| Findings: !!Anatomy
Photo (Fig.01,02):
1. Large head circumference(42cm).
Ultrasound (Fig.03,04):
1. Loss most tissue of forebrain with large amount CSF and
normal falx cerebri, in favor of hydranencephaly.
CT with IV contrast (Fig.05-07):
1. Nearly total parenchymal loss of entire forebrain with brain
tissue replaced by CSF, except small bilateral medial occipital
lobes and small both thalami, in favor of hydranencephaly,
possibly due to liquefaction from previous diffuse brain insult.
Well demonstration of falx cerebri.
2. Atrophy of midbrain and mild atrophy of hindbrain.
3. Increased head size with widening of sutures, suggesting
macrocephaly.
MRI with T2WI (Fig.08-13):
1. Newborn boy. BPD = 115mm; FOD = 143mm
Cephalic index = (115/143)*100 = 80.4 within normal range.
But HC = (115+143)x1.57 = 405.1mm. Macrocephaly should be
considered in spite of any gestational age.
2. Nearly total parenchymal loss of entire forebrain with brain
tissue replaced by CSF, except small right inferior medial
temporal lobes, small bilateral medial occipital lobes, and
small both thalami, in favor of hydranencephaly, possibly due
to liquefaction from previous diffuse brain insult.
Well demonstration of falx cerebri.
3. Atrophy of midbrain and mild atrophy of hindbrain.
Normal transcerebellar diameter (TCD= 40mm).
!!Vascular Images
Color Doppler ultrasound with Doppler spectrum (Fig.14):
1. Presence of interruption of bilateral intracranial internal
carotid arteries and their branches from color Doppler images.
2. Relative increased resistivities (around upper limit) of
stumps of bilateral internal carotid arteries and basilar
artery from Doppler spectra.
Resisative index of R't ICA= 0.81
Resisative index of L't ICA= 0.76
Resisative index of BA= 0.72 (normal range: 0.5-0.8)
CT with IV contrast (Fig.15-16):
1. Small caliber of bilateral internal carotid arteries with
distal interruption at their intracranial portions.
2. Well demonstration of bilateral vertebral arteries
but interruption of branches of basilar artery.
MRI with gadolinium-enhanced MR angiogarphy with 3D
reconstruction, ECG-gated cine phase contrast flow images,
and MR diffusion imaging (Fig.17-21):
1. Evidence of interruption of bilateral intracranial internal
carotid arteries from MR angiogarphy.
2. From phase contrast flow images: increased resistivities of
bilateral common carotid arteries, compatible with distal
occlusion.
Resisative index of R't CCA= (16.1-0)/16.1= 1.0
Resisative index of L't CCA= (27.1-3.0)/27.1= 0.89
(normal range: 0.5-0.8)
3. No abnormal finding in hindbrain from MR diffusion images.
!!Skeleton
X-ray PA and Lateral view (Fig.22):
1. Relative large size of head by compared with face, compatible
with macrocephalic hydranencephaly.
CT without IV contrast (Fig.23):
1. No abnormal finding in whole body skeleton, except macrocephaly.
!!Endocrien
MRI with T1WI (Fig.24-26):
1. Well demonstration of short T1 signal focus in sella turcica,
suggesting at least somewhat (anterior lobe) development of
pituitary gland.
2. Normal bi-lobes thyroid gland in neck with normal signals.
CT with IV contrast (Fig.27):
1. Poor demonstration of bilateral adrenal glangs from this
examination, significance to be determined. | 
070321 MACROCEPHALIC HYDRANENCEPHALY
