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|LETTER TO EDITOR
|Year : 2012 | Volume
| Issue : 6 | Page : 669-670
A rare case of lacunar skull with craniosynostosis
Prasad Krishnan, Rahul De, Rohit Mishra, Manoranjan Jena
Department of Neurosurgery, National Neurosciences Centre, Peerless Hospital Complex, Kolkata, India
|Date of Submission||06-Sep-2012|
|Date of Decision||29-Sep-2012|
|Date of Acceptance||08-Oct-2012|
|Date of Web Publication||29-Dec-2012|
Department of Neurosurgery, National Neurosciences Centre, Peerless Hospital Complex, Kolkata
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Krishnan P, De R, Mishra R, Jena M. A rare case of lacunar skull with craniosynostosis
. Neurol India 2012;60:669-70
A 3-month-old child, a product of non-consanguineous marriage delivered by caesarean section at 8 months of gestation, presented with abnormal head shape and incomplete closure of eyelids during sleep. Prenatal ultrasonography was reported normal. Head circumference was 36 cm and body weight was 5.5 kg. The child had bilateral ptosis with no neurologic deficits or papilledema, was playful and feeding well. No defects could be palpated through the scalp besides the fontanellae and the spine was normal. Computed tomography (CT) scan showed normally formed ventricles with defects in outer and inner table of skull bilaterally [Figure 1]a and poorly-formed orbits with proptosis [Figure 1]b. Three-dimensional (3D) reconstruction showed fusion of lambdoid suture with multiple cranial defects in occipital and parietal bones with open sagittal suture [Figure 1]c. The coronal suture was also fused with open metopic suture [Figure 1]d. The child underwent bicoronal scalp incision and strip craniectomies along the coronal and lambdoid sutures. Intraoperatively, the defects were covered by a membrane and there was no brain bulge. No cerebrospinal fluid CSF leak occurred during surgery. Postoperative period was uneventful. Postoperative 3D reconstructed CT scans showed craniectomies at coronal [Figure 1]e and lambdoid sutures [Figure 1]f.
|Figure 1: (a) Computed tomography (CT) scan of brain [axial images] showing normally formed ventricles with defects in outer and inner table of skull bilaterally, (b) CT scan of brain [axial images] showing poorly formed orbits and bilateral proptosis, (c) CT scan with threedimensional (3D) reconstruction showing fused lambdoid suture with multiple cranial defects in parietal, occipital and temporal bones with open sagittal suture, (d) CT scan with 3D reconstruction showed fused coronal suture with open sagittal and metopic utures, (e) postoperative 3D reconstructed CT scans showing craniectomy at coronal suture, and (f) ostoperative 3D reconstructed CT scans showing craniectomy at lambdoid suture|
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Lacunar skull (luckenschadel) is rare and usually associated with Chiari II malformation and myelomeningocele.  Association with craniosynostosis has been reported rarely. , The defects are caused by patchy areas of non-ossification of the membranous skull with bony ridges between them. These defects occur in parietal, frontal and occipital bones in that order.  The inner table is affected more than the outer with the gap being convex outwards. The gap often consists of only a thick membrane. Brain tissue may bulge into the gaps  and occasionally the brain may herniate through these defects.  This condition was first described in the West in 1875 and named as "luckenschadel" by Engstler in 1905.  Gross proptosis with lacunar skull was described in a report from India.  The child died within 2 weeks of delivery. Due to lack of CT imaging facilities at that time it was unclear if there was associated craniosynostosis, but skull skiagrams seem to suggest coronal synostosis. There was probably an associated brain herniation as well.
The cause of lacunar skull is not clearly known. Nakahara et al.  suggested that raised intracranial pressure may not be the cause since the defects do not correspond to the gyral patterns of the underlying brain. Further, most children are born with microcephaly or a normal size skull. Rather lack of ventricular distention as in open neural tube defects [meningomyelocele] or postventriculo-peritoneal shunt with resulting intracranial hypotension causes lack of induction of normal membranous ossification in the inner table and laying down of disorganized collagen tissue. Abdelmalik et al.  suggested that poorly distended ventricles may be responsible for lack of ossification. One of the patient's with lacunar skull with associated craniosynostosis had coronal, sagittal, and metopic stenosis. The authors  postulated that this may be a result of intracranial pulsations and may act as a protective mechanism to dampen the intracranial pressure. In the other case  there was premature fusion of coronal, lambdoid and basal sutures with herniation of the brain through a skull defect. The authors felt that lacunar skull defects may be a manifestation of raised intracranial pressure or a self limited phenomenon in patients with myelomeningocele.
Craniosynostosis is an extremely rare condition occurring in 0.4 per 10,000 births. Of these, lacunar skull is said to be present in only 10% of the cases.  In this child no tonsillar herniation or spinal dysraphism was present. Though proptosis was present no corneal changes or extraocular movement anomalies were seen. The ventricles were well formed. Sutural stenosis was also present but there was no papilledema. Whether this was due to damping of the intracranial pulsations through the lacunar skull defects is uncertain. Presence of lacunar skull with or without brain herniation must alert the surgeons regarding injury to these structures while raising the flap during strip craniectomies.
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