Abstract Introduction Craniosynostosis can affect the skull in various ways. The most common forms are abnormal skull shape and beaten copper pattern, while Lückenschädel (or lacunar skull) is one of the least common forms. Case presentation We report the case of a 3-month-old Caucasian boy with multiple suture craniosynostosis and with acquired craniomeningocele presenting as a bulging mass in the lateral occipital area. Conclusion To the best of our knowledge, this is the first report of a patient with multiple suture craniosynostosis and acquired craniomeningocele.</p

CE Shopfner

D Macaulay

F Schreiber

Farideh Nejat

G Du Boulay

JA Persing

JH Shin

KM Persson

M Tajima

Mostafa El Khashab

Nima Baradaran

Shahrooz Yazdani

WG Wilson

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Acquired craniomeningocele in an infant with craniosynostosis: a case report

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JOURNAL OF MEDICAL
CASE REPORTS
El Khashab et al. Journal of Medical Case Reports 2010, 4:104
http://www.jmedicalcasereports.com/content/4/1/104
Open AccessC A S E  R E P O R TCase reportAcquired craniomeningocele in an infant with 
craniosynostosis: a case report
Mostafa El Khashab2, Farideh Nejat*1, Shahrooz Yazdani1 and Nima Baradaran1
Abstract
Introduction: Craniosynostosis can affect the skull in various ways. The most common forms are abnormal skull shape 
and beaten copper pattern, while Lückenschädel (or lacunar skull) is one of the least common forms.
Case presentation: We report the case of a 3-month-old Caucasian boy with multiple suture craniosynostosis and 
with acquired craniomeningocele presenting as a bulging mass in the lateral occipital area.
Conclusion: To the best of our knowledge, this is the first report of a patient with multiple suture craniosynostosis and 
acquired craniomeningocele.
Introduction
The incidence of non-syndromic craniosynostosis is
approximately 0.4 to 1 in 10,000 live births. A single
suture is most commonly implicated. Syndromic cranio-
synostosis, with a more generalized pattern, is much less
common [1]. The majority of patients with craniosynos-
tosis have various skull alterations, which are severe in
syndromic type and are mainly considered to have arisen
from compensatory growth of the skull after stenosis of
some sutures and high intracranial pressure. Skull defor-
mities and beaten copper pattern are common findings in
craniosynostosis. Lückenschädel (lacunar skull) is rarely
observed, occuring in approximately 10% of patients with
craniosynostosis [2,3]. To the best of our knowledge, cra-
nial meningocele has not been reported with cranio-
synostosis before. We describe a patient with
craniosynostosis with acquired craniomeningocele and
hypothesize probable causes for this abnormal presenta-
tion.
Case presentation
A 3-month-old Caucasian boy was referred to our hospi-
tal due to abnormal shape of his head and a bulging mass
in the left occipital area. The mass had been found one
month before referral with a gradual increase in size, and
fluctuation in size during crying and sleeping. The lesion
was 1.5 × 2 cm in maximum diameter (Figure 1), very
tense, non-tender, and pulsatile. His head circumference
was on the 25th percentile for his age class. There was
apparent hypertelorism with exophthalmia and age-
appropriate developmental milestones. Askull X-ray (Fig-
ure 2) and a brain computed tomography (CT) scan
showed fusion of coronal, lambdoid and basal sutures
with a diffuse beaten copper appearance (Figure 3).
Hydrocephalus was not found in the CT scan. Expansive
craniotomy associated with skull reconstruction was per-
formed. There were prominent convolutional markings
in the skull exposed in the operation field, complicated by
dura matter entrapment in some places. The bone was
very thin especially in posterior regions in conjunction
with small defects in some areas. Postoperative course
was uneventful and the child's occipital mass resolved
dramatically.
Discussion
Skull shape irregularity has been recognized since antiq-
uity, but the scientific study of abnormal skull growth
related to craniosynostosis originates back to the late
1700s. Experimental studies have shown that selective
restriction of an individual cranial vault suture's growth
could result in skull deformities [4]. Moreover, cranial
base and even facial deformities develop secondary to the
cranial vault suture restrictions [5].
Beaten copper pattern is another skull change observed
in craniosynostosis. It was initially assumed to have
resulted from chronically elevated intracranial pressure
* Correspondence: Nejat@sina.tums.ac.ir
1 Department of Neurosurgery, Children's Hospital Medical Center, Medical 
Sciences, University of Tehran, Tehran, Iran
Full list of author information is available at the end of the articleBioMed Central
© 2010 El Khashab et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Com-
mons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduc-
tion in any medium, provided the original work is properly cited.
El Khashab et al. Journal of Medical Case Reports 2010, 4:104
http://www.jmedicalcasereports.com/content/4/1/104
Page 2 of 3(ICP) [6], but later it was widely considered to be a reflec-
tion of normal brain growth, without pathological signifi-
cance [7]. Diffuse, severe beaten copper pattern is an
indicator of elevated ICP, which is more common in
patients with craniosynostosis than in unaffected chil-
dren. The beaten copper pattern originates from the pres-
sure that the brain exerts on the continuously remodeling
inner cranium resulting in bony convolutions that are
manifested as a beaten copper appearance on cranial
radiographs [8].
Lückenschädel is characterized by multiple, round or
oval, radiolucent defects, sharply separated by dense
strips of bone (honeycomb-like configuration), which
tend to cluster in the cranial vault on plain skull film. It
can be a consequence of elevated ICP or can be a self-lim-
ited phenomenon seen in children with myelomeningo-
cele [9].
A comprehensive Medline search of articles from 1966
to 2008 revealed no previous cases of acquired craniocele
in craniosynostosis. The development of craniocele in the
setting of multiple suture synostosis is not clearly under-
stood and some suggestions may be considered. Severe
synostosis and reduced cranial growth in this patient
caused brain volume to exceed the cranium size, resulting
in severely elevated ICP. High ICP was observed in a
brain CT scan as multiple sites of bone defects, diffuse
beaten copper appearance and Lückenschädel. Mean-
while in the occipital area, where the cranium and brain
are closely associated, high ICP produced a gross bone
defect and dura with brain herniated through this defect
to attenuate the pressure.
Conclusion
The presence of brain herniation in a patient with multi-
ple suture synostosis is not clearly understood and has
not been reported before. Our patient presented with
occipital craniocele and we have tried to explain the
probable mechanisms causing this lesion. Severe synosto-
sis coupled with reduced cranial growth caused severe
intracranial hypertension. Meanwhile in the occipital
area, where the cranium and brain are closely associated,
high ICP can result in a bone defect and dura associated
with brain herniated through this defect.
Figure 1 Occipital lump: a unilateral mass in the left occipital area 
with pulsation but without tenderness.
Figure 2 Skull X-ray shows fusion of both coronal and lambdoid 
sutures with diffuse beaten copper appearance.
Figure 3 Tri-dimensional computed tomography scan confirmed 
the fusion in the coronal and lambdoid sutures and diffuse skull 
changes with honeycomb configuration and beaten copper ap-
pearance.
El Khashab et al. Journal of Medical Case Reports 2010, 4:104
http://www.jmedicalcasereports.com/content/4/1/104
Page 3 of 3Consent
Written informed consent was obtained from the
patient's parents for publication of this case report and
any accompanying images. A copy of the written consent
is available for review by the Editor-in-Chief of this jour-
nal.
Abbreviations
CT: computed tomography; ICP: intracranial pressure.
Competing interests
The authors declare that they have no competing interests.
Authors' contributions
FN, NB, and SY all made major contributions in patient care, literature review
and drafting of the manuscript. MEK made a substantial contribution to the lit-
erature review, correction and final approval of the manuscript. All authors
read and approved the final manuscript.
Author Details
1Department of Neurosurgery, Children's Hospital Medical Center, Medical 
Sciences, University of Tehran, Tehran, Iran and 2Department of Neurosurgery, 
Hackensack University Medical Center, New Jersey, USA
References
1. Shin JH, Persing JA: Craniofacial syndromes.  In Youmans Neurological 
Surgery 5th edition. Philadelphia: Saunders; 2004:3315-3330. 
2. Shopfner CE, Jabbour JT, Vallion RM: Craniolacunia.  Am J Roentgenol 
Radium Ther Nucl Med 1965, 93:343-349.
3. Wilson WG, Alford BA, Schnatterly PT: Craniolacunia as the result of 
compression and decompression of the fetal skull.  Am J Med Genet 
1987, 27:729-730.
4. Persing JA, Morgan EP, Cronin AJ, Wolcott WP: Skull base expansion: 
craniofacial effects.  Plast Reconstr Surg 1991, 87:1028-1033.
5. Persson KM, Roy WA, Persing JA, Rodeheaver GT, Winn HR: Craniofacial 
growth following experimental craniosynostosis and craniectomy in 
rabbits.  J Neurosurg 1979, 50:187-197.
6. Schreiber F: Intracranial pressure: The correlation of choked disc and 
roentgenologic pressure signs.  Am J Roentgenol 1930, 33:607-611.
7. Macaulay D: Digital markings in radiographs of the skull in children.  Br J 
Radiol 1951, 24:647-652.
8. Du Boulay G: The radiological evidence of raised intracranial pressure in 
children.  Br J Radiol 1957, 30:375-377.
9. Tajima M, Yamada H, Kageyama N: Craniolacunia in newborn with 
myelomeningocele.  Childs Brain 1977, 3:297-303.
doi: 10.1186/1752-1947-4-104
Cite this article as: El Khashab et al., Acquired craniomeningocele in an 
infant with craniosynostosis: a case report Journal of Medical Case Reports 
2010, 4:104
Received: 3 July 2008 Accepted: 11 April 2010 
Published: 11 April 2010
This article is available from: http://www.jmedicalcasereports.com/content/4/1/104© 2010 E  Khash b et al; licensee BioMed Central Ltd. is an Open Acc ss article distribut under the t rms of the Creativ  Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Journal of Medic  Case Reports 2010, 4:104


Abstract Introduction Craniosynostosis can affect the skull in various ways. The most common forms are abnormal skull shape and beaten copper pattern, while Lückenschädel (or lacunar skull) is one of the least common forms. Case presentation We report the case of a 3-month-old Caucasian boy with multiple suture craniosynostosis and with acquired craniomeningocele presenting as a bulging mass in the lateral occipital area. Conclusion To the best of our knowledge, this is the first report of a patient with multiple suture craniosynostosis and acquired craniomeningocele.</p

Yazdani Shahrooz

Nejat Farideh

El Khashab Mostafa

Baradaran Nima

Directory of Open Access Journals

Journal of Medical Case Reports

Craniolacunia in newborn with myelomeningocele. Childs Brain

Digital markings in radiographs of the skull in children.

G: The radiological evidence of raised intracranial pressure in children.

HR: Craniofacial growth following experimental craniosynostosis and craniectomy in rabbits.

Intracranial pressure: The correlation of choked disc and roentgenologic pressure signs.

Persing JA: Craniofacial syndromes.

Schnatterly PT: Craniolacunia as the result of compression and decompression of the fetal skull.

WP: Skull base expansion: craniofacial effects. Plast Reconstr Surg

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Acquired craniomeningocele in an infant with craniosynostosis: a case report

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