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.

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.

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.

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.

Bilateral eyelid edema: cutis laxa or blepharochalasis?

A 59-year-old woman with massive bilateral edema of the upper and lower eyelids is presented. The edema occurred suddenly and without provocation. No cause could be identified despite a multitude of examinations. Initially the patient was diagnosed as having blepharochalasis, but later skin biopsy suggested an acquired form of cutis laxa. Most likely the cutis laxa was a result of angio-edema. Pharmacological treatment was not successful, but surgical intervention provided an acceptable result. The differential diagnosis of periorbital edema is discussed with special attention to cutis laxa, angio-edema, and blepharochalasis.

[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.

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.

Hereditary angioedema: uncomplicated maxillofacial surgery using short-term C1 inhibitor replacement therapy.

We report on the successful use of a pasteurized C1 inhibitor (C1-INH) concentrate during dental surgery of a patient affected by hereditary angioedema. The patient recovered fully without complications or side effects. Within 30 min, the first 1,000 U of C1-INH concentrate raised C1-INH concentration from 19 to 55% and function from 40 to 90% of the normal mean. When measured 4 h after the second injection, a further increase of the C1-INH concentration to 86% and a function of 106% relative to the normal mean was observed. Within 2 weeks C1-INH concentration returned to pretreatment level, while the function remained above this value. Serum liver enzyme values did not change and no anti-C1-INH alloantibodies were detected 10 months post-replacement therapy. We conclude that in patients affected by C1-INH deficiency, dental surgery and likely other traumatic procedures can be safely performed in conjunction with C1-INH replacement therapy even without preliminary treatment.

Blepharochalasis. A review of 30 cases.

This paper presents the findings in a series of 30 patients with blepharochalasis, including the age of onset, sex, predisposing factors, symptoms and signs, frequency and duration of attacks, and length of the history. There were 16 bilateral and 14 unilateral cases. The condition can be divided into an active (early) and a quiescent (late) stage. The active stage is further subdivided into intumescent (hypertrophic) and atrophic forms. The sequelae included excess thin skin, fat herniation, lacrimal gland prolapse, ptosis, blepharophimosis, pseudoepicanthic fold, proptosis, conjunctival injection and cysts, entropion, and ectorpion. Surgery primarily involved blepharoplasty, ptosis correction, and lateral canthal reattachment alone or in combination. The pathology showed a variable picture of epithelial atrophy, vasculitis, and loss of elastic fibers, which did not greatly help to differentiate blepharochalasis from angioedema, lymphedema, dermatochalasis, tumors and infiltrations, and floppy lid syndromes. Blepharochalasis is probably a localized angioedema. The diagnosis depends on the clinical features of intermittent attacks of localized swelling affecting one or more eyelids associated with thinning of the skin giving either an intumescent (hypertrophic) or atrophic appearance in the active stage of the condition and progressing to atrophic changes in the quiescent (late) stage.

Reduction cheiloplasty: its role in the correction of dentofacial deformities.

The presence of prominent lips is common in individuals with various dentofacial and craniofacial deformities. The aetiology must be diagnosed to differentiate between pseudo-macrochelia and true macrocheilia. When considering surgical correction of the prominent lip, a precise description of the location and the magnitude of the deformity is required. With careful treatment planning, proper sequencing of the treatment, and a good understanding of the various surgical techniques, optimum results can be achieved. These aspects will be discussed in detail illustrated with appropriate cases.