Ehsanipoor Medicine Advanced biomedical research 2017 A 10-year-old boy is presented with extensive aplasia cutis congenita and limb anomalies as well as mild pachygyria and focal acrania in neuroimaging and it is crucial to investigate for internal organ [semanticscholar.org]

In some only hypoplastic fingers are noted whereas others present without hands and/or lower legs. Occasionally intellectual deficit and global developmental delayare seen. [epos.myesr.org]

AOS is a rare disease and even rarer with lesions on the abdomen, which is why this case is being presented [7]. [cureus.com]

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

Ferro, Josefo Pizzol, Damiano Grimalt Santacana, Ramon Cutrone, Mario ISSN Abstract A term infant born in a resource-poor setting (Mozambique) presented with extensive disepithelization of the cranial vertex with absent underlying bony plate, disepithelization [repositori.uic.es]

• Single Transverse Palmar Crease

  transverse palmar crease Abnormality of prenatal development or birth Oligohydramnios Abnormality of the eye Developmental cataract Hypertelorism Microphthalmia Optic atrophy Strabismus Abnormality of the integument Aplasia cutis congenita Cutis marmorata [ncbi.nlm.nih.gov]

• Tachycardia

  The only significant part for the entire duration of the pregnancy was the suggestion of a C-section due to fetal distress exhibited as tachycardia at term. [cureus.com]

• Eruptions

  The absence of bullous eruptions and the absence of the involvement of sites of friction sites rule out epidermolysis bullosa. Herpes simplex, although often found on the scalp, is not associated with limb or abdomen abnormalities. [cureus.com]

We present a case of a newborn with characteristic clinical and imaging features of AOS2 to familiarize the radiologist with these features, since accurate radiological workup may be valuable in determining the definite diagnosis, which ultimately is [epos.myesr.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 towards the specific symptoms that are apparent in an individual. Treatment may require the coordinated efforts of a team of specialists. [rarediseases.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]

[Aplasia cutis congenita: surgical treatment and results in 36 cases]. Cir Pediatr. 2007, 20:151-5. Mendiratta V, Marak A, Chander R, Yadav A: Adams-Oliver syndrome: report of a sporadic case with limited cutaneous expression. [cureus.com]

Surgical treatment of aplasia cutis in the Adams-Oliver syndrome. J Craniofac Surg. 2001;12:569–72. [PubMed: 11711825] Bernbeck B, Schwabe J, Groninger A, Schaper J, Messing-Jünger H, Mayatepek E, Rosenbaum T. [0-www-ncbi-nlm-nih-gov.brum.beds.ac.uk]

[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]

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]

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]

[citation needed] Epidemiology[edit] AOS is a rare genetic disorder and the annual incidence or overall prevalence of AOS is unknown. [en.wikipedia.org]

The Adams-Oliver syndrome in Spain: the epidemiological aspects An Esp Pediatr. 1996;45:57-61. [PubMed: 8849132] McBride KL, Riley MF, Zender GA, Fitzgerald-Butt SM, Towbin JA, Belmont JW, Cole SE. [0-www-ncbi-nlm-nih-gov.brum.beds.ac.uk]

Pathophysiology The pathophysiologic mechanism of AOS remains unknown. [accessanesthesiology.mhmedical.com]

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]

The altered protein is unable to bind to DNA, preventing it from turning on particular genes. These changes in gene activity impair the proper development of the skin, bones, and other tissues, leading to the features of Adams-Oliver syndrome. [encyclopedia.pub]

Wearing helmets may be recommended for some children with AOS to prevent trauma to the head and potential damage to the wide 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]