Anesthetic considerations and difficult airway management in a case of Noonan syndrome

Abstract

Noonan syndrome is a genetically transmitted autosomal dominant disorder characterized by various anatomic anomalies and pathophysiologic derangements. Anesthetic management in such cases poses a multitude of challenges, especially related to the airway management and maintenance of cardiovascular stability. We report a case of a 9-year-old male child weighing 24 kg, who was diagnosed as a case of Noonan syndrome and had undergone ligation of patent ductus arteriosus during early childhood. The child was operated on for release of bilateral neck bands under general anesthesia. The case report pertains to the successful airway and anesthetic management in the background of difficult airway and existence of various cardiac lesions.

Craniofacial and maxillary anomalies: Anesthetic implications and management

“…These challenges get accentuated when such patients present for surgical intervention. [1][2][3][4][5][6] With the advent of modern craniofacial surgical interventions, these facial defects can be corrected to a large extent and can help the person to lead a normal life. Paul Tessier, the father of craniofacial surgery, has suggested various surgical techniques for repair of these defects involving a multidisciplinary approach involving plastic surgeon, neurosurgeon, maxillofacial surgeon, an ophthalmologist, an otolaryngologist, orthodontist, pedodontist, a pediatrician, speech specialist and Numerous congenital and traumatic cranio-maxillary malformations can be encountered in clinical practice, but we are limiting our discussion to the commonly faced anomalies.…”

Anaesthesia for emergency ventriculo-peritoneal shunt in an adolescent with Noonan′s syndrome

“…[5] Though difficult airway was anticipated in our patient, intubation could be successfully accomplished with a bougie.…”

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

“…Bu durum preoperatif hava yolu değerlendirmesinin ayrıntılı yapılmasını gerektirmiştir. Bajwa ve ark' [3] ları, zor hava yolu ve değişik kardiyak lezyonları olan Noonan sendromlu hastada, bilateral boyun bandı düzeltilmesi ameliyatı için genel anestezi uy- Aggarwal ve ark' [4] ları, yaptıkları olgu sunumunda, Noonan sendromlu hipertrofik obstriktif kardiyomiyopati'si olan çocuklarda, hemodinamik dalgalanmalar nedeniyle gelişebilen akut konjestif kalp yetmezliğine rastlanabileceğini, bununla beraber ilaç ve sıvı tedavisinin en iyi şekilde yapılması, anestezinin idaresinde dikkatli gözlem ve peroperatif yeterli analjezi sağlanması gerektiğini söylemiş-lerdir. Bizim olgumuzda pulmoner stenoz dışında kalp lezyonu saptanmamıştır.…”

“…Noonan sendromlu hastalarda, hava yolu güç-lükleri olabileceği göz önüne alınarak, postoperatif dönemdeki ekstübasyon ve solunum komplikasyonları açısından dikkatli olmak gerekir [3]. Bu nedenle hastamızı tamamen uyanıkken ve refleksleri geri dönünce ekstübe ettik.…”