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CT Angiography in a Newborn Child with Hydranencephaly

Journal of Perinatology volume 24, pages 565–567 (2004)Cite this article

Hydranencephaly is a massive hemispheric necrosis occurring in the fetus, with removal of the resultant debris leaving bilateral cystic structures, but usually sparing the basal ganglia, the brainstem, and the cerebellum.1, 2 The typical “vascular-like” distribution of the lesions in hydranencephaly led to the hypothesis of an ischemic etiology, partially confirmed by animal models of carotid ligation reproducing the gross appearance of the disease. Immediately after birth, hydranencephaly may be clinically and radiologically indistinguishable from massive hydrocephalus. The differentiation between the two conditions relies on the morphology of the cerebral arteries.3 Hydranencephaly is said to be associated with bilateral internal carotid artery (ICA) occlusion, while in hydrocephalus the cerebral arteries distal to the ICA termination are splayed and distorted but present.3 However, this vascular definition appears too restrictive, in particular if one considers the almost total absence of cortical mantle characteristic of hydranencephaly only as the most extreme form of a continuum of vascular lesions.1 We report here the case of a newborn baby with a suspicion of hydranencephaly confirmed by CT angiography. The technical aspect of the imaging study is discussed, and the vascular anatomy of hydranencephaly is reviewed.

The patient was an appropriate-for-gestational-age male infant born at 39 weeks to a 19-year-old mother (G3P1102) and unrelated father. Prenatal care was not sought until 32 weeks of gestation. Sonography performed at 36 weeks suggested the absence of cortical mantle, and no brain tissue was seen above the level of the thalamus. There were no known teratogenic or infectious exposures during pregnancy. The mother's two previous pregnancies had resulted in healthy children. The patient was delivered via induced vaginal delivery with Apgar scores of 8 at 1 and 5 minutes. Birth weight was 3168 g (50th percentile), length was 46 cm (50th percentile), and head circumference was 38 cm (>95th percentile). The anterior fontanelle was soft, measured 3 × 3 cm, and the sutures were split. The eyes were in normal position, with no sundowning. The remainder of the general examination was normal and there were no cutaneous vascular anomalies. The neurologic examination showed a vigorous, alert-appearing infant with mild irritability and a high pitched cry. The baby had a fair suck, normal neonatal reflexes and a mildly decreased tone. The scalp could be transilluminated. A clinical diagnosis of hydranencephaly versus severe congenital hydrocephalus was reached.

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Authors and Affiliations

  1. Division of Interventional Neuroradiology, The Johns Hopkins Hospital, Baltimore, MD, USA

    Philippe Gailloud MD

  2. Division of Pediatric Neurology, The Johns Hopkins Hospital, Baltimore, MD, USA

    Lori Jordan MD & Gerald Raymond MD

  3. Division of Diagnostic Neuroradiology, The Johns Hopkins Hospital, Baltimore, MD, USA

    Doris Lin MD

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  1. Lori Jordan MD
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  2. Gerald Raymond MD
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  3. Doris Lin MD
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  4. Philippe Gailloud MD
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Jordan, L., Raymond, G., Lin, D. et al. CT Angiography in a Newborn Child with Hydranencephaly. J Perinatol 24, 565–567 (2004). https://doi.org/10.1038/sj.jp.7211138

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