Panhipopituitarizm, hipotiroidi ve Noonan sendromlu pediatrik hastada anestezik yaklaşım: Olgu sunumu

Noonan sendromu değişik patofizyolojik düzensizlikler ve değişik anatomik anomalilerle görülen, otozomal dominant genetik geçişle karakterize bir hastalıktır. Bu sendromla ilişkili anomaliler, hipertelorizm, pitozis, mikrognati,aşağı dönük palpebral fissürler, düşük yerleşimli-anormal heliksli kulaklar, derin oluklu filtrum, kısa ve/veya yele bo- yun, düşük ense saç çizgisi şeklinde dismorfik bulgulardır. Noonan sendromu tanısı almış hastalar zor hava yolu ile karşımıza çıkar. Trakeal entübasyon hava yolu ve servikalvertebral anomalilere ve maske ile ventilasyon asimetrikyüz yapısına bağlı olarak zor olabilir. Biz Noonan send-romlu bir olguya anestezik yaklaşımımızı sunmak istedik.

Anesthetic management in a pediatric patient with Noonan syndrome, hypopituitarism and hypothyroidism: A case report

Noonan syndrome is a genetically transmitted autosomal dominant disorder characterized by various anatomic anomalies and pathophysiologic derangements. Associ- ated anomalies include hyperthelorism, ptosis, micrognathia, downward sloping palpebral fissures, low-set ears, abnormal helix of ear, deeply grooved philtrum, short and/ or webbed neck, low hairline and cervical vertebral anomalies. Patients with Noonan syndrome are known to present with challenging airways. Tracheal intubation can be difficult because of airway and cervical vertebral anomalies and bag mask ventilation may be difficult because of asymmetrical face. We present a case of anesthetic management for Noonan syndrome.

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1. Allanson JE. Noonan syndrome. J Med Genet 1987;24:9-13.
2. Sharland M, Burch M, McKenna WM, Patton MA. A clinical study of Noonan syndrome. Arch Dis Child 1992;67:178-183.
3. Bajwa SJ, Gupta S, Kaur J, Panda A, Bajwa SK, Singh A et al. Anesthetic concideration and difficult airway management in a case of noonan syndrome. Saudi J Anaesth 2011;5:345-347.
4. Aggarwal V, Malik V, Kapoor PM, Kiran U. Noonan syndrome: an anesthesiologist’s perspective. Ann Card Anaesth 2011;14:214-217.
5. De Rocca Serra-Nedelec A, Edouard T, Trequer K, et al. Noonan syndrome- causing SHP2 mutants inhibit insulin-like growth factor 1 release via growth hormone-induced ERK hyperactivation, which contributes to short stature. Proc Natl Acad Sci USA 2012;109:4257-4262.
6. Magboul NM. Anaesthetic management of emergency caeserean section in a patient with Noonan’s syndrome-case reports and literature review. Middle East J Anesthesiol 2000;15:611-617.
7. Macksey LF, White B. Anesthetic management in a pediatric patient with noonan syndrome, mastocytosis, and von willebrand disease: a case report. AANAJ 2007;75:261-264.
8. Nakagawa M, Kinouchi K, Matsunami K, et al. Anesthetic management of a child with noonan syndrome and hypertrophic obstructive cardiomyopathy. Masui 2006;55:92-95.
9. Roizen MF, Anethesic implication of concurrent diseases, Miller RD, Anesthesia. 5st ed, Philadelphia, Churchill Livingstone, 2000;903-1015.