Fig. 2.

Photographs of AKS patients. a Patient 1 at 19 years of age. b Patient 1 demonstrates narrow palate and deeply grooved tongue. c Patient 2 at 9 years of age. Note metopic ridging. d Patient 3 at 11 years. e–g Patient 8 as a newborn, age 16 months and age 4 years 5 months. h Patient 6. Note “M” shaped upper lip and deep groove to tongue. i Patient 7 at age 7. j Patient 7 demonstrates deep groove to tongue and mildly bifid tip. k, l Patient 9 at 6 months. Note excess nuchal skin. m, n Patient 10 at 4 weeks. o Patient 10 at 1 year 9 months. p Patient 10 at 8 years