His Life was On the Edge Due to Adenoid Hypertrophy
A 15-month-old boy with Noonan syndrome was referred to our pediatric pulmonary department to assess the need for noninvasive positive pressure ventilation (NPPV). He required hospital care during the first 3 weeks of life because of transient upper airway obstruction requiring nasal continuous positive airway pressure (CPAP). He was discharged home with nasal oxygen therapy for a few weeks. He also presented a moderated hypertrophic cardiomyopathy treated with beta-blocker therapy that was stopped after 6 months after improvement on ultrasonographic examination.
At the age of 6 months, he was hospitalized during several weeks for an important persistent dyspnea due to rhinitis and major nasal obstruction and at the age of 12 months for a pneumopathy that rapidly responded to oral antibiotics. He was hospitalized again at the age of 15 months because of important desaturations during his daytime nap. Clinical examination was normal during wakefulness, while numerous obstructive apneas, with repeated oxyhemoglobin desaturation followed by frequent awakenings and sweating, were observed during daytime naps. Nocturnal sleep study was therefore undergone and confirmed the previous findings. The patient was discharged home as he was clinically stable. It was noticed that obstructive symptoms improved when the patient slept on supine position.
The majority of children with Noonan syndrome will grow up and function normally in adulthood—they finish high school and have paying jobs—and most adults with Noonan do not require special medical care. So, even though guidelines recommend major precautions for adenoidectomy in patients with severe underlying conditions like our patient, the risk of subsequent complications due to the presence of severe nasal obstruction and a complex clinical history in a patient with Noonan syndrome outweighs the risk of perioperative and/or postoperative complications.
At the age of 6 months, he was hospitalized during several weeks for an important persistent dyspnea due to rhinitis and major nasal obstruction and at the age of 12 months for a pneumopathy that rapidly responded to oral antibiotics. He was hospitalized again at the age of 15 months because of important desaturations during his daytime nap. Clinical examination was normal during wakefulness, while numerous obstructive apneas, with repeated oxyhemoglobin desaturation followed by frequent awakenings and sweating, were observed during daytime naps. Nocturnal sleep study was therefore undergone and confirmed the previous findings. The patient was discharged home as he was clinically stable. It was noticed that obstructive symptoms improved when the patient slept on supine position.
The majority of children with Noonan syndrome will grow up and function normally in adulthood—they finish high school and have paying jobs—and most adults with Noonan do not require special medical care. So, even though guidelines recommend major precautions for adenoidectomy in patients with severe underlying conditions like our patient, the risk of subsequent complications due to the presence of severe nasal obstruction and a complex clinical history in a patient with Noonan syndrome outweighs the risk of perioperative and/or postoperative complications.
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