J Korean Assoc Oral Maxillofac Surg.  2018 Aug;44(4):198-203. 10.5125/jkaoms.2018.44.4.198.

Classical oral manifestations of Dyke-Davidoff-Masson syndrome: a case report with review of the literature

• Kalaskar R
• Kalaskar A

• KMID: 2419236
• DOI: http://doi.org/10.5125/jkaoms.2018.44.4.198

Abstract

Dyke-Davidoff-Masson syndrome is a non-inherited rare condition that presents during childhood and is characterized by seizures, hemiplegia, mental retardation, cerebral hemiatrophy, calvarial thickening, and hyperpneumatization of the frontal sinuses. The present article highlights a case of a 12-year-old male child with additional clinical findings of café-au-late pigmentation and ocular lipodermoid. This is the first case report of Dyke-Davidoff-Masson syndrome to describe oral manifestations, such as unilateral delayed eruption of teeth, hypoplasia, and taurodontism, which could be unique and characteristic of this condition. Oral health care providers and physicians should be aware of these oral observations as dental referrals could warrant early dental prophylactic care and can be useful in diagnosing the possible time of injury and type of Dyke-Davidoff-Masson syndrome.

Keyword

Cerebral atrophy; Enamel hypoplasia; Open bite; Impacted deciduous tooth; Seizures

MeSH Terms

Child
Dental Enamel Hypoplasia
Frontal Sinus
Hemiplegia
Humans
Intellectual Disability
Male
Open Bite
Oral Health
Oral Manifestations*
Pigmentation
Referral and Consultation
Seizures
Tooth

Figure

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  Fig. 1 Extraoral photograph showing facial asymmetry (A), café-au-late pigmentation (B), and lipodermoid of the left eye (C).

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  Fig. 2 Intraoral photograph showing hypoplastic anterior teeth, severe malocclusion with an open bite (A), and a bucally erupting #35 (B).

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  Fig. 3 Panoramic radiograph showing delayed eruption of the right side permanent teeth and taurodontism.

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  Fig. 4 Computed tomography image of the brain showing mild atrophy of the left cerebral hemisphere, dilatation of the left lateral ventricle, sulcogyral space prominence, and calvarial thickening of the frontal and parietal bones.

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  Fig. 5 Computed tomography image of the brain showing left frontal sinus enlargement, thickening of the squamous part of the temporal bone (A), and mild elevation of the left orbital roof (B).

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