Severe

Constricted Head – An Extreme Form Of Crouzon

Syndrome Posing Challenging Fronto-Orbital Advancement :
Case Reports

Ciptomurti J. Lupitasari a, Lobredia Zarasade a*, Magda R. Hutagalung a
aDepartment of Plastic Reconstructive and Aesthetic Surgery, Faculty of Medicine Universitas Airlangga
*Corresponding author: Lobredia Zarasade - Department of Plastic Reconstructive and Aesthetic Surgery,
Faculty of Medicine Universitas Airlangga. Email address : lobredia@gmail.com

ARTICLE INFO ABSTRACT

Background : Constricted head or cloverleaf deformity in Crouzon syndrome
Keywords: is a severe form of the syndrome involving trilobed coronal and lambdoid
Crouzon syndrome synostosis. Crouzon syndrome with acanthosis nigricans is distinct from the
Constricted head classic Crouzon syndrome, characterized by thick and dark skin in body folds.
Arduous surgical The major problems resulting from constricted head are related to
technique craniostenosis, orbitostenosis developing from abnormalities of the skull base
associated with progressive rise in intracranial and intraorbital pressures
which could progress to hydrocephalus and cerebellar herniation.
Case: Two cases with severe Crouzon syndrome were reported. The first was
a five month old girl associated with acanthosis nigricans and the other was a
sixteen month old boy, both whose phenotypic expressions were at the
extreme severe end of the disease spectrum. On examination there was
serious corneal exposure, visual loss, severely narrow head, midface
hypoplasia, and beaked nose. CT scan showed typical cloverleaf skull,
expanded middle cranial fossa, foreshortened anterior and posterior fossae
and honeycomb appearance in the occiput involving pansutural synostosis
and extensive copper beaten deformity from the high intracranial pressure .
Both were complicated with hydrocephalus requiring ventriculoperitoneal
shunt. This required an arduous effort in releasing the brain from the multiply
punctured calvaria, avoiding excessive dural tear and bleeding and ultimately
preserving the brain. Excessive bleeding was also caused by the raised ICP.
Both patients are planned to undergo occipital expansion three months later.
Conclusion: Severely constricted head in Crouzon syndrome is an extreme
manifestation and late stage of the syndrome which not only will result in
irreversible complication but will require meticulous surgical technique.

M
odern era of craniostenosis surgical the basic techiques remain unchanged after
treatment began in 1970, when treatment of over 1.600 patients3.
plastic surgeons and neurosurgeons Crouzon Syndrome is among the rare
joined forces to form craniofacial teams and syndromic craniosynostosis inherited by
described new techniques to solve the autosomal dominant transmission, affecting
functional and the structural problems at the cranial development in consequence of
same time. The techniques developed at the formation from two or more suture
Hopital des Enfants Malades in Paris were premature fusion called craniofacial
published in detail in 1982, and although dysostosis. This is characterized by
some minor changes have been introduced craniosynostosis, exophthalmos, and midface

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hypoplasia. The birth prevalence of Crouzon Case Report

syndrome is estimated to be 15–16 per one
million births3. Presented two cases of severe Crouzon
The majority of patients with Crouzon syndrome with constricted head, a five month
syndrome have mutations in the extracellular female baby and a sixteen month male baby
immunoglobulin III domain of the fibroblast who presented to Dr.Soetomo general
growth-factor receptor 2 (FGFR2) gene. hospital referred from the general hospitals
Crouzon syndrome with acanthosis nigricans in their area.
is rare; this condition occurs in about 1 Their chief complaints were progressively
person per million. For unknown reasons, it abnormal head shapes since birth, difficulty
affects females more than twice as often as in breathing, inflammation of both eyes, and
males. The genetic change involved in this
disorder causes the FGFR3 protein to be
Disclosure: The authors herely declare they
overly active, which disrupts the normal
growth of skull bones and affects skin have no financial interest in the information
pigmentation10. The main point in discussed in this article
Crouzonsyndrome is that the synostosis is progressively rapid visual loss with
frequently progressive. Usually, the sutural protruding eyes. They always snored and
fusions do not exist at birth. The coronal and would wake up suddenly in the night
sagittal fusions appear at about one year of apparently from difficult breathing. Perinatal
age, the lambdoid later in life5. history was unremarkable. Family history
The craniofacial deformity depends on which revealed no craniofacial deformity.
sutures are affected. Constricted head
deformity in Crouzon syndrome is a severe Case 1
form of the syndrome which may be a A 6 month old female, admitted to hospital
consequence of multiple sutural fusion, due to with abnormal head shape from birth. She is
a constriction ring developing in the the second child from her family with normal
lambdoid-squamosal zone, causing bulging in aterm birth, weight 3300 g, length 49 cm,
the frontal and temporal bones and a trilobar with normal limbs.
head shape(8). Examination of the head the baby showed
This constricted head has other names such severe constricted head clover leaf or
as cloverleaf skull (CLS), kleeblatshadel and trilobular deformity with bulging at the
triphylIocephaly9. The main features are anterior fontanalle like oxycephaly as-
cloverleaf deformity characterized by facial sociated with bilateral temporal bulges.
hypoplasia involving the orbits causing brachicephaly, turricephaly, midface
usually severe exophthalmos, and the nose hypoplasia that make a baked nose, with
and upper maxilla and occasionally severe cephalic index 13,3 and head circumference
hydrocephalus and poor prognosis due to 38 cm
progressive intracranial hypertension5. The
forehead is recessed vertically, with retrusion
of the supraorbital rim and root of the nose3.
A review article by Cinalli (2005) reported
that Chiari I malformation was found in 100%
of cloverleaf skull deformity12. Management
of CLS in Crouzon syndrome follows the same
established protocols as for other
craniosynostosis. Two universally accepted Figure 1. Craniofacialis region from 5 month female
patient
protocols are those from ACFU and Erasmus
CF center12.

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There was bilateral exorbitism with arriving at the ER, the neurosurgeon
the left eye being very proptotic with performed a emergency ventriculoperitoneal
constant epiphora since two weeks before she shunt in which the dissection was difficult
came to Dr.Soetomo general hospital. There due to the entrapped dura in the multiple
was bilateral lagophtalmus, corneal ulcer on holes on the thin calvaria causing extensive
the left eye, and exposure keratitis with papil bleeding. Because long durante and difficult
atrophy on the right eye. From operation cause a lot of 1500ml bleeding.
ultrasonography examination there was no It was decided to do a
sign of intraocular infection, and normal craniotomy/suturectomy, fronto-orbital
vitreoretinal. ENT finding. This patient advancement (FOA) and cranial vault
snored during sleep with nasal congestion remodelling with the neurosurgical team
without a history apnea. This patient had after splitting the palate to secure the airway
delayed global developmental problem in after extubation.
both pyschomotor and psychosocial. All
extremities were normal in function and
morphology. A CT scan of the head showed
hydrocephalus non communicans, synostosis
of the metopic, bilateral coronal and
lambdoid sutures with severe copper beaten
appearance

Figure 6. Before fronto-orbital advancement

Figure 7. Before and after palatal split, fixing the soft
palate to the alveolus with 4.0 silk suture.

Figure 4. CT-Scan showed cloverleaf deformity and
hydrocephalus

Figure 5. Cranial 3D reconstruction CT-Scan showed

Figure 8. Showed hole in duramater
copper beaten apearance



This patient had eye infection

resulting from severe exorbitism. Surgery had
to be postponed until the infection was
healed. While awaiting surgery his left eye
was protected with a tarsorrhaphy. Soon after

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severe constricted head clover leaf deformity,

brachicephaly, turricephaly, trigonocephaly,
with ridging on both coronal, sagital sutures,
and both lambdoid sutures with head
circumference 47 cm.

Figure 11. Preoperative appearance


Figure 8. Durante Operation, 1cm fronto-orbital Both eyes were displaced forward whenever
advancement
baby cried since seven months old. But in
thirteen month age, the protrusion became
more fixed . There are bilateral lagophtalmus,
bilateral proptosis with corneal ulcer on the
left eye, and exposure keratitis on the right
eye. Intra-oral examination revealed high
arched palate, hypoplastic maxilla and
chronic adenotonsilitis. Polysomnography
revealed mixed moderate central and
obstructive sleep apnea. This patient had
Figure 9. Filling the remaining cranial gaps with delayed global developmental problem in
fibrin glue and bone chips
both pyschomotor and psychosocial. All

extremities were normal in function and

morphology.

CT scan showed Kleeblattschadel head
deformity with premature closure of sagittal,
bilateral coronal, frontal, both lambdoid
sutures. Right and left lateral system ventricle
III-IV were very dilated indicating a
communicating hydrocephalus. The
exophthalmos on the right eye protruded 1,16
Figure 10. After frontoorbital advancement
cm and the left eye 1,19 cm.


Case 2

A-16 month old male patient was admitted to
hospital with abnormal head shape and
eyeball protrusion since seven month old
before. He is the last child from his family
birth atterm with section secaria, weight
3300g, length 50 cm, with normal limbs.
Examination of the head the baby showed

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Difficulties from this patient are high
intracranial pressure caused by
hydrocephalus communicans and make a
copper beaten appearance. Difficult airway
caused obstructive sleep apnea make a
challenge to anesthesiologist. Estimation
blood lost of this surgery is 180 ml. It was
decided to do a split palate to secure the
airway after extubation then a

craniotomy/suturectomy, fronto-orbital
advancement (FOA), cranial vault
remodelling and tapping the ventricle with
the neurosurgical team after

Figure 13. Bone window on computed tomography
scan of Figure 15. Before operative fronto-orbital
advancement

Figure 16. Before and after palatal split, fixing the
soft palate to the alveolus with 4.0 silk suture.

Figure 14. Cranium CT performed; 9 month after
fronto-orbital advancement operation

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Figure 17. Pre and post fronto-orbital advancement Table 2. Standard protocol from Erasmus CF
procedure, showed temporo parietal occipital region center12.
Australian Craniofacial Unit

Discussion Birth – 3 months Total Assessment

3 – 6 months Planning meeting
Constricted head in the form of CLS or
Surgery :
kleeblatshadel can be found in several
- Fronto-orbital
craniosynostosis syndrome including
Advancement
Crouzon syndrome, Boston type and and - Bone Graft
Thanatophoric dysplasia type II (11). - Lambdoid
Craniectomy
Table 1. Showed form of Cloverleaf skull 1 year old - Total review
deformity11. - Craniofacial clinic
No. Condition Craniofacial Gene 1 – 10 years old - Yearly review and
Phenotype craniofacial clinics 3
yearly
- Dentistry 6 monthly
1. Boston Type Craniosynostosis, MSX2 checks
cloverleaf skull, - Orthodontic
forehead treatment
retrusion, frontal Surgery :
boosing. - Facial/Fronto-facial
Advancement
2. Thanatop- Craniosynostosis, FGFR3
- Bone graft
horic cloverleaf skull,
Teenage years - Complete Workup
dysplasia severe bone
- Dentistry 6 monthly
type II growth
checks
disturbance
- Orthodontic
treatment
- Orthognathic surgery
Both cases were diagnosed with Maturity Touch-up surgery
Crouzon syndrome based on clinical findings. Erasmus Medical Center Rotterdam
Both has CLS which is a severe form of Pre operative CT, MRI, Funduscopy, Sleep Study
Crouzon syndrome with serious 6 months - Occipital expansion
complications including compromised airway with springs
and breathing, increased intracranial - Endoscopy of upper
pressure, visual loss and ulceration. These airway
were neglected cases because of delay in - If luxating
diagnosis and subsequently in treatment. eyeball/severe OSA :
Early Monobloc
This condition was supposed to have 8 – 12 years or > 18 Monobloc or Facial
been managed by standard protocols. In both years old Bipartition
patients, suturectomy, FOA and cranial vault 18 years old Le Fort I + BSSO
remodeling were done to correct fronto-
supraorbital retrusion, to protect the globes
and to expand the intracranial volume. Fusion of lambdoid sutures often
The underlying pathology of a CLS is a result in significant posterior cranial fossa
constriction ring in the lambdoid-squamosal volume, narrowing of central nervous system
zone8. and associated with hydrocephalus which
also be caused by venous hypertention13.

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The cystic dilatation of the temporal of Crouzon syndrome and Pfeiffer syndrome.
horns is the earliest sign of hydrocephalus Am J Hum Genet 2000;66:768-77.
and occurs secondary to disturbed growth of 2. Granger B., Evan G., John B., Analysis of
the cerebral capsule 9. In both patients, there Fronto-orbital Advancement for Apert,
Crouzon, Pfeiffer, and Saethre-Chotzen
was hydrocephalus. One had to be treated
Syndromes, 2000.
with emergency VP shunt before cranial 3. Renier D., Lajeunie E., Arnaud E., Marchac D.,
expansion and the other with tapping of the Management of craniosynostoses, 2000.
ventricle at the same time with cranial 4. Ghassan S., Jeelani O., Dunaway D., Hayward
expansion. R., Raised Intracranial pressure in crouzon
The skull X-ray showed typicalCLS syndrome : incidence, causer, and
with expanded middle cra- nial fossa, management., 2016.
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and honey- comb appearance in the occiput. *Cloverleaf Skull Syndrome”, 1974
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Hydrocephalus in Crouzon’s syndrome, 1989.
sutures9.
7. Granger B., Evan G., John B., Analysis of
On the head CT scans of both patients, Fronto-orbital Advancment for Apert,
there were the synostosis involved almost all Crouzon, Pfeiffer, and Saethre-Chotzen
the cranial sutures including the lambdoid Syndromes, 2000.
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complicated with hydrocephalus, the and Management. Springer-Verlag London.
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dissection had to be carried out to avoid dural of Crouzon’s Syndrome : a New Concept in
Aetiology ,1991
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10. Hill.J, Lister Hill National Center For
also caused by the raised ICP. The mechanism Biomedical Communication, Crouzon
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could be caused by constriction of the Surgery Indcations and Practice, Saunders
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27:1683–1691

Conclusion
Severely constricted head is an
extreme manifestation and late stage of
Crouzon syndrome. This could have been
prevented by early diagnosis and
multidisciplinary management according to
established protocol.

References
1. Glaser RL, Jiang W, Boyadjiev SA, Tran AK,
Zachary AA, Van Maldergem L, et al. Paternal
origin of FGFR2 mutations in sporadic cases

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