurs together with rachischisis of the cervical and thoracic spine, and retroflexion of the head. We report the ultrasound (US) and magnetic resonance (MR) imaging findings of a case of iniencephaly with clubfeet and arthrogryposis. The diagnosis of iniencephaly is easy to make on ultrasound due to the typical star-gazing fetus. However, the details of the fetal brain and spinal cord may not be adequately delineated on US. We found MR imaging to be superior for depicting central nervous system abnormalities. MR imaging has evolved as an imaging modality and it is complementary to fetal US, yet US remains the screening modality of choice.

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Related in: MedlinePlus

Club foot is visualized on US (A) and MR imaging (B) (white arrows).
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Figure 4: Club foot is visualized on US (A) and MR imaging (B) (white arrows).

Mentions: Transabdominal gray-scale US was performed on a HDI 5000 US scanner (ATL-Philips, Netherlands) using a broadband 2-5 MHz curved-array transducer. It revealed a dichorionic, diamniotic twin pregnancy. Fetus I was normal. Fetus II had increased head parameters for its age. The neck was short and hyperextended. The face was upturned, corresponding to the description of a star-gazing fetus (Fig. 1). The lateral and third ventricles were normal in size, but there was enlargement of the fourth ventricle (Fig. 2). No cephalocele or meningocele was detected. The craniocervical junction appeared deformed with fusion of the occiput with the upper cervical region (Fig. 3). This fetus did not demonstrate any limb activity during the examination, which lasted for 30 minutes, suggesting akinesia. There was hyperextension at the knees, with flexion of the arms and clenching of the fists. Equinovarus deformity of both feet was detected (Fig. 4), and polyhydramnios was also noted. The possibility of iniencephaly with arthrogryposis and fetal akinesia was raised.


Antenatal diagnosis of iniencephaly: sonographic and MR correlation: a case report.

Pungavkar SA, Sainani NI, Karnik AS, Mohanty PH, Lawande MA, Patkar DP, Sinha S - Korean J Radiol (2007 Jul-Aug)

Club foot is visualized on US (A) and MR imaging (B) (white arrows).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2627161&req=5

Figure 4: Club foot is visualized on US (A) and MR imaging (B) (white arrows).
Mentions: Transabdominal gray-scale US was performed on a HDI 5000 US scanner (ATL-Philips, Netherlands) using a broadband 2-5 MHz curved-array transducer. It revealed a dichorionic, diamniotic twin pregnancy. Fetus I was normal. Fetus II had increased head parameters for its age. The neck was short and hyperextended. The face was upturned, corresponding to the description of a star-gazing fetus (Fig. 1). The lateral and third ventricles were normal in size, but there was enlargement of the fourth ventricle (Fig. 2). No cephalocele or meningocele was detected. The craniocervical junction appeared deformed with fusion of the occiput with the upper cervical region (Fig. 3). This fetus did not demonstrate any limb activity during the examination, which lasted for 30 minutes, suggesting akinesia. There was hyperextension at the knees, with flexion of the arms and clenching of the fists. Equinovarus deformity of both feet was detected (Fig. 4), and polyhydramnios was also noted. The possibility of iniencephaly with arthrogryposis and fetal akinesia was raised.

Bottom Line: However, the details of the fetal brain and spinal cord may not be adequately delineated on US.We found MR imaging to be superior for depicting central nervous system abnormalities.MR imaging has evolved as an imaging modality and it is complementary to fetal US, yet US remains the screening modality of choice.

View Article: PubMed Central - PubMed

Affiliation: Department of MRI, Mammography and BMD, Balabhai Nanavati Hospital & Research Centre, S.V. Road, Vile Parle (West), Mumbai, INDIA. drsonap@yahoo.co.in

ABSTRACT
Iniencephaly is an uncommon and fatal neural tube defect involving the occiput and inion, this occurs together with rachischisis of the cervical and thoracic spine, and retroflexion of the head. We report the ultrasound (US) and magnetic resonance (MR) imaging findings of a case of iniencephaly with clubfeet and arthrogryposis. The diagnosis of iniencephaly is easy to make on ultrasound due to the typical star-gazing fetus. However, the details of the fetal brain and spinal cord may not be adequately delineated on US. We found MR imaging to be superior for depicting central nervous system abnormalities. MR imaging has evolved as an imaging modality and it is complementary to fetal US, yet US remains the screening modality of choice.

Show MeSH
Related in: MedlinePlus