Septodermoplasty
Nithin D. Adappa
INTRODUCTION
Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, is an autosomal dominant vascular disease with an incidence of approximately 1 in 5,000. It is characterized by mucocutaneous telangiectasias and visceral arteriovenous malformations (AVMs). There are a number of genetic abnormalities that cause the disease. Genetic expression of HHT is due to an inappropriate maturation of blood vessels in response to angiogenic stimuli. The blood vessels lack the muscular or elastic tissue and thus tend to bleed from minor trauma, including nasal airflow over attenuated epithelium. Ultimately, the vessels are unable to contract or retract hindering the physiologic coagulation cascade to form a thrombus and control the bleeding.
Treatment is initially conservative therapy with the intent of reducing the frequency and severity of epistaxis. A number of topical agents including nasal saline irrigations, emollients, and hormonal agents such as tamoxifen have been shown to reduce the number and severity of bleeding episodes.
Initial first-line surgical therapy includes laser coagulation of individual telangiectasias. Laser coagulation is preferred over chemical or electrocautery as it is felt to decrease the trauma to surrounding healthy nasal mucosa. When these treatment modalities fail, septodermoplasty is often considered.
Septodermoplasty was first described by Saunders in 1964 with the concept of replacing the anterior nasal mucosa with a split-thickness skin graft (STSG). He described removal of the mucosa along the septum, floor of the nose, and lateral nasal wall. Since its initial description, the procedure is still performed in a similar fashion; however, multiple modifications have been described. Septodermoplasty is a procedure in stepwise treatment of HHT epistaxis following failure of laser cauterization. Failure of septodermoplasty can result in further laser cauterization, revision septodermoplasty, or nasal closure procedures such as Young’s procedure. (See Chapter 21)
Approximately 95% of individuals with HHT eventually develop recurrent epistaxis with 80% to 90% developing them by 21 years of age. Most patients will initially have a small number of bleeding episodes that gradually increase in frequency and duration as they age. Approximately 20% to 25% of patients develop gastrointestinal (GI) bleeding, although it is typically much less severe than any concurrent epistaxis. Pulmonary AVMs present in approximately 40% of patients can result in shunting of air, thrombi, and bacteria that can lead to transient ischemic attacks, embolic strokes, and cerebral or other abscesses. Alternatively, they can present with hemoptysis, spontaneous hemothorax, migraine headaches, polycythemia, and hypoxemia. Cerebral AVMs are present in 5% to 20% of patients. They are generally asymptomatic but can present with neurologic sequelae ranging from seizures to cerebral hemorrhage.
HISTORY
A thorough history including a focused otolaryngologic history should be undertaken with any patient suspected of having HHT. The patient should be specifically questioned on duration, frequency, and laterality of epistaxis. The history should also include current and past topical therapies, and any history of surgery including
prior laser cauterizations or other procedures for epistaxis should be reviewed. Also, current hemoglobin levels as well as the need for oral or intravenous iron supplementation and a history of any blood transfusions should be noted. Finally, a medical history discussing other AVMs of the lungs, brain, liver, and GI tract should be sought. HHT occurs in a wide ethnic and geographic distribution. In most situations, a thorough history including family history reveals either a confirmatory diagnosis or anecdotal history of a family member with HHT. Therefore, upon diagnosis of a new case of HHT, a full genetic evaluation of family members is prudent. There are four diagnostic criteria for the diagnosis of HHT including the following: (1) Recurrent and spontaneous epistaxis; (2) Multiple telangiectasias on the skin of the hands, lips or face, or inside the nose or mouth; (3) AVMs or telangiectasias in the internal organs including the lungs, brain, liver, intestines, stomach, and spinal cord; and (4) A family history (a first-degree relative) who meets criteria or has been genetically diagnosed. A patient has possible HHT if he or she meets two criteria, and a definite HHT diagnosis is made if the patient meets at least three. Patients with less than two criteria are considered unlikely to have HHT.
prior laser cauterizations or other procedures for epistaxis should be reviewed. Also, current hemoglobin levels as well as the need for oral or intravenous iron supplementation and a history of any blood transfusions should be noted. Finally, a medical history discussing other AVMs of the lungs, brain, liver, and GI tract should be sought. HHT occurs in a wide ethnic and geographic distribution. In most situations, a thorough history including family history reveals either a confirmatory diagnosis or anecdotal history of a family member with HHT. Therefore, upon diagnosis of a new case of HHT, a full genetic evaluation of family members is prudent. There are four diagnostic criteria for the diagnosis of HHT including the following: (1) Recurrent and spontaneous epistaxis; (2) Multiple telangiectasias on the skin of the hands, lips or face, or inside the nose or mouth; (3) AVMs or telangiectasias in the internal organs including the lungs, brain, liver, intestines, stomach, and spinal cord; and (4) A family history (a first-degree relative) who meets criteria or has been genetically diagnosed. A patient has possible HHT if he or she meets two criteria, and a definite HHT diagnosis is made if the patient meets at least three. Patients with less than two criteria are considered unlikely to have HHT.
PHYSICAL EXAMINATION
Patients present with multiple telangiectasias along the septum, floor of the nose, and inferior and middle turbinate, which can be seen endoscopically (Fig. 6.1). Often, there will be significant crusting over any recent bleeding sites. Telangiectasias of the face, mouth, or hands are often visualized (Fig. 6.2). Telangiectasias are small erythematous spots that disappear when pressure is applied to them. In this evaluation, particular attention is directed toward the nose.
INDICATIONS FOR SEPTAL DERMATOPLASTY
Failure of conservative management for epistaxis
Laser ablation no longer effective
Anemia secondary to epistaxis
Significant downgrade in the quality of life due to severe recurrent epistaxis
CONTRAINDICATIONS
Medical comorbidities precluding surgery—HHT patients may have pulmonary and/or cerebral AVMs increasing their risks for stroke, brain abscess, and pulmonary sequelae.
Low or unstable hemoglobin levels should be stabilized with iron or blood transfusion prior to the procedure to ensure no adverse effects from blood loss during surgery.
PREOPERATIVE PLANNING
If there is a history of cerebral, pulmonary, and GI AVMs, the patient should have a complete HHT evaluation and appropriate medical evaluation prior to general anesthesia. This includes a full multidisciplinary evaluation that
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