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Hereditary angioedema: A rare presentation after anterior cervical discectomy and fusion.

Umerani MS, Alzahrani K, Mostafa GA - Asian J Neurosurg (2015 Jul-Sep)

Bottom Line: Hereditary angioedema is an autosomal dominant disorder following a genetic defect of C1 inhibitor gene on chromosome 11.This rare condition presents itself as a recurrent attack of submucosal swelling mostly involving skin, gastrointestinal tract and upper respiratory airway and exacerbates with any physical and emotional stress.Prompt diagnosis and prevention of an acute attack with early recognition and effective treatment can protect the patient from potential laryngeal edema and fatality.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosciences, King Fahad Military Medical Complex, Dhahran, Kingdom of Saudi Arabia.

ABSTRACT
Hereditary angioedema is an autosomal dominant disorder following a genetic defect of C1 inhibitor gene on chromosome 11. This rare condition presents itself as a recurrent attack of submucosal swelling mostly involving skin, gastrointestinal tract and upper respiratory airway and exacerbates with any physical and emotional stress. Prompt diagnosis and prevention of an acute attack with early recognition and effective treatment can protect the patient from potential laryngeal edema and fatality.

No MeSH data available.


Related in: MedlinePlus

Photograph of the patient taken 8 h after surgery showing swelling of lips and tongue
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Figure 1: Photograph of the patient taken 8 h after surgery showing swelling of lips and tongue

Mentions: Hereditary angioedema is a rare form of autosomal dominant inherited disease due to deficiency of C1-INH. We report a case of a 50-year-old male known to have diabetes mellitus, hypertension, ischemic heart disease, dyslipidemia, addiction to tobacco smoking and some unknown allergic reaction to face, mouth and legs, which according to him was mostly after taking milk or milk products. He presented to us with chronic cervical pain and intractable bilateral brachalgia with X-rays cervical spine showing C5/6 cervical spondylosis and magnetic resolution imaging revealing severe canal stenosis at the same level. He had angioplasty done 2 years back under general anesthesia for anterior wall myocardial infarction (ejection fraction of 35%). He gave a vague history of some allergic reaction to face and oral cavity a year back. No work up was done and without knowing the actual culprit, he was advised to restrain from milk and milk products. In view of his past medical history, cardiology and internal medicine consultations were taken for risk stratification. Internal medicine physician after evaluation advised to send serum levels of C3, C4 and C1 inhibitor levels and to transfuse about 2 L of FFP before surgery. Considering his cardiac status the anesthetist did not allow for the transfusion. So with the informed and written consent he underwent C5/6 anterior cervical discectomy with fusion that was uneventful. Eight hours after surgery he developed a progressively increasing swelling at both legs, lips, and tongue with difficulty in swallowing without any pruritus or urticaria that did not subside with glucocorticoid or antihistamine therapy [Figure 1]. His condition improved with the administration of intravenous plasma-derived C1-INH advised by the internal medicine physician, and the clinical features of the state started to disappear in the very next hour [Figure 2]. He thereafter recovered and was later discharged in good health.


Hereditary angioedema: A rare presentation after anterior cervical discectomy and fusion.

Umerani MS, Alzahrani K, Mostafa GA - Asian J Neurosurg (2015 Jul-Sep)

Photograph of the patient taken 8 h after surgery showing swelling of lips and tongue
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4553747&req=5

Figure 1: Photograph of the patient taken 8 h after surgery showing swelling of lips and tongue
Mentions: Hereditary angioedema is a rare form of autosomal dominant inherited disease due to deficiency of C1-INH. We report a case of a 50-year-old male known to have diabetes mellitus, hypertension, ischemic heart disease, dyslipidemia, addiction to tobacco smoking and some unknown allergic reaction to face, mouth and legs, which according to him was mostly after taking milk or milk products. He presented to us with chronic cervical pain and intractable bilateral brachalgia with X-rays cervical spine showing C5/6 cervical spondylosis and magnetic resolution imaging revealing severe canal stenosis at the same level. He had angioplasty done 2 years back under general anesthesia for anterior wall myocardial infarction (ejection fraction of 35%). He gave a vague history of some allergic reaction to face and oral cavity a year back. No work up was done and without knowing the actual culprit, he was advised to restrain from milk and milk products. In view of his past medical history, cardiology and internal medicine consultations were taken for risk stratification. Internal medicine physician after evaluation advised to send serum levels of C3, C4 and C1 inhibitor levels and to transfuse about 2 L of FFP before surgery. Considering his cardiac status the anesthetist did not allow for the transfusion. So with the informed and written consent he underwent C5/6 anterior cervical discectomy with fusion that was uneventful. Eight hours after surgery he developed a progressively increasing swelling at both legs, lips, and tongue with difficulty in swallowing without any pruritus or urticaria that did not subside with glucocorticoid or antihistamine therapy [Figure 1]. His condition improved with the administration of intravenous plasma-derived C1-INH advised by the internal medicine physician, and the clinical features of the state started to disappear in the very next hour [Figure 2]. He thereafter recovered and was later discharged in good health.

Bottom Line: Hereditary angioedema is an autosomal dominant disorder following a genetic defect of C1 inhibitor gene on chromosome 11.This rare condition presents itself as a recurrent attack of submucosal swelling mostly involving skin, gastrointestinal tract and upper respiratory airway and exacerbates with any physical and emotional stress.Prompt diagnosis and prevention of an acute attack with early recognition and effective treatment can protect the patient from potential laryngeal edema and fatality.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosciences, King Fahad Military Medical Complex, Dhahran, Kingdom of Saudi Arabia.

ABSTRACT
Hereditary angioedema is an autosomal dominant disorder following a genetic defect of C1 inhibitor gene on chromosome 11. This rare condition presents itself as a recurrent attack of submucosal swelling mostly involving skin, gastrointestinal tract and upper respiratory airway and exacerbates with any physical and emotional stress. Prompt diagnosis and prevention of an acute attack with early recognition and effective treatment can protect the patient from potential laryngeal edema and fatality.

No MeSH data available.


Related in: MedlinePlus