Taming the Tongue: The Surgical Approach to Macroglossia.

While traditionally in the realm of otorhinolaryngology or oral maxillofacial surgery, conditions involving the tongue may also be managed by plastic surgeons. The authors present an unusual case of acquired macroglossia resulting from angiotensin-converting enzyme inhibitor-induced angioedema and review the literature to discuss its surgical management from a plastic surgery perspective. A 62-year-old female suffered severe airway obstruction, respiratory arrest, and anoxic brain injury from angioedema-associated macroglossia. After tracheostomy, the patient was managed nonsurgically, with bite wound care and medications to minimize angioedema to marginal effect. Ultimately, a partial glossectomy was planned. The edematous distal tongue was amputated and closed primarily. On postoperative day 2, she was successfully weaned off mechanical ventilation and no longer suffers trauma from tongue biting. The simple anterior tongue resection described in this paper was an appropriate approach for our patient. More research is needed to guide plastic surgeons in an optimal approach for clinical scenarios.

A cross-sectional questionnaire assessing patient and physician use of short-term prophylaxis for hereditary angioedema.

BACKGROUND: Current guidelines recommend short-term prophylaxis (STP) before invasive procedures to prevent hereditary angioedema (HAE) attacks; however, adherence to these guidelines may be variable because this indication lacks Food and Drug Administration approval in the United States. OBJECTIVE: To ascertain the STP experiences of patients with HAE and HAE-treating physicians. METHODS: Online questionnaires focusing on STP experiences were distributed by the US Hereditary Angioedema Association to the first 250 patients with HAE and to registered HAE-treating physicians. SAS 9.3 was used to perform descriptive statistics and to test the difference between patients who underwent procedures and those who did not using Pearson χ(2) test, Fisher exact test, and 2-sample t test. RESULTS: For the patient survey, 219 respondents met the criteria for HAE type 1 and 2; 37 (17%) underwent 66 invasive procedures, and all reported receiving STP. Eight patients (22%) reported failed STP, but only 3 required on-demand therapy. For STP, anabolic steroids and plasma-derived C1 inhibitor were the most and second-most commonly used, respectively. For the physician survey, 37 physicians reported caring for 433 patients with HAE. Depending on the procedure, 19% to 54% of physicians used STP and 30% to 86% prescribed on-demand therapy; 69% and 78% of physicians prescribed plasma-derived C1 inhibitor as STP for minimally invasive and invasive procedures, respectively. Physicians reported excellent efficacy for the STP treatments used. CONCLUSION: Physicians reported excellent outcomes using primarily newer STP therapies, namely plasma-derived C1 inhibitor, which was discordant to patient-reported outcomes using older STP therapies, namely anabolic steroids. Well-controlled STP studies are needed to clarify use for patients with HAE in the United States.

Adverse drug effects and angioedema hospitalizations in the United States from 2000 to 2009.

Since angiotensin-converting enzyme (ACE) inhibitors became common treatments, there have been increasing reports of angioedema (AE). AE hospitalization (AEH) trend data in the new millennium are limited. This study calculates hospitalization rates for AEs and describes clinical characteristics of AEHs in the United States, especially as related to specific adverse drug effects (ADEs). The National Inpatient Samples 2000-2009 were queried for AEHs to calculate hospitalization rates and to examine for associations with specified ADEs, certain comorbidities, and demographic features. AEHs requiring intubation or tracheostomy were also examined for associations. There was a significant increase in the AEH rates (3.4 per 10(5) to 5.4 per 10(5)) over the study period (p < 0.0001) and the hospitalization rates for African Americans (AAs) were consistently higher. Throughout the study the proportions of AEH coding any ADEs, or an ADE due to a cardiovascular (CV) or antihypertensive (aHTN) drug increased over time. By 2009, 61.7% AEHs coded an ADE. Of these, 58.7% were caused by CV or aHTN drugs. In AEHs, having an ADE from a CV or aHTN medication had the strongest adjusted associations with hypertension and renal disease as well as with alcohol-related disorders. In AEHs, intubation/tracheostomy had the strongest ADE associations related to CV or aHTN medication (adjusted odds ratio, 1.4; 95% CI, 1.3, 1.6). AEHs continue to increase, but this can only be partially attributed to ACE inhibitor usage. Intubation/tracheostomy appears to be greater in AEHs with ADE due to CV/aHTN drugs. Alcohol-related disorders may have a role in ACE inhibitor-associated AEH.

Angiotensin-converting enzyme inhibitor-induced small-bowel angioedema: clinical and imaging findings in 20 patients.

OBJECTIVE: The purpose of this article is to retrospectively review the radiologic and clinical findings in patients with angiotensin-converting enzyme inhibitor (ACEI)-induced small-bowel angioedema, with an emphasis on CT findings. MATERIALS AND METHODS: Imaging findings, with an emphasis on CT, and clinical characteristics of 20 patients (23 presentations) presenting to two institutions' emergency departments from 1996 through 2010 with ACEI-induced small-bowel angioedema were retrospectively reviewed by two abdominal radiologists who were aware of the diagnosis. Examinations were reviewed in consensus to determine common radiographic findings. RESULTS: Patient age range was 23-83 years (mean, 56 years). Sixteen of the 20 patients were women, and 15 of 20 were obese. All had acute onset of severe abdominal pain. The date of the initial episode prompting CT evaluation ranged from 2 days to 10 years after the start of ACEI therapy (average, 3.3 years). All patients underwent abdominal CT examinations while symptomatic; five patients also underwent a small-bowel series. Three patients underwent urgent surgery for presumed small-bowel ischemia. All patients had resolution of symptoms within 4 days of hospitalization. CT findings included ascites in all patients, small-bowel wall thickening (mean, 1.3 cm), mild dilatation (mean, 2.9 cm), and straightening. There was no small-bowel obstruction. CONCLUSION: ACEI-induced small-bowel angioedema should be included in the differential diagnosis when patients receiving ACEI therapy present with abdominal complaints and the following combination of findings on CT examination: ascites, small-bowel wall thickening, dilatation without obstruction, and straightening.

[Anesthesia and perioperative management in a patient with acquired angioedema].

We experienced anesthesia and perioperative management for hysterectomy in a patient with acquired angioedema. Angioedema due to C1 esterase inhibitor (C1-INH) deficiency (loss or dysfunction of C1-INH) is one of the rarest diseases, and is characterized by recurrent episodes of regional hard edema and ascites induced by mechanical stimuli or mental stress. Edema spreads to the subcutaneous and submucosal layer, and laryngeal edema may cause the upper airway obstruction. Tranexamic acid and C1-INH concentrates were administered perioperatively for prophylaxis of attacks, and combined spinal and epidural anesthesia was performed for hysterectomy. We could manage perioperative care without causing edema.

Small bowel intussusception: an unusual presentation of angioedema.

This article presents a case report of a 13-year-old boy who presented to the Emergency Department with abdominal pain and vomiting. He had a known history of recurrent swelling but no previous abdominal episodes. A computed tomography scan revealed small bowel intussusception and he was scheduled for surgery. The patient had a history of multiple episodes of swelling of extremities, face, and genitalia. The Allergy Consult Service was consulted for perioperative management of his angioedema.

ACE inhibitor-induced angioedema in elderly African American females requiring tracheostomy.

Angioedema is a well-demarcated localized edema involving the deeper layers of the skin, subcutaneous tissue, mucosa, and submucosa. It is a well-recognized side effect of angiotensin-converting enzyme (ACE) inhibitor therapy. Prolonged angioedema is rare. In most cases, the angioedema is self-limited and responds to cessation of ACE inhibitor therapy. ACE inhibitor-related angioedema occurs more commonly in African American patients and tends to be particularly severe in elderly African American women. We report 2 cases of extremely severe angioedema of prolonged duration that required tracheostomy to stabilize the airway.

The blepharochalasis syndrome.

Blepharochalasis is a rare eyelid disorder that often presents in childhood or early adolescence. It is characterized by exacerbations and remissions of painless edema of the upper and occasionally lower eyelids. Although the average duration of attack is only two days, multiple attacks eventually lead to atrophic, wrinkled, and discolored periorbital skin. Other clinical manifestations include ptosis, acquired forms of blepharophimosis, lower lid retraction, pseudoepicanthal folds, proptosis, prolapse of orbital fat, and lacrimal tissue. The etiology of blepharochalasis has yet to be fully elucidated, but histpathologic examinations indicate that elastolytic activity, immunoglobulin A (IgA), and other inflammatory processes might play a substantial role in the pathogenesis of the disease. The treatment of blepharchalasis is primarily surgical, and therefore understanding the natural history is essential to avoid overcorrection and recurrences after surgery. In this review we present the clinical characteristics, differential diagnosis, and treatment options of blepharochalsis.

Hashimoto's thyroiditis associated with urticaria and angio-oedema: disappearance of cutaneous and mucosal manifestations after thyroidectomy.

A 60 year old woman affected by Hashimoto's thyroiditis presented with a history of recurring episodes of urticaria and angio-oedema. Clinical and laboratory evaluation of the patient excluded allergy to external agents, hereditary angio-oedema, and occult infections. A pathogenic relation between Hashimoto's thyroiditis and chronic urticaria/angio-oedema was suspected. However, treatment with L-thyroxine had no influence on the frequency and severity of the cutaneous and mucosal manifestations, which occurred almost daily and required repeated administration of steroids. The patient therefore underwent total thyroidectomy. Cytometric analysis of intrathyroidal lymphocyte subsets showed unusual abnormalities. Urticaria and angio-oedema completely remitted after surgery; 18 months postoperatively the patient was still asymptomatic.

Blepharochalasis syndrome: a proposed pathophysiologic mechanism.

The blepharochalasis syndrome is an uncommon disorder seen in young people that is characterized by recurrent episodes of eyelid edema. After repeated episodes various eyelid supportive structures are stretched, giving rise to the characteristic features of thin excess lid skin, blepharoptosis, pseudoepicanthal folds, and disinserted lateral canthal tendons. Surgical repositioning and reconstruction are often required. The author describes 10 patients with this disorder and suggests a relation to angioedema, a common dermatologic condition with numerous triggering mechanisms.

Angioedema secondary to angiotensin-converting enzyme inhibitors.

Angioedema secondary to angiotensin-converting enzyme (ACE) inhibitors is rare, but it is a side effect which is likely to be seen more frequently because of the increased use of these drugs to treat cardiac failure and hypertension. Presentation is variable and the diagnosis may go unrecognized for many months or years. The cases reported illustrate problems both in the diagnosis and management of this life-threatening condition.

[Simultaneous occurrence of hereditary angioneurotic edema and Crohn disease]. / Hereditaer angioneuroticus oedema és Crohn-betegség együttes elöfordulása.

Hereditary C1 esterase inhibitor deficiency is often associated with immunpathologic disorders. The authors present a case of the rare coincidence of hereditary angioedema (HAE) and Crohn's disease. The history of the patient is analysed along with the familial occurrence of the disease. Characteristic abdominal manifestations of C1 esterase inhibitor deficiency are compared to the clinical signs of Crohn's disease. Differential diagnostic pitfalls are described along with efficatious therapeutic options.

[Hereditary angioneurotic edema (Quincke's edema). Report of a case and literature review]. / Edema angioneurótico familiar (edema de Quincke). Presentación de 1 caso y revisión de la literatura.

Familiar angioneurotic edema or Quincke's edema is an uncommon variant of urticaria, associated or not, that involves the subcutanean cell tissue and mucous of the face and upper airdigestive tract. It can produce an acute dyspnea and risk of suffocation with intubation or tracheostomy up to a 20% of the cases. The disease is a result of deficit in C esterasa inhibitor which autosomal dominant inheritance. We are reporting one clinical case confirmed as such corresponding to a 81 years-old male whom was operated by tracheotomy after sending from UCI with an emergency coniotomy and failure for orotracheal intubation.

[Is tracheotomy of angioedema associated with ACE-inhibitor therapy?]. / Tracheotomie bei Angioödem unter ACE-Hemmer-Therapie?

Angioedema of the upper airways associated with angiotensin-converting enzyme inhibitors (ACEI) represent a rare but serious problem. We present two cases from our hospital in order to illustrate the therapeutic consequences resulting from angioedema associated with the intake of ACEIs. Surgical airway management should be considered early if medical treatment fails.