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2. A 13 year old male patient
Master Uma Shankar reported
to Department of Oral
Medicine & Radiology with a
complaint of missing lower
front teeth since 5 to 6 years.

3.  Present history
 Patient lost his milk teeth 5-6 years back.
 Permanent teeth never erupted
 No history of any traumatic injury in oro-facial region.
 Late eruption of teeth.
 Anamnesis
 Uneventful prenatal history
 No childhood disease
 First visit to dentist
 Family history
 Absence of parental consanguinity
 Patient’s one elder and one younger sibling showed none of
clinical aspects typical of the syndrome

4.  Mentally sound
 Normal intelligence
 Short stature

5. Bulging forehead
Depressed nasal bridge
Micrognathia
Retrognathia

6. Beak shaped nose
Hypertelorism
Long philtrum
Down slanting palpeberal
fissure
Triangular mouth

7. Partial Ankyloglossia Midline indentation of tongue

8.  Missing mandibular
anterior teeth
 Multiple carious teeth
 Overretained teeth
 Malaligned teeth

9.  Robinow’s Syndrome

10.  Van der Woude’s Syndrome
 Frasers Syndrome
 Meckle’s Syndrome
 Orofacial Digital Syndrome
 Pierre Robin Anamalad
 Patau Syndrome

11. IOPA: Mandibular Anterior RegionIOPA: Mandibular Anterior Region

14.  Autosomal Dominant Robinow’s Syndrome

15.  Dr Meinhard Robinow in 1969
 Genetic Heterogenicity: Two types
• Dominant type
 Common
 Parents are not carrier of dominant gene that produces syndrome.
• Recessive type
 Rare
 Both parents are carrier(not affected)
(Robinow’s Syndrome, Robinow-Siverman-Smith Sydrome,
Robinow Dwarfism, Fetal Face Syndrome , Fetal Facies Syndrome)

16.  Autosomal recessive form is linked to ROR2 gene on
9q22 chromosome
 Autosomal Dominant- definite gene responsible not
known
 Dominant form is milder form of disease.

17.  Incidence, 1:500,000
 Prevalence, 5-10 % patients die in infancy or
early childhood.
 The male to female ratio, 1:1.

18.  Midfacial hypoplasia
 Hypertelorism
 Pseudoexopthalmous (deficiency of the lower eyelid)
 Wide and down slanting palpebral fissure
 Short upper limbs
 Vertebral anomalies
 Genital hypoplasia
 Brachydactyly

19.  Resemblance to fetal face
• Small face
• Laterally displaced eyes
• Forward pointing alae nasi
Fetal facies become less prominent with time
Short stature
I. Reduced birth length
II. Some rare cases of normal growth

20.  Supernumerary teeth
 Triangular mouth
 Frontal bossing
 Long or short philtrum
 Micrognathia
 Malaligned teeth
 Shortened tongue with midline indentations
 Tented upper lip with Inverted V with tethering in the
center
 Midline clefting of the lower lip

21.  A fetal ultrasound-19 weeks into pregnancy
 Clinical and radiographic findings
 Genetic Mapping

22.  Partial Denture
 Orthodontic treatment
 Restorations
 Frenectomy

23. Robinow’s Syndrome is a rare congenital
genetic disorder with many orofacial and
dental abnormailities. The Oral Physicain
plays an important role in multidiscplinary
approach to the management of these
patients.

24.  M A Patton, A R Afzal. Robinow syndrome; J Med Genet 2002;39:305–310
 M A Sabry et al. Unusual traits associated with Robinow’s Syndrome; J
Med Genet 1997;34:736-740.
 Juliana Forte Mazzeu et al. Clinical Characterization of Autosomal
Dominant and Recessive Variants of Robinow Syndrome; American Journal
of Medical Genetics Part A 143A:320–325 (2007)
 Nadkarni UM. The Robinow Syndrome: A Case Report; J Ind Soc Pedo
Prev Dent 2001; 24-26.
 S Suresh. Robinow syndrome; IJO:October-December 2008/Volume
42/Issue 4