PMC4061147_01
(A, B) Initial T1-weighted MR images of the patient's brain, including a Dandy-Walker malformation with hydrocephalus. (C) Brain CT images at 5 months after shunt surgery, showing decreased size of lateral ventricles. (D) Brain CT images at 7 months after shunt surgery, presenting slightly dilated lateral ventricles. (E) T2-weighted MR image at 9 months after shunt surgery, with an obliterated right lateral ventricle and a small left lateral ventricle. (F) Brain CT image and (G) T2-weighted MR images at 10 months after shunt surgery, with catastrophic manifestation. The left lateral ventricle is slightly expanded, but the right lateral ventricle appears to be slit-like. (H) A sign of impending bilateral uncal herniation appears in T2-weighted MR images.
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Brain computerized tomography (CT) and magnetic resonance imaging (MRI) showed a Dandy-Walker malformation with hydrocephalus (Figure 1A and 1B). A follow-up CT scan showed small lateral ventricles (Figure 1C) and decreased size for the 4th ventricular cyst. A CT scan showed slight dilatation of both lateral ventricles and the 4th ventricle cyst (Figure 1D). MRI revealed a slit-like right lateral ventricle and a small left lateral ventricle (Figure 1E). The right lateral ventricle was still small (Figure 1F and 1G). Nearly every sulcus of the cerebral hemispheres was obliterated, and perimesencephalic cisterns were invisible, which indicated impending bilateral uncal herniation (Figure 1H).
Retrospective review of the brain CT scans at (A) 0 months, (B) 5 months, (C) 7 months, and (D) 10 months after the initial shunt operation. The sagittal suture began to close at 7 months after initial shunt surgery (at the time of the first shunt revision).
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We re-evaluated all previous CT scans, and the closure of the sagittal suture began at 7 months after initial shunt surgery (at the time of the previous shunt revision) (Figure 2).
(A) 3D-CT brain image and (B) intraoperative photograph taken before cranial expansion surgery, with the same orientation. The closed sagittal suture is clearly visible on both images. (C) Sagittal suturectomy with barrel-stave osteotomy was performed. (D) Postoperative brain CT image 1 day after cranial expansion surgery. The ventricles and sulci are all effaced.
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The shunt proximal catheter and valve were replaced simultaneously (Figure 3).
The transition of the patient's HC on a standard Korean girl's head circumference chart (marked with red dots). Dramatic changes in HC around the cranial expansion surgery are described in the table. The patient's HC declined after shunt surgery and remained small thereafter.
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Nine months after cranial expansion surgery (age 23 months), she could walk without assistance, climb up stairs, and play with puzzles, and her HC was 45.2 cm (3th-5th percentile) (Figure 4).