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The metopic-sagittal craniosynostosis-report of 35 operative cases

Authors:
Takeyoshi Shimoji
Takeyoshi Shimoji
Takeyoshi Shimoji
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Takaoki Kimura at Juntendo University
Takaoki Kimura
Kazuaki Shimoji
Kazuaki Shimoji
Kazuaki Shimoji
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Masakazu Miyajima
Masakazu Miyajima
Masakazu Miyajima
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Abstract and Figures

Purpose: We have diagnosed 35 cases of the supposedly rare condition metopic-sagittal synostosis in the past 20 years. Here, we introduce their clinical symptoms, neuroradiological findings, and surgical treatment methods, as well as discuss the relevant literature. Methods: Subjects included 35 patients (33 boys and 2 girls; mean age 4.2 years; range 1-8 years). Magnetic resonance imaging (MRI) confirmed that there were no abnormal findings in the brain. Thirty patients presented with symptoms including speech delay, hyperactivity, autistic tendency, motor impairment, self-mutilation, and panic/temper tantrum behaviors. No other congenital malformation was observed, and all cases were considered to be the non-syndromic type. The final diagnosis was made using three-dimensional computed tomography (3D-CT) scans. The surgery was done the fronto-orbital advancement in addition to remove the large parts of sphenoid bones including sphenoid ridges at the skull base and trimmed the calvarium as necessary to reduce pressure. Results: Surgical intervention improved clinical symptoms in nearly all 35 patients; cosmetic problems in patients with scaphocephaly were also corrected. Conclusions: In the cases of child patients with metopic-sagittal synostosis who had clinical symptoms, surgical intervention improved such symptoms, suggesting its potential utility for metopic-sagittal synostosis with clinical symptoms. A surgical procedure focusing on the skull base was important for our successes. Based on the fact that metopic-sagittal synostosis was diagnosed in 35 patients at one institution over a relatively short period of time, this pathological condition may not be as rare as is currently believed.
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ORIGINAL PAPER
The metopic-sagittal craniosynostosis—report of 35
operative cases
Take y o s h i S himoji
1
&Takaoki Kim u r a
2
&Kazuaki Shimoji
2
&Masakazu Miyajima
2
Received: 11 February 2017 /Accepted: 21 April 2017 /Published online: 3 May 2017
#Springer-Verlag Berlin Heidelberg 2017
Abstract
Purpose We have diagnosed 35 cases of the supposedly rare
condition metopic-sagittal synostosis in the past 20 years.
Here, we introduce their clinical symptoms, neuroradiological
findings, and surgical treatment methods, as well as discuss
the relevant literature.
Methods Subjects included 35 patients (33 boys and 2 girls;
mean age 4.2 years; range 1–8 years). Magnetic resonance
imaging (MRI) confirmed that there were no abnormal find-
ings in the brain. Thirty patients presented with symptoms
including speech delay, hyperactivity, autistic tendency, motor
impairment, self-mutilation, and panic/temper tantrum behav-
iors. No other congenital malformation was observed, and all
cases were considered to be the non-syndromic type. The final
diagnosis was made using three-dimensional computed to-
mography (3D-CT) scans. The surgery was done the fronto-
orbital advancement in addition to remove the large parts of
sphenoid bones including sphenoid ridges at the skull base
and trimmed the calvarium as necessary to reduce pressure.
Results Surgical intervention improved clinical symptoms in
nearly all 35 patients; cosmetic problems in patients with
scaphocephaly were also corrected.
Conclusions In the cases of child patients with metopic-
sagittal synostosis who had clinical symptoms, surgical inter-
vention improved such symptoms, suggesting its potential
utility for metopic-sagittal synostosis with clinical symptoms.
A surgical procedure focusing on the skull base was important
for our successes. Based on the fact that metopic-sagittal syn-
ostosis was diagnosed in 35 patients at one institution over a
relatively short period of time, this pathological condition may
not be as rare as is currently believed.
Keywords Metopic-sagittal synostosis .Developmental
delays .Mild trigonocephaly .Sphenoid ridge
Introduction
We have dealt with cases of mild trigonocephaly exhibiting
various clinical symptoms since 1994 [20–23]; within these
cases, we diagnosed cases where the metopic suture is fused
with the sagittal suture, a phenomenon known as metopic-
sagittal synostosis. This pathological condition is currently
considered to be rare [3].
Currently, we have diagnosed 35 patients with metopic-
sagittal synostosis. Patients presented with clinical symptoms
that were improved to varying degrees following surgery in
almost all cases.
No study of a large number of patients with this patholog-
ical condition has been reported, but the frequency of such
patients being seen by our hospital suggests there may be
many more cases than conventionally believed. Therefore, to
improve the diagnosis and treatment of metopic-sagittal syn-
ostosis, we report here the analysis of clinical symptoms, neu-
roradiological diagnostic procedures, and surgical procedures
and outcomes in our patients.
*Takeyoshi Shimoji
trigono.research@gmail.com
1
Department of Neurosurgery, Amekudai Hospital, 1123, Ameku,
Naha, Okinawa 900-0005, Japan
2
Department of Neurosurgery, Juntendo University School of
Medicine, 2-1-1, Hongou Bunkyou-ku, Tokyo 113-8421, Japan
Childs Nerv Syst (2017) 33:1335–1348
DOI 10.1007/s00381-017-3430-1
Content courtesy of Springer Nature, terms of use apply. Rights reserved.
... Although our cohort included pediatric patients only and is not comparable with Morrison et al's study, the most affected sutures in our series were bicoronal, followed by sagittal, metopic, unicoronal, multiple, and bilateral lambdoid synostosis. 1 Previously published series have shown different ordinaries regarding the most affected sutures. [1][2][3][4][5][6] The most common type of craniosynostosis was sagittal synostosis (scaphocephaly), followed by coronal synostosis. [2][3][4][5][6] In the bicoronal synostosis (brachycephaly) cases, the coronal sutures on both sides of the child's head close too early. ...
... [1][2][3][4][5][6] The most common type of craniosynostosis was sagittal synostosis (scaphocephaly), followed by coronal synostosis. [2][3][4][5][6] In the bicoronal synostosis (brachycephaly) cases, the coronal sutures on both sides of the child's head close too early. Thus, the child's head will grow broad and short. ...
... Thus, the child's head will grow broad and short. 3,4,6 Pogliani et al reported 16 (57.1%) sagittal and 8 (28.6%) metopic synostoses among the true craniosynostosis group, who were first diagnosed with the USG. 5 Sloan et al reported 107 (42.8%) sagittal, 30 (12.0%) multiple suture, 30 (12.0%) unilateral lambdoid, and 28 (11.2%) ...
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