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Last visited 09-03-2010 070321 MACROCEPHALIC HYDRANENCEPHALY Random Case
Authored By: pedrad CSH, Radiologist, Chung Shan Medical University Hospital, Taiwan.
Patient: 1 day old male
History:

Large head circumference(42cm) and poor activity since birth.

G3P3, Gestational age: 38+ weeks, Birth weight: 2750gm.
C-section due to breech.

Images:[small]mediumlargeas-submittedimages only

Fig. 1: Photo

Fig. 2: Photo

Fig. 3: Ultrasound_coronal

Fig. 4: Ultrasound_coronal and sagittal

Fig. 5: CT with IV contrast_axial

Fig. 6: CT with IV contrast_coronal

Fig. 7: CT with IV contrast_sagittal

Fig. 8: MRI_T2WI_axial

Fig. 9: MRI_T2WI_axial

Fig. 10: MRI_T2WI_coronal

Fig. 11: MRI_T2WI_coronal

Fig. 12: MRI_T2WI_sagittal

Fig. 13: MRI_T2WI_sagittal

Fig. 14: Color Doppler ultrasound (coronal, left upper) with Doppler spectrum (sagittal, the others)

Fig. 15: CT with IV contrast_coronal

Fig. 16: CT with IV contrast_sagittal

Fig. 17: MRI_gadolinium-enhanced MR angiogarphy with 3D reconstruction

Fig. 18: MRI_ECG-gated cine phase contrast flow images

Fig. 19: MRI_ECG-gated cine phase contrast flow images

Fig. 20: MRI_ECG-gated cine phase contrast flow images

Fig. 21: MRI_MR diffusion imaging

Fig. 22: X-ray_AP and lateral

Fig. 23: CT without IV contrast

Fig. 24: MRI_T1WI_sagittal

Fig. 25: MRI_T1WI_sagittal

Fig. 26: MRI_T1WI_sagittal

Fig. 27: CT with IV contrast_axial
Images:[small]mediumlargeas-submittedimages only
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.

Diagnosis: Macrocephalic hydranencephaly
Comments: post a comment

When you see large amount CSF in skull...

1. Alobar holoprosencephaly-- absent falx cerebri

2. Severe hydrocephalus-- formed falx cerebri, thin compressed cortex

3. Hydranencephaly-- formed falx cerebri, loss most part of brain tissue

By Dr. Tsao

--pedrad CSH, 2007-03-23 01:22:29
Additional Details:

Case Number: 8501554Last Updated: 03-22-2007
Rating:

5 ratings
Anatomy: Cranium and Contents   Pathology: Other
Modality: CT, Conventional Radiograph, Photograph, MR, US, 3D ReconstructionAccess Level: Readable by all users
Keywords: macrocephaly, hydranencephalyACR: 138.99Contained in: Featured Case Candidates, Filip_em, maryberner
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