Kersey Syndrome

At a glance

It is a genetically transmitted syndrome characterized by disorders of hair (short anagen growth phase of hair follicles) and teeth growth. The Kersey Syndrome is often called the Short Anagen Syndrome (SAS) in comparison with the Loose Anagen Syndrome. This is a condition in which hair does not grow long. It usually observed by parents as the child grows around 2 to 4 years of age. It is a benign disease of the hair cycle. Although this condition is usually benign, its associations with the Trichodental Syndrome (variant of the Kersey Syndrome), synchronized pattern of scalp hair growth, and micronychia have been reported.

Synonyms

Short Anagen Syndrome; Trichodental Syndrome; Trichodental Dysplasia-Microcephaly-Mental Retardation Syndrome.

Incidence

The exact incidence for this medical condition is unknown; however, it is probably not uncommon as it is often misdiagnosed as alopecia areata or trichotillomania. It is only reported in whites and is more common in females than males.

Genetic inheritance

Autosomal dominant transmission. However, it is also believed to be sporadic.

Diagnosis

Suspected by “gentle hair pull” that yields much more hairs than the zero to two hairs per pull in normal patients; confirmed microscopic examination. Affected patients do not feel discomfort as hairs are extracted.

Clinical aspects

Onset between 2 and 5 years of age. The most common dental abnormalities are missing teeth, peg-shaped incisors, and shell teeth. The hair is sparse, brittle, and slow-growing because of a short anagen phase of the hair cycle (hair cannot grow long and affected patients almost never need to have their hair cut). Eyebrows and eyelashes are typically curly. Microcephaly with biparietal narrowing and a thin nose are usually seen. Mild mental retardation is common.

Anesthetic considerations

Careful manipulation of the airway must be provided to avoid damaging potentially brittle teeth. Craniofacial abnormalities must be evaluated for abnormal airway anatomy that could render face-mask ventilation and/or laryngoscopy difficult. Patients with mental retardation may be uncooperative and sedative premedication as well as the presence of the primary caregiver for induction of anesthesia may be helpful.

Other conditions to be considered

• Alopecia Areata: Autoimmune disease resulting in varying amounts of patchy hair loss (it is believed to be a variant of vitiligo).

• Pseudopelade of Brocq: Scarring alopecia resembling alopecia areata resulting in dermal atrophy (“footprints in the snow”), mostly because of lichen planopilaris and chronic cutaneous lupus erythematosus.

• Telogen Effluvium: Nonscarring alopecia with diffuse hair shedding caused by metabolic or hormonal stress or by drug absorption.

• Tinea Capitis: Superficial fungal infection of the skin of the scalp, eyebrows, and eyelashes.

• TIBOLA Syndrome: This is an acronym that stands for TIck-B...