03/12/2010 Oral manifestations of Ehlers-Danlos syndrome Type VII: histological examination of a primary tooth

Resident: J. Hencler
Date: 03/12/2010
Article title: Oral manifestations of Ehlers-Danlos syndrome Type VII: histological examination of a primary tooth
Author(s): Ooshima ET AL.
Journal: Pediatric Dentistry: April/May, 1990 ~ Vol. 12, No. 2, Pg 102-106
Major topic: Ehlers-Danlos syndrome Type VII and associated dental histology

Type of Article: Case Presentation

Main Purpose: Describe a patient with E-D syndrome Type VII and define the associated dental manifestations.

Background:
E-D syndrome is a heterogeneous grp of hereditable disorders of connective tissue. Clinical feature common to all E-D syndromes include skin hyperextensibility, joint hypermobility, and skin fragility. At least 11 types are known. Oral manifestations include fragility of oral mucosa w/ delayed healing, microdontia, short or malformed roots, large pulp stones and calcification of the pulp, bleeding after tooth brushing, and PD.

Patient:
2 year old Japanese girl with E-D syndrome Type VII dx at 3 months. Physical exam at birth revealed dislocation of right hip, swan neck deformities of the fingers, joint hypermobility, skin fragility, and skin hyperextensibility. Her unaffected parents were first cousins. At 3 year old she had severe genus valgus making it impossible for her to walk.

Materials and Methods:
Measurements of MD/BL dimensions of the primary teeth were made on study models and compared to a mean value. EXTed teeth and pulp tissue were examined under microscope with various dye treatments.

Key points in the article discussion:
E-D syndrome Type VII is caused by the deficiency of procollagen peptidase in collagen biogenesis. In this patient presence of marked joint hypermobility, congenital dislocation of hip, and various skin symptoms at birth were consistent w/ dx of E-D syndrome Type VII. Dental finding in our pt were yellow teeth, microdontia, and bleeding after tooth brushing. The MD/BL dimensions of the pts teeth fell below the mean crown size of primary teeth in normal Japanese children. Pulp stones and pulp obliteration, the principle dental manifestations described in previous reports of E-D syndrome were not observed in our patient on radiographic examination. H & E stained sections of the pulp showed a few minute calcified bodies, etiology unknown. The incidence of pulp calcification appears to increase with age. Therefore it is difficult to conclude that the pulp calcification in the present case is one of the principal oral manifestations of Type VII E-D syndrome. There were several inclusions that appeared fistulous in the dentin of this patient. The inclusions were surrounded by an inner highly calcified region and surrounded by an outer poorly calcified region. The staining revealed lesser amounts of collagen or the presence of non-collagenous substances. A laminated calcified matrix w/ fewer dentinal tubules and multiple cell inclusions also was found in the dentin adjacent to the pulp in the tooth of this patient. The tooth had pulpal inflammation, which may have contributed to the devel of the laminated calcified matrix. The extent of the laminated calcified matrix was unusually thick when compared histologically to tertiary dentin of carious incisors in normal children. These findings may support that the dentin matrix has formed rapidly and elements in the pulp are incorporated into the dentin. However, the odontoblast function appears to be deficient in the production of collagen w/in the dentin matrix. The collagen in the pulp tissue in this E-D syndrome patient showed a wavy pattern, which was different from normal pulp tissue characterized by a network structure.

Summary of conclusions:
Most of the findings in this patient reflect the deficiency in collagen biosynthesis of E-D syndrome Type VII. Other dental manifestations such as PD, poor wound healing after EXT, and malformed roots of deciduous teeth were not observed in this patient.

Assessment of article:
Article was ok. All E-D syndrome patients will not present with every characteristic symptom while symptom severity may vary greatly in different patients. It is important to know the cardinal features of any syndrome so it will be recognized and the appropriate treatment approach can be determined.