Definition

What is Adams-Oliver syndrome?

Adams-Oliver syndrome (AOS) is a rare disease characterized by an abnormality of skin development (areas of missing skin on the scalp called aplasia cutis congenita) and malformations of the hands and feet (terminal transverse limbs defects). The aplasia cutis may involve only the skin or include the skull under the skin. The terminal transverse limb defects may include webbed fingers or toes (syndactyly) and short or missing fingers or toes (brachydactyly or oligodactyly, respectively).

How common is Adams-Oliver syndrome?

Adams-Oliver syndrome is extremely uncommon.Please discuss with your doctor for further information.

Symptoms

What are the symptoms of Adams-Oliver syndrome?

Infants born with Adams-Oliver syndrome will have classic presentation of hairless scarred areas on the scalp. These lesions may be mild to severe but usually their depth is variable. These lesions in some cases tend to get infected and may also ulcerate, although mostly these tend to heal on their own in a few months’ time.

As a result of the wide blood vessels due to Adams-Oliver syndrome, there may be hemorrhage which may complicate the condition a bit.

Apart from the scalp deformities, there are also significant musculoskeletal abnormalities seen with Adams-Oliver syndrome. These abnormalities tend to range from mild to severe.

There may be bone defect or even some cases of complete absence of bone in some areas of the skull. The bones defects in skull may be in cases so severe that the underlying tissues may get exposed predisposing the child to bacterial infections.

Other musculoskeletal abnormalities as a result of Adams-Oliver syndrome are malformations fingers, in the toes or hands which may be either abnormally short or in some cases may be completely absent. Webbed toes and hypoplastic toenails are also one of the characteristic features of Adams-Oliver syndrome.There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes Adams-Oliver syndrome?

The exact cause of Adams-Oliver syndrome is still not known but researchers believe it to be caused by reduced or interrupted blood flow through some arteries during the development of the fetus. These arteries usually are the subclavian artery or the vertebral artery. As stated, Adams-Oliver syndrome follows an autosomal dominant trait in majority of the cases but some cases have been identified as autosomal recessive of Adams-Oliver syndrome.

Risk factors

What increases my risk for Adams-Oliver syndrome?

Please consult with your doctor for further information.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is Adams-Oliver syndrome diagnosed?

Adams-Oliver syndrome can be easily suspected and diagnosed at the time of birth of the child just by visual inspection of the scalp defects in conjunction with malformation of the fingers of the hands and toes. This diagnosis may be further confirmed with a detailed family history to determine whether any other member of the family has had a diagnosis of Adams-Oliver syndrome.

In case if there is a family history of Adams-Oliver syndrome, then a prenatal ultrasound may be recommended to check whether the baby to be born carries this disease condition or not. Advanced imaging studies in the form of MRI or CT scan are also of great value in confirmatively diagnosing Adams-Oliver syndrome.

How is Adams-Oliver syndrome treated?

There is no clear cut treatment for Adams-Oliver syndrome. The treatment for this condition is basically symptomatic and supportive. The treatment for Adams-Oliver syndrome requires a multidisciplinary effort with inputs from various specialists so as to formulate the best treatment options for the child with Adams-Oliver syndrome.

The lesions on the scalp usually tend to heal themselves with time. In some cases, surgery may be required to correct some deformities of the scalp and other musculoskeletal abnormalities. It is also recommended that children wear protective head gear due to the dilated blood vessels to prevent any trauma to the head which can cause significant damage as the skull area is extremely sensitive in children with Adams-Oliver syndrome.

In cases where there is absence or abnormal shortening of the fingers of the hands or toes which may not allow proper functioning of the extremity, prosthesis may be recommended to correct this. Regular cardiac monitoring is also required in case if a cardiac defect is seen in children with Adams-Oliver syndrome.

Genetic counseling is highly recommended for families who have members affected with this disorder so as to determine the risk of having an offspring with Adams-Oliver syndrome.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage Adams-Oliver syndrome?

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Sources

Review Date: February 22, 2018 | Last Modified: February 22, 2018

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