Congenital Disorders of the Mouth and Pharynx Flashcards
T/F: Cleft palate is typically associated with a genetic disorder.
False. The disorder can run in families but the majority of cases are not involved with any genetic syndrome.
What is the zona pellucida and what does its presence indicate?
A blue line visible in the midline of the soft palate due to a lack of midline musculature. It indicates the presence of a submucosal cleft palate.
How is tongue-tie usually managed?
Most newborns can adjust and do well without operative intervention.
If intervention is indicated in an infant with tongue-tie, what is the recommended procedure?
Frenulectomy can be performed in the outpatient office setting by a dentist or oral surgeon if necessary.
What is the most common location for an ectopic thyroid?
A lingual thyroid is the most common location for an ectopic thyroid (90% of cases).
What physical exam findings would you expect in a patient with a lingual thyroid?
Usually, the lingual thyroid appears as a raised, violaceous mass visible at the base of the tongue.
What are some characteristics of a thyroglossal duct cyst?
They are cystic masses in the midline of the neck which typically move with swallowing or movement of the tongue.
What is the significance of thyroglossal duct cysts?
Generally, the cysts are symptomatic unless they become infected. If infection occurs, the cyst can increase in size rapidly and cause respiratory compromise.
What is the recommended management of thyroglossal duct cysts?
They should be removed surgically. However, if infection is involved, treat with antibiotics first and remove surgically once the infection has resolved.
How does Oral-facial-digital syndrome Type 1 present?
It is an X-linked disorder that presents with cleft tongue, hypoplasia of the alar nasal cartilages, medial cleft of the upper lip, asymmetric cleft of the palate, digital malformations, and mild intellectual disability. About half of patients have a hamartoma between the lobes of the divided tongue.
What is Mohr syndrome?
An autosomal recessive disorder with lobulated nodular tongue, conductive hearing loss, cleft lip, high arched palate, hypoplasia of the mandible, polydactyly, and syndactyly.
Which two syndromes are associated with the presence of a cleft tongue?
Oral-Facial-Digital syndrome Type 1 and Mohr syndrome.
