A CASE REPORT OF MARFAN’S SYNDROME AND A REVIEW OF THE LITERATURE
A case of Marfan syndrome
Keywords:
Marfan sundrome, Paediatric patient, Skeletal, Ocular, Cardiovascular systemsAbstract
BACKGROUND: Marfan’s syndrome is an autosomal dominant,
multisystemic disorder of the connective tissue with variable
inheritance patterns. Its cardinal features affect mostly the
cardiovascular, ocular and skeletal systems. It is panethnic with no
sex predilection and may be diagnosed prenatally, at birth,
childhood or well into adulthood.
OBJECTIVE: This article aimed at enlightening dental
professionals on the presentation and early recognition of patients
with Marfan’s syndrome.
CASE REPORT: An 11-year old female previously undiagnosed of
any syndrome, presented to the dental clinic with a resolving dental
abscess on tooth 26 and dental caries of 16, 36 and 46. She showed
features of skeletal disproportion, arachnodactyly (long slender
fingers with prominent finger joints), flat feet and pectus carinatum
(pidgeon chest). Facial examination revealed a convex profile and
elongated face, there was no dental crowding. Her dental abscess
was managed by extraction with antibiotic cover while other
carious lesions were restored. She was then referred to an
ophthalmologist and a cardiologist for further examinations and
investigations, which revealed she had features of Marfan
syndrome.
CONCLUSION: This case report demonstrates the early
recognition of signs and symptoms of this syndrome and the
referral of the patient to other professionals for management to
prevent severe life threatening complications associated with the
syndrome.