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Megalencephaly-capillary malformation syndrome and anesthesia

Year 2015, Volume: 25 Issue: 4, 136 - 139, 01.12.2015

Abstract

Megalencephaly-capillary malformation syndrome is characterized with macrocephaly, cerebral cortical malformations, polymic- rogria, asymmetry of brain and whole body, generalized or facial capillary malformations, digital anomalies such as syndactyly or polydactyly and connective tissue disease. Bleeding may be more than expected in these patients because of visceral subcutaneous cavernous hemangiomas and varicose dilatations and may complicate the anesthetic management. The aim of this report is to desc- ribe the anesthetic management of a patient with Megalencephaly-capillary malformation syndrome undergoing surgery for varicose dilatations of the lower extremity

References

  • Moore CA, Toriello HV, Abuelo DN, et al. Macrocephalycutis mar- morata telangiectatica congenita: a distinct disorder with develop- mental delay and connective tissue abnormalities. Am J Med Genet 1997;70:67–73.
  • Mirzaa GM, Conway RL, Gripp KW, et al. Megalencephaly-capil- lary malformation (MCAP) and megalencephaly-polydactyly-pol- ymicrogyria-hydrocephalus (MPPH) syndromes: two closely rela- ted disorders of brain overgrowth and abnormal brain and body morphogenesis. Am J Med Genet A 2012;158A:269-91.
  • Martínez-Glez V, Romanelli V, Mori MA, et al. Macrocephaly–ca- pillary malformation: Analysis of 13 patients and review of the di- agnostic criteria. Am J Med Genet A 2010;152A:3101-6.
  • Yano S, Watanabe Y. Association of arrhythmia and sudden death in macrocephaly-cutis marmorata telangiectatica congenita synd- rome. Am J Med Genet 2001;102:149-52.
  • Aldrete JA, Kroulik D. A postanesthetic recovery score. Anesth Analg 1970;49:924-34.

Megalensefali-kapiller malformasyon sendromu ve anestezi

Year 2015, Volume: 25 Issue: 4, 136 - 139, 01.12.2015

Abstract

Megalensefali-kapiller malformasyon sendromu; makrosefali, kortikal beyin malformasyonları ve polimikrogri, beyin ve vücutta asimetrik büyüme, jeneralize veya fasiyal kapiller malformasyonlar, sindaktili/polidaktili gibi dijital anomaliler ve bağ dokusu has- talıkları ile seyretmektedir. Özellikle visseral subkutanöz-kavernöz hemanjiyomlar ve variköz genişlemeler gibi hastalığa eşlik eden patolojiler ameliyat sürecinde beklenenden daha fazla kanamaya yol açarak anestezi yönetimini komplike hale getirebilmektedir. Bu olgu sunumun da Megalensefali-Kapiller Malformasyon sendromu tanısıyla izlenen ve alt ekstremitede variköz genişlemeler nedeniy- le cerrahi uygulanan bir hastadaki anestezi deneyimimizi paylaşmayı amaçladık

References

  • Moore CA, Toriello HV, Abuelo DN, et al. Macrocephalycutis mar- morata telangiectatica congenita: a distinct disorder with develop- mental delay and connective tissue abnormalities. Am J Med Genet 1997;70:67–73.
  • Mirzaa GM, Conway RL, Gripp KW, et al. Megalencephaly-capil- lary malformation (MCAP) and megalencephaly-polydactyly-pol- ymicrogyria-hydrocephalus (MPPH) syndromes: two closely rela- ted disorders of brain overgrowth and abnormal brain and body morphogenesis. Am J Med Genet A 2012;158A:269-91.
  • Martínez-Glez V, Romanelli V, Mori MA, et al. Macrocephaly–ca- pillary malformation: Analysis of 13 patients and review of the di- agnostic criteria. Am J Med Genet A 2010;152A:3101-6.
  • Yano S, Watanabe Y. Association of arrhythmia and sudden death in macrocephaly-cutis marmorata telangiectatica congenita synd- rome. Am J Med Genet 2001;102:149-52.
  • Aldrete JA, Kroulik D. A postanesthetic recovery score. Anesth Analg 1970;49:924-34.
There are 5 citations in total.

Details

Primary Language Turkish
Journal Section Case Report
Authors

Metin Alkan This is me

Mustafa Arslan This is me

Kutluk Pampal This is me

Gürsel Levent Oktar This is me

Elife Türkan This is me

Publication Date December 1, 2015
Published in Issue Year 2015 Volume: 25 Issue: 4

Cite

Vancouver Alkan M, Arslan M, Pampal K, Oktar GL, Türkan E. Megalensefali-kapiller malformasyon sendromu ve anestezi. Genel Tıp Derg. 2015;25(4):136-9.