An unusual case of adult airway obstruction from a lymphovenous malformation.

Abstract

Lymphatic, venous, and mixed lymphovenous malformations are low-flow lesions that are present at birth and grow proportionately with the patient. We describe an unusual presentation of a lymphovenous malformation in an adult. A 19-year-old man presented to the emergency department with complaints of recent upper respiratory tract symptoms, increasing left-sided sore throat, voice change, odynophagia, dysphagia, and occasional subjective fevers and blood-tinged sputum. Examination revealed the presence of a left peritonsillar bulge consistent with a peritonsillar abscess; however, findings on needle aspiration were negative. The patient was admitted for intravenous steroid and antibiotic therapy. Within 24 hours, his airway became compromised, and he underwent an awake tracheotomy and biopsy, which showed a lymphovenous malformation. Magnetic resonance imaging the following day revealed a large, poorly circumscribed, heterogeneous left parapharyngeal mass consistent with a vascular malformation. With continuation of the steroids and antibiotics, the lesion regressed, and the patient was subsequently decannulated. At the 1-year follow-up, he exhibited no clinical symptoms, and he was in good health off steroids.

Introduction

Vascular anomalies are classified as malformations and hemangiomas, based on certain clinical and histologic characteristics. (1) Vascular malformations are subclassified as either capillary, venous, lymphatic, or arterial according to the vascular channel that predominates; some malformations involve a combination of vessels (e.g., lymphovenous and arteriovenous).

Only infrequently do lymphatic, venous, and mixed lymphovenous malformations make their first clinical presentation during adulthood. In this article, we describe the case of an adult who presented with a lymphovenous malformation that masqueraded as a peritonsillar abscess and caused airway obstruction.

Case report

A 19-year-old man presented to the emergency department at Madigan Army Medical Center in Tacoma, Wash., with a 3- to 4-day history of upper respiratory tract symptoms and occasional subjective fevers and blood-tinged sputum and a 1-day history of increasing left-sided sore throat, voice change, odynophagia, and dysphagia. Examination revealed the presence of a left peritonsillar bulge with purple discoloration of the anterior tonsillar pillar and asymmetric uvular elevation. We attempted needle aspiration of the left peritonsillar space, but it yielded no purulence. The patient was sent home on oral amoxicillin/clavulanate.

The following day, the patient returned to our clinic with mild progression of his symptoms. Flexible nasopharyngoscopy revealed a moderate medial bulging of the left lateral pharyngeal wall without airway compromise.

The peritonsillar bulging was more pronounced than it had been the previous day. Incision and drainage was attempted, but again, no purulence was found. The patient was admitted for intravenous steroids and antibiotics (ampicillin/sulbactam).

By the following morning, the patient had not improved, and he was sent for contrast- enhanced computed tomography (CT) of the neck. CT detected a 4 x 7 x 4-cm mass in the left pharyngeal/parapharyngeal space that extended from the nasopharynx to the hyoid bone (figure 1). The mass had displaced the vascular structures laterally and effaced much of the upper pharyngeal airway. Later in the day, the patient was taken to the operating room, where he underwent an awake tracheotomy for impending airway compromise and biopsy of the left peritonsillar region. Tissue cultures were negative. The biopsy specimen exhibited dilated vascular channels with flattened endothelium that were mostly blood-filled--findings that were consistent with a vascular malformation containing lymphatic and venous channels (figure 2). The fibrous stroma featured a considerable amount of edema, but it lacked smooth muscle. Focal areas of internal hemorrhage were also seen.

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The patient was then sent for magnetic resonance imaging (MRI). A T2-weighted image demonstrated a large, amorphous, heterogeneous, hyperintense mass that involved the left pharyngeal mucosa; the parapharyngeal, vascular, and retropharyngeal spaces; the deep substance of the parotid gland; and the base of the tongue (figure 3, A). MRI also showed significant effacement of the nasopharyngeal, oropharyngeal, and cervical tracheal airspace. Angiography the following day revealed tortuosity of the left internal maxillary and lingual arteries without identifiable feeding vessels.

We continued the patient's intravenous steroids and antibiotics, and he improved rapidly over the ensuing 3 days. He was then discharged on oral amoxicillin/ clavulanate (for a total 10-day course) and prednisone 60 mg/day, which was tapered over the following 2 months. On day 13 following the tracheotomy, he was decannulated.

Over the ensuing 2 months, the patient experienced a near-complete resolution of the lateral pharyngeal wall mass. A repeat MRI the following month showed a marked decrease in mass size and mass effect on the airway (figure 3, B). At follow-up 1 year after decannulation, the patient exhibited no clinical symptoms, and he was in good health off steroids.

Discussion

In 1982, Mulliken and Glowacki proposed subclassifying pediatric vascular anomalies as malformations and hemangiomas based on the endothelial characteristics of the anomalies. (1) This classification represents the accepted terminology in the medical literature today.

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Vascular malformations are usually present at birth, and they grow proportionately with the child. These lesions may contain any combination of capillary, lymphatic, venous, and arterial components, and they are classified according to the type of channel that predominates. Often, malformations are also classified as high-flow or low-flow lesions. Low-flow lesions include lymphatic, venous, and lymphovenous malformations. The endothelium of these malformations exhibits normal mitotic activity, a characteristic that distinguishes them histologically from hemangiomas, which exhibit increased mitotic activity of the endothelium during their proliferative phase. Vascular malformations are believed to arise as the result of errors in the morphogenesis of the vascular system between the 4th and 10th weeks of gestation. (1-3)

Venous malformations. Venous malformations--which are often referred to in the literature as cavernous hemangiomasor phlebangiomas--are soft, compressible, nonpulsatile, low-pressure, low-flow lesions that can be deep, superficial, or mixed. Most venous malformations are found in the head/neck and the extremities. Histologic features include dilated channels with scant smooth muscle, adventitial fibrosis, and phleboliths.

CT usually demonstrates heterogeneous or hypodense lesions and slow, if any, contrast uptake. MRI will show intermediate signal intensity on Tl-weighted imaging and increased signal intensity on T2-weighted imaging. Treatment options include compression, sclerotherapy, embolization, and resection. (1-3)

Lymphatic malformations. Lymphatic malformations--also known as cystic hygromas and cystic lymphangiomas--are believed to arise from either (1) sequestration of lymphatic sacs or channels that fail to communicate with peripheral channels or (2) localized stasis secondary to downstream blockage of lymphatic outflow. They are subclassified as microcystic, macrocystic, and mixed. They most commonly arise in the head and neck, and 80 to 90% are clinically apparent by age 2 years. (4) Many are associated with Turner syndrome, Noonan syndrome, and the trisomies. They often enlarge acutely secondary to trauma, internal hemorrhage, and/or infection in conjunction with upper respiratory infections. Airway compromise is often encountered in children.

CT will show a low-attenuation mass with enhancement of the wall with contrast, and MRI will show low signal intensity on T1-weighted imaging and high intensity on T2-weighted imaging. Acute enlargement is treated with systemic steroids, antibiotics against upper respiratory pathogens, and supportive measures. Although compression and Nd:YAG lasers have a role in management, surgical excision remains the mainstay of treatment. (1-4)

References

(1.) Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: A classification based on endothelial characteristics. Plast Reconstr Surg 1982;69(3):412-22.

(2.) Dubois J, Garel L. Imaging and therapeutic approach of hemangiomas and vascular malformations in the pediatric age group. Pediatr Radiol 1999;29(12):879-93.

(3.) Van Aalst JA, Bhuller A, Sadove AM. Pediatric vascular lesions. J Craniofac Surg 2003;14(4):566-83.

(4.) de Serres LM, Sie KC, Richardson MA. Lymphatic malformations of the head and neck. A proposal for staging. Arch Otolaryngol Head

Maj. Wayne J. Harsha, MD; Maj. James V. Crawford, MD; Lt. Col. Douglas M. Sorensen, MD, FACS 

From the Division of Otolaryngology, Department of Surgery, Madigan Army Medical Center, Tacoma, Wash.

Corresponding author: Wayne J. Harsha, MD, Department of Otorhinolaryngology, Mayo Clinic Arizona, 5777 E. Mayo Blvd., Phoenix, AZ 85054. Phone: [phone]; fax: [phone]; e-mail: harsha. [e-mail]

The information in this article was originally presented at the annual meeting of the American Academy of Otolaryngology-Head and Neck Surgery; Sept. 19-22, 2004; New York City.

The opinions and assertions in this article are those of the authors and should not be construed as representing the views of the Department of the Army or the Department of Defense. Neck Surg 1995;121(5):577-82.