Nasal Closure for Hereditary Hemorrhagic Telangiectasia (HHT): (The Lund Modification of Young’S Procedure)
Valerie J. Lund
INTRODUCTION
Hemorrhagic telangiectasia (HHT) is an inherited disorder characterized by telangiectasia with endothelium deficient in muscle or elastic tissue. It was first described in the 1860s by Sutton (1864) and Babington (1865) although the names of Osler, Rendu, and Weber (1901, 1896, and 1907) are more commonly associated with the condition that is autosomal dominant and non-sex linked. Genetic mutations in Cr 9,12,5 with signal transforming growth factor (TGF-beta) cause abnormal development of blood vessels.
HHT is found throughout the world with an incidence of 12.5 to 15.6 per 1,000 of the population, but there are significant geographical variations. The condition can affect any part of the body, in particular cutaneous and mucosal surfaces where the small telangiectasia may be readily seen. However, in organs such as the liver, lung, and brain, larger arteriovenous malformations may be found, which, in the lungs, can lead to significant shunting.
HISTORY
Telangiectasia can potentially cause problems wherever they occur, but the most common and troublesome symptom is epistaxis, which is found in over 80% of patients. This can vary from slight spotting of blood to repeated life-threatening hemorrhages. Bleeding usually starts in childhood or the early teens, and there is a tendency for it to become worse with age, although this may be due to other age-related comorbidities. Patients can also present in later life; for instance, in my personal cohort of 344 patients, the age at presentation ranged from 2 to 70 years.
Just over half of my patients experience nosebleeds daily and nearly two-third have more than three episodes a day on most days. The bleeding can be precipitated by minor events such as drinking a cup of tea, putting on a jumper, or undertaking any form of physical exercise. Extremes of temperature and humidity are also associated with increased bleeding, and many patients cite stress as a precipitating factor.
PHYSICAL EXAMINATION
In many patients, the telangiectasia are obvious on the facial skin, lips, pinna, and hands; particularly on the fingertips and nail beds. In the oral cavity, they are often seen on the tongue and on the hard palate, often anteriorly in relation to the incisive canal. Examination of the nose can easily precipitate bleeding and so must be done with great care and a “no-touch” technique. This will often reveal blood clots and crusts, but lesions may be visible on the anterior aspect of the inferior and middle turbinates and on the anterior septum. The appearance of the telangiectasia does not necessarily correlate with the severity of bleeding, and it is important to assess whether the septum is perforated (usually due to previous cauterization) if closure of the nose is contemplated.
INDICATIONS
Closure of the nose should be considered when other options such as coagulating laser, septodermoplasty, or hormone manipulation have failed or in individuals in whom the bleeding is so severe as to require repeated blood transfusion (Fig. 21.1).
The rationale of nasal closure is to prevent air flow through the nose. This is predicated on the inability of the telangiectasia to constrict in response to even slight trauma including the drying effect of air flow. In my experience, in order for the operation to be successful, the closure must be completely airtight. It is therefore reserved for the most severely affected patients whose quality of life is already poor and whose nose is already permanently blocked with blood clots.
The operation is generally undertaken on both sides of the nose, though it can be confined to the worst side. However, if there is a perforation of the septum, then both sides must be closed to achieve an air tight closure.
CONTRAINDICATIONS
The human is not an obligate nasal breather after the first few months of life, and therefore, there is no specific contraindication to closing the nose other than the patient’s fears (see Pre-operative planning). However, there may be a theoretical concern in those who are morbidly obese or who suffer from sleep apnea.
PREOPERATIVE PLANNING
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