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Lymphangioma: An unusual cause of pharyngeal and epiglottic edema
INTERNATIONAL CASE REPORTS EUGENE N. MYERS, MD International Editor
Lymphangioma: ,An unusual cause of pharyngeal and epiglottic edema BOAZ KARMAZYN, MD, TSILA HEFER,MD, HENRY ZVI JOACHIMS, MD, and DORITH GOLDSHER, MD, Halfa, Israel
A
swelling of the epiglottis and pharyngeal edema in an adult with sore throat and respiratory distress carries a large differential diagnosis that ranges from acute epiglottitis and pharyngitis to malignant infiltration of that anatomic structure. We present a rare case of a diffuse head and neck lymphangioma ir~volving the epiglottis and the pharynx, which was heralded by acute upper respiratory tract infection. The radiologic appearance and the methods of treatment of lymphangioma are also discussed. CASE REPORT A 32-year-old man was admitted to the emergency department with a 2-day history of sore throat, mild stridor, and
mild dyspnea. Previously he had not had significant problems with his respiration or throat. On physical examination, a massive edema of the submandibular area and the neck was noted. The tonsils were enlarged and bright red with a diffuse erythema of the pharynx. The uvula was edematous, and there was generalized swelling of the pharynx. The lungs were clear. Indirect laryngoscopy demonstrated a huge, thick epiglottis without erythema that obstructed the view of the vocal cords and the rima glottis. His temperature was 37.9 ° C, and his white blood count was 9600 with 89% potymorphonuclear neutrophils, 7% lymphocytes, 4% monocytes, and no band forms. A plain lateral radiograph of the neck (Fig. 1) revealed thickening of the epiglottis with the "thumb sign," increased aryepiglottic width, a narrowed tracheal air column, and a prevertebral soft tissue swelling. A C T scan of the neck revealed marked thickening of the fat tissue, both in the subcutaneous and deep areas along the
From the Departments of Radiology (Drs. Karmazyn ~nd Goldsber) and Otolaryngology-Head and Neck Surgery (Drs. Hefer and Joachims), Rambam Medical Center, Technion, Faculty of Medicine. Reprint requests: Tsila Hefer, MD, Department of Otolaryngology, RO. Box 3360, Haifa 31033, Israel. Otolaryngol Head Neck Surg 1997;117:409-1l. Copyright © 1997 by the American Academy of OtoJaryngologyHead and Neck SurgeryFoundation, Inc. 0194-5998/97/$5.00 + 0 23/78/83257
neck, face, and the occipital region. There were multiple septations along the fascia consistent with massive infiltration and fluid-filled compartments. There was also thickening of the intermuscalar fascia and thickening of the pharyngeal structures such as the uvula, epiglottis, and aryepiglottic folds, which were of low attenuation, compatible with edematous soft tissue. There is a marked thickening of the false vocal cords as well. No adenopathy was found. The muscle structures were of normal size and course (Fig. 2). The patient was admitted to the otolaryngology ward and was treated with antibiotics and Dexacort, On the following day a review of his medical records revealed that he had a diffuse, biopsy-proven tymphangioma involving the face and neck, which was cosmetically disfiguring and he had had since birth. His symptoms of sore throat and respiratory discomfort gradually subsided, but the huge amount of pharyngeal and epiglottic edema remained during 9 months of follow-up. DISCUSSION
Epiglottis enlargement, especially in adults, carries a large differential diagnosis. Acute epiglottitis is the most common cause of epiglottic enlargement in children and adults. It is an emergency situation with lethal potential. The signs and symptoms of patients with acute epiglottitis are sore throat, dysphagia, fever, drooling, stridor, hoarseness, and respiratory distress. Typically, acute epiglottitis is caused by Haemophilus influenza type B, but other bacteria and also viruses have been reported to cause acute epiglottitis. Causes of acute or chronic epiglottic enlargement other than acute epiglottitis include allergic reaction, angioneurotic edema, epiglottic hematoma, trauma, ingestion of caustic and other corrosive substances, inhalation of chemical substances, radiation, foreign body, retention cyst, primary tumors of the epiglottis or epiglottic infiltration by metastatic tumoral cells, and finally lymphangioma, all of which may cause the same symptoms mentioned above. Lymphangioma is a benign mass that arises from sequestration of lymphatic tissue. It is characterized by 409
410 KARMAZYNet al.
OtolaryngologyHead and Neck Surgery October 1997
Fig, 2. CT scan of the neck in the axial plane through the vocal cord showing a massive infiltration of the deep and superficial fatty tissue with small fluid compartments mainly along the fasciae, Note thickening of the retrolaryngeal soft tissue with low attenuation.
Fig. 1. Plain-film radiograph of the cervical region demonstrating a soft tissue thickening including the epiglottis and prevertebral space.
regional lymphatic dilatation. There are three histologic types: simple or capillary, cavernous, and cystic (cystic hygroma). All of these types might be present in the same specimen2-4; 50% to 65% of these lesions are present at birth, and 80% to 90% are present by the end of second year. 2 Lymphangioma may be localized, but it is often diffuse or multifocal. These are painless, soft, and at times poorly defined masses. The neck is most commonly involved, 2'3 but lymphangioma has also been reported in the retroperitoneum, axilla, mediastinum, bone, 2 and pancreas. 5 In 80% of patients the lesion in the neck appears as a mass in the posterior cervical triangle, 2 but it may occur anywhere from the mastoid process to the clavicle] In our case the extensive, diffuse lymphangioma was located in the pharynx, the epiglottis, and the submandibular area. Cervical lymphangioma rarely causes symptoms other than cosmetic deformity.6 Although nerves of the face and the neck or brachial plexus are often surrounded, rarely is there pain or motor weakness. 3 Large masses in the anterior neck may produce com-
pression of the airway, causing respiratory and/or deglutitory disturbance. This condition may be precipitated by a sudden enlargement because of acute upper respiratory tract infection or hemorrhage into the mass.2,3,6 Radiographic study may suggest the diagnosis. Lymphangiography may sometimes show communication of the mass with the lymphatic tree. However, a routine use of that technique is unnecessary today. CT scan is an important diagnostic modality for lymphangioma. The CT characteristic of these lesions is influenced by their vascularity and the presence of fat and fluid within the cystic spaces. A tentative diagnosis may be suggested because of a low attenuation number of the mass (-15 Hounsfield units), corresponding to fat within the lymphatic fluid. However, in many cases the CT shows attenuation values consistent with fluid density. Calcifications can occur rarely.5 The morphology, location, extent of the lymphangioma, and symptoms it produces dictate the method of treatment. Surgical excision is the primary therapeutic modality of lymphangioma, especially for cystic lymphangioma (cystic hygroma). 24 Although complete excision should always be the surgical goal, it is not always possible. This mass is sometimes poorly defined and generalized, involving a number of important anatomic structures. In that case a more conservative surgical approach is taken, ~,3 resulting in a recurrence
OtolaryngologyHead and Neck Surgery Volume 117 Number 4
rate of 15% or more. 2,4 In general, successful excision is more likely when lymphangioma consists primarily of cystic elements rather than capillary lymphangiomatous components. 3 Highly extensive, poc,rly defined, diffuse lymphangioma involving a number of anatomic structures, as was the case in our patien',;, cannot be treated surgically. Radiotherapy and injection of sclerosing agents have been used unsuccessfully and therefore are not recommended. 3,4 If l y m p h a n g i o m a causes severe upper airway obstruction with respiratory distress, t r a c h e o t o m y should be performed for airway maintenance. 3 The case presented here is a rare presentation of an extensive diffuse lymphangioma involving numerous anatomic structures, making excision impossible. In addition, after resolution of the acute upper respiratory tract infection, the stridor and dysphagia ceased completely. Therefore no additional therapy was needed.
CONCLUSIONS Lymphangioma o f the pharynx and epiglottis is extremely rare. It may present as a localized or diffuse swelling of those anatomic structures causing dyspha-
KARMAZYN et aL 411
gia and respiratory distress, and in our patient it mimicked acute epiglottitis. The CT scan is an important tool to delineate the configuration of the lesion and its exact location. Lymphangioma, although rare, should be considered in the differential diagnosis of pharyngeal and epiglottic enlargement, and CT scan of the head and the neck is recommended in such cases.
REFERENCES 1. Harel My Eviatar E, Flaxman H, Segal S. Acute epiglottitis in adults. Haxefuah 1990;119:128-31. 2. Goodman R Yeung CST, Batsalds JG. Retropharyngeal lymphangioma presenting in an adult. Otolaryngol Head and Neck Surg 1990;103:476-9. 3. Hirshowitz B, Eliachar I. Treatment of lymphangioma and cystic hygroma. In: Goldwyn RM, editor. Long-term results in plastic and reconstructive surgery. Boston: Little, Brown and Co.; 1980. p. 195-216. 4. Karmady CS, Fortson JK, Calcaterra VE. Lymphangiomasof the head and neck in adults. Otolaryngol Head and Neck Surg 1982;90:283-8. 5. Salimi Z, Fishbein M, Wolverson MK, Johnson FE. Pancreatic lymphangioma: CT, MRI, and angiograph~c features. Gastrointest Radiol 1991;16:248-50. 6. Pincus RL. Congenital neck masses and cysts. In: Bailey BJ, editor. Head & Neck Surgery-Ototaryngology.Voh 1, Philadelphia: JB Lippincott; 1993. p. 756.
Lymphangioma: ,An unusual cause of pharyngeal and epiglottic edema BOAZ KARMAZYN, MD, TSILA HEFER,MD, HENRY ZVI JOACHIMS, MD, and DORITH GOLDSHER, MD, Halfa, Israel
A
swelling of the epiglottis and pharyngeal edema in an adult with sore throat and respiratory distress carries a large differential diagnosis that ranges from acute epiglottitis and pharyngitis to malignant infiltration of that anatomic structure. We present a rare case of a diffuse head and neck lymphangioma ir~volving the epiglottis and the pharynx, which was heralded by acute upper respiratory tract infection. The radiologic appearance and the methods of treatment of lymphangioma are also discussed. CASE REPORT A 32-year-old man was admitted to the emergency department with a 2-day history of sore throat, mild stridor, and
mild dyspnea. Previously he had not had significant problems with his respiration or throat. On physical examination, a massive edema of the submandibular area and the neck was noted. The tonsils were enlarged and bright red with a diffuse erythema of the pharynx. The uvula was edematous, and there was generalized swelling of the pharynx. The lungs were clear. Indirect laryngoscopy demonstrated a huge, thick epiglottis without erythema that obstructed the view of the vocal cords and the rima glottis. His temperature was 37.9 ° C, and his white blood count was 9600 with 89% potymorphonuclear neutrophils, 7% lymphocytes, 4% monocytes, and no band forms. A plain lateral radiograph of the neck (Fig. 1) revealed thickening of the epiglottis with the "thumb sign," increased aryepiglottic width, a narrowed tracheal air column, and a prevertebral soft tissue swelling. A C T scan of the neck revealed marked thickening of the fat tissue, both in the subcutaneous and deep areas along the
From the Departments of Radiology (Drs. Karmazyn ~nd Goldsber) and Otolaryngology-Head and Neck Surgery (Drs. Hefer and Joachims), Rambam Medical Center, Technion, Faculty of Medicine. Reprint requests: Tsila Hefer, MD, Department of Otolaryngology, RO. Box 3360, Haifa 31033, Israel. Otolaryngol Head Neck Surg 1997;117:409-1l. Copyright © 1997 by the American Academy of OtoJaryngologyHead and Neck SurgeryFoundation, Inc. 0194-5998/97/$5.00 + 0 23/78/83257
neck, face, and the occipital region. There were multiple septations along the fascia consistent with massive infiltration and fluid-filled compartments. There was also thickening of the intermuscalar fascia and thickening of the pharyngeal structures such as the uvula, epiglottis, and aryepiglottic folds, which were of low attenuation, compatible with edematous soft tissue. There is a marked thickening of the false vocal cords as well. No adenopathy was found. The muscle structures were of normal size and course (Fig. 2). The patient was admitted to the otolaryngology ward and was treated with antibiotics and Dexacort, On the following day a review of his medical records revealed that he had a diffuse, biopsy-proven tymphangioma involving the face and neck, which was cosmetically disfiguring and he had had since birth. His symptoms of sore throat and respiratory discomfort gradually subsided, but the huge amount of pharyngeal and epiglottic edema remained during 9 months of follow-up. DISCUSSION
Epiglottis enlargement, especially in adults, carries a large differential diagnosis. Acute epiglottitis is the most common cause of epiglottic enlargement in children and adults. It is an emergency situation with lethal potential. The signs and symptoms of patients with acute epiglottitis are sore throat, dysphagia, fever, drooling, stridor, hoarseness, and respiratory distress. Typically, acute epiglottitis is caused by Haemophilus influenza type B, but other bacteria and also viruses have been reported to cause acute epiglottitis. Causes of acute or chronic epiglottic enlargement other than acute epiglottitis include allergic reaction, angioneurotic edema, epiglottic hematoma, trauma, ingestion of caustic and other corrosive substances, inhalation of chemical substances, radiation, foreign body, retention cyst, primary tumors of the epiglottis or epiglottic infiltration by metastatic tumoral cells, and finally lymphangioma, all of which may cause the same symptoms mentioned above. Lymphangioma is a benign mass that arises from sequestration of lymphatic tissue. It is characterized by 409
410 KARMAZYNet al.
OtolaryngologyHead and Neck Surgery October 1997
Fig, 2. CT scan of the neck in the axial plane through the vocal cord showing a massive infiltration of the deep and superficial fatty tissue with small fluid compartments mainly along the fasciae, Note thickening of the retrolaryngeal soft tissue with low attenuation.
Fig. 1. Plain-film radiograph of the cervical region demonstrating a soft tissue thickening including the epiglottis and prevertebral space.
regional lymphatic dilatation. There are three histologic types: simple or capillary, cavernous, and cystic (cystic hygroma). All of these types might be present in the same specimen2-4; 50% to 65% of these lesions are present at birth, and 80% to 90% are present by the end of second year. 2 Lymphangioma may be localized, but it is often diffuse or multifocal. These are painless, soft, and at times poorly defined masses. The neck is most commonly involved, 2'3 but lymphangioma has also been reported in the retroperitoneum, axilla, mediastinum, bone, 2 and pancreas. 5 In 80% of patients the lesion in the neck appears as a mass in the posterior cervical triangle, 2 but it may occur anywhere from the mastoid process to the clavicle] In our case the extensive, diffuse lymphangioma was located in the pharynx, the epiglottis, and the submandibular area. Cervical lymphangioma rarely causes symptoms other than cosmetic deformity.6 Although nerves of the face and the neck or brachial plexus are often surrounded, rarely is there pain or motor weakness. 3 Large masses in the anterior neck may produce com-
pression of the airway, causing respiratory and/or deglutitory disturbance. This condition may be precipitated by a sudden enlargement because of acute upper respiratory tract infection or hemorrhage into the mass.2,3,6 Radiographic study may suggest the diagnosis. Lymphangiography may sometimes show communication of the mass with the lymphatic tree. However, a routine use of that technique is unnecessary today. CT scan is an important diagnostic modality for lymphangioma. The CT characteristic of these lesions is influenced by their vascularity and the presence of fat and fluid within the cystic spaces. A tentative diagnosis may be suggested because of a low attenuation number of the mass (-15 Hounsfield units), corresponding to fat within the lymphatic fluid. However, in many cases the CT shows attenuation values consistent with fluid density. Calcifications can occur rarely.5 The morphology, location, extent of the lymphangioma, and symptoms it produces dictate the method of treatment. Surgical excision is the primary therapeutic modality of lymphangioma, especially for cystic lymphangioma (cystic hygroma). 24 Although complete excision should always be the surgical goal, it is not always possible. This mass is sometimes poorly defined and generalized, involving a number of important anatomic structures. In that case a more conservative surgical approach is taken, ~,3 resulting in a recurrence
OtolaryngologyHead and Neck Surgery Volume 117 Number 4
rate of 15% or more. 2,4 In general, successful excision is more likely when lymphangioma consists primarily of cystic elements rather than capillary lymphangiomatous components. 3 Highly extensive, poc,rly defined, diffuse lymphangioma involving a number of anatomic structures, as was the case in our patien',;, cannot be treated surgically. Radiotherapy and injection of sclerosing agents have been used unsuccessfully and therefore are not recommended. 3,4 If l y m p h a n g i o m a causes severe upper airway obstruction with respiratory distress, t r a c h e o t o m y should be performed for airway maintenance. 3 The case presented here is a rare presentation of an extensive diffuse lymphangioma involving numerous anatomic structures, making excision impossible. In addition, after resolution of the acute upper respiratory tract infection, the stridor and dysphagia ceased completely. Therefore no additional therapy was needed.
CONCLUSIONS Lymphangioma o f the pharynx and epiglottis is extremely rare. It may present as a localized or diffuse swelling of those anatomic structures causing dyspha-
KARMAZYN et aL 411
gia and respiratory distress, and in our patient it mimicked acute epiglottitis. The CT scan is an important tool to delineate the configuration of the lesion and its exact location. Lymphangioma, although rare, should be considered in the differential diagnosis of pharyngeal and epiglottic enlargement, and CT scan of the head and the neck is recommended in such cases.
REFERENCES 1. Harel My Eviatar E, Flaxman H, Segal S. Acute epiglottitis in adults. Haxefuah 1990;119:128-31. 2. Goodman R Yeung CST, Batsalds JG. Retropharyngeal lymphangioma presenting in an adult. Otolaryngol Head and Neck Surg 1990;103:476-9. 3. Hirshowitz B, Eliachar I. Treatment of lymphangioma and cystic hygroma. In: Goldwyn RM, editor. Long-term results in plastic and reconstructive surgery. Boston: Little, Brown and Co.; 1980. p. 195-216. 4. Karmady CS, Fortson JK, Calcaterra VE. Lymphangiomasof the head and neck in adults. Otolaryngol Head and Neck Surg 1982;90:283-8. 5. Salimi Z, Fishbein M, Wolverson MK, Johnson FE. Pancreatic lymphangioma: CT, MRI, and angiograph~c features. Gastrointest Radiol 1991;16:248-50. 6. Pincus RL. Congenital neck masses and cysts. In: Bailey BJ, editor. Head & Neck Surgery-Ototaryngology.Voh 1, Philadelphia: JB Lippincott; 1993. p. 756.