KLEEBLATTSCHADEL

SYNDROME
BY BILBAO,BUENAFE,DE LOS SANTOS
I.INTRODUCTION/DEFINITION
What is Kleeblattschadel Syndrome?
KLEEBLATTSCHADEL SYNDROME IS A VERY UNCOMMON SKULL
MALFORMATION THAT OCCURS WHEN MANY SUTURES BETWEEN
A BABY'S SKULL FUSE TOO EARLY.

IT IS A TYPE OF CRANIOSYNOSTOSIS THAT MAKES THE

SKULL PROTRUDE AND BROADENS THE FACE.

IT IS A SEVERE DEFORMITY THAT GIVES THE SKULL A

CLOVERLEAF SHAPE

IT IS RARE WITH LESS THAN 130 CASE REPORTS GLOBALLY

II. STATISTICAL DATA /EPIDEMIOLOGY

ASPECT INFORMATION

Prevalence Epidemiological data on kleeblattschädel is limited due to its rarity, but it is

estimated to occur in about 3-6 0ut of 10,000 live births.

Environmental:
maternal smoking, teratogen exposure, intrauterine constraint, fetal
Predisposing Factors
positioning
Genetic:
mutations

Genetic Causes Almost 20% of all cases; most inherited in autosomal dominant fashion;
new mutations in 50% of cases

Types Non-syndromic: 75% of cases

Syndromic: 25% of cases
 III. ETIOPATHOLOGY
Kleeblattschadel sydrome a type of Classification is based on the affected
Craniosynostosis from premature fusion of cranial suture:
sutures, disrupting normal skull growth. •sagittal
Genetic mutations and environmental factors like
•coronal
maternal smoking and teratogen exposure •metopic
contribute to its development. •lambdoid being primary classifications.

Genetic causes account for about 20% of cases,

Clinical identification typically occurs within
with most being inherited autosomal dominantly or
arising from new mutations.Non-syndromic the first year of life.
craniosynostosis affects 75% of cases, while
syndromic craniosynostosis affects 25% and is
associated with specific genetic syndromes
IV. CLINICAL FEATURES
The skull has a trilobed appearance
with high forehead, beaked nose, and
depressed premaxillary region
Intellectual Impairment and
Hydrocephalus is common.
Neonatal death is frequent.

Other features involve marked

proptosis, exophthalmos, corneal
ulceration in the eyes and elbow
ankylosis, dysostosis multiplex,
syndactyly of toes and fingers,
restricted joint mobility in the limbs.
Abnormal vertebral size has
occasionally been reported.
IV. ORAL FEATURES
Trilobal skull configuration with downward
displacement of the ears, facial deformity
with hypertelorism, nasal flattening, jaw
abnormalities, skeletal abnormalities.
Reports of optic atrophy, respiratory
problems, disorders of speech and hearing.

Anterior 3D volume rendering images showing multiple cranial deformities status post
craniotomies for hydrocephalus, as well as severe skeletal and occlusal abnormalities.

Blindness, obstructed nasolacrimal ducts,

absent external auditory canals,
macrostomia, macroglossia, oblique facial
clefting, cleft lip-palate, bifid uvula, highly
arched palate, natal teeth, and
malocclusion.

Para-sagittal and Lateral 3D volume rendering images displaying prominent apertognathia

and deficiency of the mid-maxilla with rotation of the maxillary alveolus superiorly
V. DIAGNOSIS
DIAGNOSTIC AIDS TO HELP YOU IDENTIFY KLEEBLATTSCHADEL SYNDROME

EXAMINING AND MEASURING THE

HEAD

RADIOGRAPHS

DIAGNOSTIC IMAGING
EXAMINING AND
MEASURING THE HEAD
RADIOGRAPHS
DIAGNOSTIC IMAGING
OR CT SCANS
VI. TREATMENT MANAGEMENT
The major goals of corrective surgery
eliminate the pressure on the brain
correct midfacial deformities
open nasopharyngeal airways,
 SURGERY
Surgery must be done as early as possible
Minimally invasive surgery for infants
younger than 3 months to open skull
sutures for normal skull and brain
growth
Traditional surgery for infants 6 months
or older to correct the skull’s shape
VII. RELATION TO DENTISTRY
The relation of clover leaf skull in dentistry is that
patients with craniofacial anomalies require
orthodontic services as a direct result of the
medical condition and as an integral part of the
rehabilitative process.
Whether a deformity is congenital, acquired or
developed, maxillofacial treatment offers life-
altering physical, cosmetic and psychosocial
benefits
3D reconstruction of the child’s skull which could be used to operate on and successfully
repair the deformity in Brazil.

Craniofacial orthodontics uniquely combines

various disciplines within the medical community,
including orthodontics, dentistry, head and neck
oncology, speech therapy, congenital
malformation reconstruction, plastic surgery, etc.
for optimal results for each patient.
REFERENCES:
• HTTPS://WWW.DEEPDYVE.COM/LP/SPRINGER-JOURNAL/CROUZON-SYNDROME-WITH-KLEEUBLATTSCH-DEL-
SKULL-SATHGA0NP0?KEY=SPRINGER

• HTTPS://WWW.NCBI.NLM.NIH.GOV/BOOKS/NBK544366/

• HTTPS://WWW.CHILDRENSNATIONAL.ORG/GET-CARE/HEALTH-LIBRARY/CLOVERLEAF-DEFORMITY?
FBCLID=IWAR3ETQ9RSTTAUU68NLC6QPWMZYCOCPCY8OYDA1Z0BAY9WH5YKC5B_OUQB9E

• HTTPS://L.MESSENGER.COM/L.PHP?U=HTTPS%3A%2F%2FRADIOPAEDIA.ORG%2FARTICLES%2FCLOVERLEAF- S
KULL-CRANIOSYNOSTOSIS&H=AT0-U9CSU5B7QTJHWN3RFVRG-ZKN1JAOLOCJ1TQYMIYXVDPTXPHVPSFKDKELJXJF6-
RKUISJ4WDM8BTLV0UMHNT5JN4Y_OVP7MJJCZZ_PURPEBV4H6NVMRFDTRHESVMOSTNHHNMAKRDWU_RZJIH_GA

(PDF) KLEEBLATTSCHÄDEL SKULL: A REVIEW OF ITS HISTORY, DIAGNOSIS, ASSOCIATIONS, AND TREATMENT
(RESEARCHGATE.NET)

HTTPS://WWW.SEMANTICSCHOLAR.ORG/PAPER/CLOVERLEAF-SKULL-SYNDROME-

HTTPS://ACCESSANESTHESIOLOGY.MHMEDICAL.COM/CONTENT.ASPX?BOOKID=852&SECTIONID=49517406

HTTPS://WWW.HINDAWI.COM/JOURNALS/IJD/2012/145749/
 QUIZ
1. What is Kleeblattschadel Syndrome? 4. What percentage of Kleeblattschadel Syndrome
a) A common skull malformation cases are attributed to genetic causes?
b) A rare skull malformation resulting from premature a) 5%
fusion of cranial sutures b) 20%
c) A type of brain disorder c) 50%
d) A genetic syndrome affecting limb development d) 75%

2. What is the estimated prevalence of Kleeblattschadel 5. What are the primary types of craniosynostosis?
Syndrome? a) Sagittal, coronal, metopic, and lambdoid
a) 1 in 100 live births b) Occipital, temporal, frontal, and parietal
b) 1 in 1,000 live births c) Central, peripheral, axial, and appendicular
c) 3-6 out of 10,000 live births d) Anterior, posterior, lateral, and media
d) 1 in 1,000,000 live births es

3. Which of the following is NOT a predisposing factor for

Kleeblattschadel Syndrome?
a) Maternal smoking
b) Teratogen exposure
c) Fetal positioning
d) Neonatal nutrition
 QUIZ
1. What is Kleeblattschadel Syndrome? 4. What percentage of Kleeblattschadel Syndrome
a) A common skull malformation cases are attributed to genetic causes?
b) A rare skull malformation resulting from premature a) 5%
fusion of cranial sutures b) 20%
c) A type of brain disorder c) 50%
d) A genetic syndrome affecting limb development d) 75%

2. What is the estimated prevalence of Kleeblattschadel 5. What are the primary types of craniosynostosis?
Syndrome? a) Sagittal, coronal, metopic, and lambdoid
a) 1 in 100 live births b) Occipital, temporal, frontal, and parietal
b) 1 in 1,000 live births c) Central, peripheral, axial, and appendicular
c) 3-6 out of 10,000 live births d) Anterior, posterior, lateral, and media
d) 1 in 1,000,000 live births es

3. Which of the following is NOT a predisposing factor for

Kleeblattschadel Syndrome?
a) Maternal smoking
b) Teratogen exposure
c) Fetal positioning
d) Neonatal nutrition