Otodental Syndrome: A Case Report

Resident: Adam J. Bottrill
Date: 11JUN10
Region: Providence
Article title: Otodental Syndrome
Author(s): Colter, J.D. et al
Journal: Pediatric Dentistry
Page #s: pp. 482-485
Year: 2005, 27:6
Major topic: Otodental Syndrome
Minor topic(s): NA
Type of Article: Case Report
Main Purpose: Present a case report of the rare Otodental Syndrome

Key points in the article discussion:

I. Otodental Syndrome

A. Abnormalities in dental crown morphology

1. Max/Man primary and permanent incisors normal
2. Canines and molars large and bulbous in both dentitions
3. Canines of both dentitions present with yellow hypoplastic areas on labial surf.
4. Cusps of affected molars separated by deep vertical enamel fissures.
5. Abnormal molars MAY be result of fusion.
6. Pulp chambers of molars often duplicated. (difficult endo)
7. Absent or micro premolars.
8. Conical supernumeraries and odontomas.

B. Hearing

1. Loss of hearing typically above 1,000Hz.
2. Can start as early as 2-3 yo or as late as puberty.
3. Not all patients present with hearing loss.

C. Etiology

1. Inherited... Autosomal dominant.
2. Variable penetrance and expressivity.
3. Genes: BMP4, MSX1, FGF8, BARX1, DLX1/2

D. Case

1. 9 yo male, NSMH (hearing was not effected),
2. DH: ext of Max left prim 2nd molar due to ectopic eruption of perm molar.
3. Exam: Multiple carious lesions, canines and molars large, spherical and bulbous in perm and prim dentition, deep vert enamel fissures, 6-8 cusps on each tooth, hypoplastic areas on labial surfaces of canines, duplicated pulp chambers, supernumeraries or odontomas, micro premolars, 4. Hearing test not performed.

E. Dental Concerns

1. Dx from dentist (semper vigilans!!!)
2. Future endo concerns
3. OH and preventative care EXTREMELY IMPORTANT

Assessment of article: If I were to do a case presentation on otodental syndrome, I probably would have waited for the hearing tests to be done before I published... shenanigans.

Severe Periodontitis in a 5 year old Girl with Hyperimmunoglublin E Syndrome

Resident: Roberts

Date: 6/11/10

Article title: Severe Periodontitis in a 5 year old Girl with Hyperimmunoglublin E Syndrome

Author: Tsang, P et al.

Journal: Pediatric Dentistry

Volume: 27:1 pages: 73

Year: 2005

Type of article: Case report

Discussion:

Hyperimmunoglobulin E syndrome is a multisystem(HIES) disorder that affects the 1. dentition 2. skeleton 3. connective tissues 4. immune system. This disorder is also known as Jobs syndrome and Buckley’s syndrome. It can be autosomal dominant or recessive or may not have any apparent genetic link at all. Major manifestations of the disease include elevated serum E levels, chronic eczematoid dermatitis, recurrent skin abcesses, prominant forehead, deep set eyes, broad nasal bridge, mild prognathism, scoliosis, joint hyperextensibility and decreased bone density. Retention of primary teeth is a feature found in Autosomal dominant patients. In the recessive form skeletal and dental abnormalities are not usually present.

Case report

A 5 year old girl who has suspected autosomal recessive HIES syndrome presented with profuse bleeding, painful gingiva and generalized aggressive periodontitis. A microbiological examination detected P. gingivalis, T. forsythia, P, nigrescens, T. denticola, E. corrodens, and C. rectus but did not show the presence of A. A. All teeth except E, F, G showed 5 mm or more of probing depths and class 3 mobility. Advanced alveolar bone loss was present and only minor interproximal lesions present. Treatment included extraction of all primary teeth which resulted in elimination of soft tissue inflammation. Main management strategies are 1. Prophylatic antibiotics, 2. timely treatment of infections, 3. surgical intervention as necessary.

Trisomy 9

Resident’s Name: Joanne Lewis Date: June 11, 2010

Article title: Dental Management of a Child With Trisomy 9 Mosaicism: A Case Report

Author(s): Moti Moskovitz, DMD, PhD, et al

Journal: Pediatric Dentistry – 28:3 2006

Type of Article: case report

Summary: Affected patients present with congenital heart disease, skeletal and genito-urinary anomalies, abnormal palmar creases, failure to thrive, hypotonia, and mental retardation. Facial/oral manifestations include upward-slanted eyes, small palpebral fissures, microphthalmos, broad base and prominent tip of the nose, low-set malformed ears, microcephaly, micrognathia, protruding upper lip, pouched cheeks, cleft lip/palate, narrow high-arched palate, small mouth, and down-turned mouth. Mosaicism for trisomy 9 predicts longer survival than non mosaic trisomy 9. The patient in this case report was born at 37 weeks gestation after an uncomplicated pregnancy to Ashkenazi Jewish parents who have 4 other healthy children. Facial dysmorphic features include: narrow high arched palate, short philtrum, low set ears. Oral findings include a supernumerary tooth, and opalescent color change in maxillary central incisors. Also, severe psychomotor retardation and short stature. The patient was treated at age 11 under GA, and again at age 13 under oral conscience sedation. Skeletal abnormalities and tendency to vomit were reasons to treat in a more upright position and to use constant suction during treatment.

Dental Treatment of a Child with Rubinstein-Taybi Syndrome

Department of Pediatric Dentistry

Lutheran Medical Center

Kris Hendricks Date: 6-11-10

Article title: Dental Treatment of a Child with Rubinstein-Taybi Syndrome

Author(s): Davidovish, Eimerl, Peretz

Journal: Pediatric Dentistry

Volume (number): 27/5

Month, Year: 2005

Major topic: Rubinstein-Taybi syndrome (RTS)

Minor topics: N/A

Type of Article: Case Report

Main Purpose:

Demonstrate the oral and dental manifestations of a 12-year old girl with RTS and discuss her dental treatment with emphasis on her medical consideration.

Findings:

RTS is a genetic disorder characterized by mental retardation, and physical abnormalities including broad thumbs, big and broad toes, short stature and craniofacial abnormalities.

The oral manifestations include small opening, pouting lower lip, retro/micrognathia and higher arched, narrow palate.

Key points/Summary :

There are very few reports of this syndrome in the dental literature.

Rarely this syndrome can present with clefting.

This patient had to be treated under GA.

The anesthesia was complicated, so they used fiberoptic endoscopy.

This patient had significant crowding and an orthodontist recommended premolar extractions to alleviate crowding, but the foster parents did not choose to do that.

The foster parents were unable to convince the dental team that they could care for the child’s needs.

Assessment of article:

Nice article.