Table 1.

Medical Problems in Williams Syndrome* by Organ System and Age

Organ SystemIncidence (%)Age
InfancyChildhoodAdult
Ocular and visual
 Esotropia50x
 Hyperopia50xx
Auditory
 Chronic otitis media50xx
 Hypersensitivity to sound90xxx
Dental
 Malocclusion85xx
 Microdontia95xx
Cardiovascular
 Any abnormality (total)80xxx
 SVAS75xxx
 SVPS25xxx
 PPS50x
 Renal artery stenosis45xxx
 Other arterial stenosis20xx
 VSD10x
 Hypertension50xx
Genitourinary
 Structural anomaly20xxx
 Enuresis50x
 Nephrocalcinosis<5xxx
 Recurrent urinary tract infections30x
Gastrointestinal
 Feeding difficulties70xx
 Constipation40xxx
 Colon diverticula30xx
 Rectal prolapse15xx
Integument
 Soft lax skin90xxx
 Inguinal hernia40x
 Umbilical hernia50x
 Prematurely gray hair90x
Musculoskeletal
 Joint hypermobility90xx
 Joint contractures50xxx
 Radioulnar synostosis20xxx
 Kyphosis20x
 Lordosis40xx
 Awkward gait60xx
Calcium
 Hypercalcemia15xx
 Hypercalciuria30xxx
Endocrine
 Hypothyroidism2xxx
 Early puberty (but rarely true precocious puberty)50x
 Diabetes mellitus15x
 Obesity30x
Neurologic
 Hyperactive deep tendon reflexes75xx
 Chiari I malformation10xxx
 Hypotonia (central)80xx
 Hypertonia (peripheral)50xx
Cognitive
 Developmental delay95xx
 Mental retardation75xx
 Borderline intellectual functioning20xx
 Normal intelligence5xx
 Impaired visuospatial constructive cognition95xx
Behavioral
 Attention-deficit hyperactivity disorder70x
 Generalized anxiety disorder80xx
  • * Percentages based on the following: 1) review of rates of complications in several reports of series of patients with Williams syndrome, and 2) database of 315 children and adults with Williams syndrome evaluated by Colleen A. Morris, MD. SVAS indicates supravalvar aortic stenosis; SVPS, supravalvular pulmonic stenosis, PPS, peripheral pulmonary artery stenosis; and VSD, ventricular septal defect.