If an underlying skull defect, is present then a bone graft may be necessary. [dermatologyadvisor.com]

The most frequent clinical manifestation of AOS is limb anomaly which is commonly asymmetric and presents in different forms such as syndactyly, brachydactyly, oligodactyly, polydactyly, and hypoplastic nails.[3] The second most common presentation is [advbiores.net]

Acronym AOS5 Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. [uniprot.org]

In some cases, additional abnormalities may also be present. Most cases of AOS appear to follow autosomal dominant inheritance but autosomal recessive inheritance has also been reported. [rarediseases.org]

Depending on the presentation of the limb anomalies, modifications to living space may be needed. Mode of inheritance AOS may be inherited in one of two patterns of inheritance: autosomal dominant and autosomal recessive. [my46.org]

• Nail Abnormality

  This can include nail abnormalities, fusion of digits (syndactyly) or short digits (brachydactyly) or missing digits (oligodactyly) Cutis marmorata telangiectatica congenita. [dermnetnz.org]

[…] valvular defects, stenotic lesions of left heart, cardiomyopathy, heart block, malformation of pulmonary vessels, and progressive pulmonary hypertension have been reported in AOS patients.[6],[15],[16] Therefore, echocardiography for screening or regular workup [advbiores.net]

Standard Therapies Treatment The treatment of Adams-Oliver syndrome is directed toward the specific symptoms that are apparent in each individual. Treatment may require the coordinated efforts of a team of specialists. [rarediseases.org]

It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. Our staff consists of biologists and biochemists that are not trained to give medical advice. [uniprot.org]

Treatment Options Treatment of AOS is mainly non-operative. ACC of the scalp should be allowed to heal in by secondary intention. As the child’s hair grows, the ACC scar can be concealed. [dermatologyadvisor.com]

Some individuals with AOS need surgical treatment of their scalp defects by skin grafting, and surgery to repair their heart defects. Depending on the presentation of the limb anomalies, modifications to living space may be needed. [my46.org]

Long-term prognosis for Adams-Oliver syndrome is difficult to predict: it is not adequately described in medical literature due to a dearth of cases. [dermnetnz.org]

[citation needed] Prognosis[edit] The overall prognosis is excellent in most cases. Most children with Adams–Oliver syndrome can likely expect to have a normal life span. [en.wikipedia.org]

Another proposed etiology of AOS includes abnormal pericyte recruitment, supported by a mouse model in which ALK1-deficient mice show poor migration, recruitment, and proliferation of pericytes. [dermatologyadvisor.com]

Minoo Saeidi Imam Hossein Children Hospital, Isfahan University of Medical Sciences, Isfahan Iran Source of Support: None, Conflict of Interest: None DOI: 10.4103/2277-9175.221861 Adams–Oliver syndrome (AOS) is a rare congenital disorder with unknown etiology [advbiores.net]

The Adams-Oliver syndrome in Spain: The epidemiological aspects. An Esp Pediatr 1996;45:57-61. 6. Whitley CB, Gorlin RJ. Adams-Oliver syndrome revisited. Am J Med Genet 1991;40:319-26. 7. Sybert VP. [ijpd.in]

[citation needed] Epidemiology[edit] AOS is a rare genetic disorder and the annual incidence or overall prevalence of AOS is unknown. Approximately 100 individuals with this disorder have been reported in the medical literature. [en.wikipedia.org]

Pathophysiology Many genes have been implicated in the development of AOS. [dermatologyadvisor.com]

The exposed dura was covered with artificial dermis on the seventh day of life to prevent hemorrhage and infection. Postoperative complications included septicemia and repeated longitudinal sinus bleeding despite abdominal muscle fascia grafting. [jamanetwork.com]

In addition, physicians may recommend that some affected children wear helmets in order to prevent trauma to the head and potential damage to abnormally wide (dilated) blood vessels. [rarediseases.org]

If the dura is exposed, it should be covered with full-thickness skin flaps or moist dressings to prevent eschar formation, as they carry high risk of infection and life-threatening hemorrhage. [dermatologyadvisor.com]