A case of adams-oliver syndrome.
@article{Seo2010ACO, title={A case of adams-oliver syndrome.}, author={Jong-Keun Seo and Ju-Hyun Kang and Hyun-Jae Lee and Deborah Lee and Ho Suk Sung and Seon-Wook Hwang}, journal={Annals of dermatology}, year={2010}, volume={22 1}, pages={ 96-8 } }
Adams-Oliver syndrome (AOS) is a congenital condition characterized by aplasia cutis congenita, transverse limb defects, and cutis marmorata telangiectatica. AOS can also be associated with extensive lethal anomalies of internal organs, including the central nervous, cardiopulmonary, gastrourointestinal, and genitourinary systems. Generally, the more severe these interrelated anomalies are, the poorer the prognosis becomes. In the relevant literature on this topic, it is somewhat unclear as to…
15 Citations
A Case of Adams–Oliver Syndrome
- MedicineAdvanced 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 involvements.
Adams – Oliver Syndrome
- Medicine, Biology
- 2016
An infant with this uncommon disease associated with multiple midline lesions, which is a rare occurrence is described.
Adams–Oliver syndrome
- MedicineInternational journal of dermatology
- 2014
Adams–Oliver syndrome is characterized by congenital midline scalp defects, asymmetrical distal limb reduction anomalies and mottling of the skin. It is an autosomal dominant trait with highly…
Adams–Oliver Syndrome: A Rare Congenital Disorder
- MedicineCureus
- 2022
We present a case of a two-day-old Asian female infant with typical symptoms of Adams-Oliver syndrome (AOS): two cutaneous lesions including aplasia cutis congenita (ACC) and hypoplastic phalanges.…
Oliver Syndrome in a Newborn : Case Report
- Medicine
- 2017
Adams-Oliver syndrome (AOS) is characterized by a combination of congenital scalp defects (ACC), terminal transverse limb defects and often cutis marmorata telangiectatica congenita (CMTC).
Distal Limb Defects and Aplasia Cutis: Adams-Oliver Syndrome.
- MedicineThe Journal of hand surgery
- 2016
Adams–Oliver syndrome: Report of a sporadic case with limited cutaneous expression
- Medicine
- 2017
A rare case of adams oliver syndrome with limited expression involving scalp, transverse limb defects along with unreported truncal involvement is reported.
A practical approach to the evaluation and treatment of an infant with aplasia cutis congenita
- MedicineJournal of Perinatology
- 2018
This discussion covers the diagnosis of ACC and its subtypes and associations in order to provide a pragmatic, clinically relevant and patient-centered approach to evaluation and treatment.
A Newborn with Scalp Defect and Terminal Limbs Anomalies
- MedicineChallenging Cases in Dermatology Volume 2
- 2019
A newborn presented with aplasia cutis congenita of the scalp, widespread cobblestone appearance, marked atrophy of skin, prominent blood vessels, and many purpuric plaques, which fulfilled the criteria of an extremely rare hereditary disorder.
Aplasia cutis congenita in Korea: Single center experience and literature review
- MedicinePediatrics international : official journal of the Japan Pediatric Society
- 2020
The basic clinical data regarding ACC in Korea is elucidated, including demographics, clinical features, radiological and therapeutic results, to elucidate the rare congenital malformation.
References
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The first child suffered from a very severe expression of the syndrome, with a combination of ACC, syndactyly, cutis marmorata telangiectatica congenita and multiple cardiac and central nervous system malformations which resulted in fatal central respiratory insufficiency.
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The need for thorough medical evaluation in cutis marmorata telangiectatica congenita to exclude associated congenital anomalies is highlighted.
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A Belgian Adams–Oliver syndrome family with 10 affected individuals over four generations, of which six were available for this study, is presented and the MSX1, CART1, P63 (P73L), RUNX2, and HOXD13 genes are sequenced.
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This is the first description of the prenatal diagnosis of Adams–Oliver syndrome and all fetuses showed anomalies of the extremities, aplasia cutis and symmetric defects of the skull, with bone being replaced by collagenous tissue.
Adams–Oliver Syndrome: A Sporadic Occurrence With Minimal Disease Expression
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A child with the sporadic form of the Adams–Oliver syndrome who had minimal disease expression is reported, illustrating the wide clinical spectrum of the syndrome.
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A female neonate born out of the first pregnancy of a healthy, non-consanguineous, Hindu Indian couple, mother and father aged 20 and 30 years, respectively, revealed central cyanosis and generalized reticulate dilated cutaneous vasculature (cutis marmorata telangiectatica congenita [CMTC]).
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An autopsy case of a male fetus with Adams-Oliver syndrome, a healthy, 31-year-old woman and her family and past histories were unremarkable, which has not been reported in Korea is reported.