Floating-Harbor Syndrome (FHS)

At a glance

Rare, autosomal dominant syndrome associated with short stature, delayed bone age, and characteristic triangular face with a prominent nose and deep-set eyes. Language delay is frequent (usually in the presence of normal motor development).

Synonyms

Leisti Hollister Rimoin Syndrome; Pelletier-Leisti Syndrome.

History

The unusual name of this syndrome is derived from the Boston Floating Hospital and the Harbor General Hospital, where the first cases were described.

Clinical aspects

Features include characteristic facies (dolichocephaly/scaphocephaly, short neck, prominent occiput, triangular face, short midface, low-set ears, broad nasal root, bulbous nose, long eyelashes, enophthalmos, thickened gingivae, narrow palate), limbs anomalies (brachydactyly, delayed bone age, joint dislocation, restricted joint mobility, clinodactyly of toes, clinodactyly of fifth finger), genitourinary signs (micropenis, urethral anomalies), and short stature. Difficult feeding in infancy, mental retardation, and speech defect are common. Cardiac septal defect and celiac disease may be associated.

Anesthetic considerations

Tracheal intubation can be difficult because of skull and neck abnormalities and may require adapted anesthetic management. Avoid using muscle relaxants until airway is secured. Careful intraoperative positioning is needed. Prophylactic antibiotics in case of cardiopathy as indicated.

References