MOMO Syndrome

MOMO is an acronym for macrosomia, obesity, macrocephaly, and ocular abnormalities. Macrocephaly and tall stature, delayed bone development, generalized obesity, numerous ocular abnormalities (retinal coloboma and nystagmus), and mental retardation.

Only three cases described; suggested to be a new mutation with autosomal dominant inheritance.

Two unrelated sibs were first described with significant obesity, mental retardation, macrocephaly, delayed bone maturation, retinal coloboma, nystagmus, and downward slant of palpebral fissures. Tall stature, macrostomia, and delayed eruption of teeth.

Based on the few cases described, special attention must be paid to the airways because of the generalized, often morbid obesity. The increased risk of gastric content regurgitation must be kept in mind in patients with severe obesity. The presence of macrocephaly may be associated with macroglossia and potential difficult tracheal intubation. Difficulty in mechanical ventilation preoperatively and postoperatively is possible in presence of severe obesity.