Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature.

Case Presentation:

The patient is a 1-month-old, full-term, African American female, who presented with progressively worsening "noisy breathing" since birth. Her mother also noted elevation and protrusion of her tongue. Pregnancy and delivery were without complications. She had no difficulties feeding and was gaining weight. Past medical and surgical histories were unremarkable. She was not taking medications.Physical exam was significant for inspiratory stridor, tachypnea, and mild intercostal retractions. Facial features were symmetric. The nose was filled with copious secretions. The oral cavity demonstrated tongue protrusion and elevation to the level of the hard palate with a cystic structure filling the floor of mouth symmetrically. Flexible nasopharyngoscopy revealed copious secretions and marked edema of the arytenoids with significant epiglottic and arytenoid prolapse. The vocal cords were not visualized. No masses were present in the nasopharynx, hypopharynx, or larynx.The patient was admitted to the Pediatric Intensive Care Unit for observation. Oxygen saturation and vital signs remained stable. MRI of the head and neck was obtained. On the T1-weighted image, there was a hypointense, poor circumscribed signal within the anterior and middle tongue. On T2 and T1 with gadolinium image, this region became hyperintense. A swallowing study was obtained that showed severe gastroesophageal reflux to the level of the cervical esophagus with intermittent nasopharyngeal regurgitation. No laryngeal penetration or aspiraiton occurred. These findings were consistent with lymphatic malformation of the tongue complicated by severe gastroesophageal reflux disease and laryngomalacia.The patient underwent tracheostomy, gastrostomy tube placement, and fundoplication. Her course was complicated by a spontaneous, tension pneumothorax on the sixth postoperative day. Recovery was uneventful. She was discharged home on postoperative day 32 with tracheostomy collar at room air. Last follow-up was 1-15-99. The size of the mass was unchanged. She was breathing comfortably with a well-healed tracheostomy site.

Bibliography:

Apfelberg DB, Maser MR, Lash H, White DN. Benefits of the CO2 laser in oral hemangioma excision. Plast Reconstr Surg 1985;75:47-50.

Apfelberg DB, Maser MR, Lash H. Review of usage of argon and carbon dioxide lasers for pediatric hemangiomas. Ann Plast Surg 1984;12:353-360.

April MM, Rebeiz EE, Friedman EM, Healy GB, Shapshay SM. Laser therapy for lymphatic malformations of the upper aerodigestive tract. Arch Otolaryngol Head Neck Surg 1992;118:205-208.

Balakrishnan A, Bailey CM. Lymphangioma of the tongue. A review of pathogenesis, treatment and the use of surface laser photocoagulation. J Laryngol Otology 1991;105:924-930.

Batsakis JG. Tumors of the Head and Neck. Baltimore: Waverly Press; 1979.

Bhaskar SN. Oral lesions in infants and newborn. Dental Clin Norht Am 1996;2:421-435.

Biller HF, Krespi YP, Som PM. Combined therapy for vascular lesions of the head and neck with intra-arterial embolization and surgical excision. Otolaryngol Head Neck Surg 1982;90:37-47.

Brock ME, Smith RJH, Parey SE, Mobley DL. Lymphangioma. An otolaryngologic perspective. Int J Pediatr Otorhinolaryngol 1987;14:133-140.

Brown RL, Azizkhan RG. Pediatric head and neck lesions. Pediatr Clin North Am 1998;45:889-905.

Brown SH, Neerhout RC, Fonkalsrud EW. Prednisone therapy in the management of large hemangiomas in infants and children. Surgery 1972;71:168-173.

Bull TR. A Color Atlas of ENT Diagnosis, revised edition. London: Buttermarket; 1987.

Cohen SR, Wang C-I. Steroid treatment of hemangioma of the head and neck in children. Ann Otolaryngol 1972;81:584-590.

Crockett DM, McGill TJI. Benign lesions of the nose, oral cavity, and larynx in children: excision by carbon dioxide laser. Ann Otol Rhinol Laryngol 1985;94:489-493.

Dixon JA, Davis RK, Gilbertson JJ. Laser photocoagulation of vascular malformations of the tongue. Laryngoscope 1986;96:537-541.

Edgerton MT. The treatment of hemangiomas: with special reference to the role of steroid therapy. Am Surg 1976;183:517-532.

Ezekowitz RAB, Mulliken JB, Foikman J. Interferon Alfa-2a therapy for life-threatening hemangiomas of infancy. N Engl J Med 1992;326:1456-1463.

Filston HC. Hemangiomas, cystic hygromas, and teratomas of the head and neck. Sem Pediatr Surg 1994;29:147-159.

Godin DA, Guarisco JL. Cystic hygromas of the head and neck. J La State Med Soc 1997;149:224-228.

Grabb WC, Dingman RO, Oneal RM, Dempsey PD. Facial hamartomas in children: neurofibroma, lymphangioma, and hemangioma. Pediatr Res Surg 1980;66:509-527.

Guarisco JL. Congenital head and neck masses in infants and children. Ear Nose Throat J 1991;70:75-82.

Ikarashi T, Inamura K, Kimura Y. Cystic lymphangioma and plunging ranula treated by OK-432 therapy: a report of two cases. Acta Otolaryngol Suppl 1994;511:196-199.

Kasabach HH, Merritt KK. Capillary hemangioma with extensive purpura. Am J Dis Child 1940;59:1063-1067.

Kennedy MM, Kennedy R, Cramer B, Smith T. Sclerosing of recurrent lymphangioma using OK-432. J Pediatr Surg 1995;30:1159-1160.

Kennedy TL. Cystic hygroma-lymphangioma: a rare and still unclear entity. Laryngoscope 1989;99:1-10.

Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg 1982;69:412-420.

Ogita S, Tsuto T, Nakamura K, Deguchi E, Iwai N. OK-432 therapy in 64 patients with lymphangioma. J Pedatri Surg 1994;29:784-785.

Ogita S, Tsuto T, Nakamura K, Deguchi E, Tokiwa K, Iwai N. OK-432 therapy for lymphangioma in children: why and how does it work? J Pediatr Surg 1996;31:477-480.

Ohlms LA, Forsen J, Burrows PE. Venous malformation of the pediatric airway. Int J Pediatr Otorhinolaryngol 1996;37:99-114.

Parkin JL, Dixon JA. Argon laser treatment of head and neck vascular lesions. Otolaryngol Head Neck Surg 1985;93:211-216.

Pindborg JJ. Atlas of Diseases of the Oral Mucosa, 5th edition. Philadelphia: W.B. Saunders Company; 1993.

Raveh E, de Jong AL, Taylor GP, Forte V. Prognostic factors in the treatment of lymphatic malformations. Arch Otolaryngol Head Neck Surg 1997;123:1061-1065.

Sasaki GH, Pang CY, Wittliff JL. Pathogenesis and treatment of infant skin strawberry hemangiomas: clinical and in vitro studies of hormonal effects. Plast Reconstr Surg 1984;73:359-363.

Smith RJ, Burke DK, Sato Y, Poust RI, Kimura K, Bauman NM. OK-432 therapy for lymphangiomas. Arch Otolaryngol Head Neck Surg 1996;122:1195-1199.

Som PM, Bergeron T, editors. Head and Neck Imaging, 2nd edition. St. Louis: Mosby - Year Book; 1991.

Stal S, Hamilton S, Spira M. Hemangiomas, lymphangiomas, and vascular malformations of the head and neck. Otolaryngol Clin North Am 1986;769-796.

Warrell RP, Kempin SJ. Treatment of severe coagulopathy in the Kasabach-Merritt syndrome with aminocaproic acid and cryoprecipitate. Med Intelligence 1985;313:309-312.

Zarem HA, Edgerton MT. Induced resolution of cavernous hemangiomas following prednisolone therapy. Plast Reconstr Surg 1967;39:76-83.

Zhong PQ, Zhi FX, Li R, Xue JL, Shu GY. Long-term results of intratumorous bleomycin-A5 injection for head and neck lymphangioma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1998;86:139-144.

Click here to view slides from this presentation (pdf).

BCM Public | BCM Intranet | Privacy Notices | Contact BCM | BCM Site Map |

©2001-2005 Baylor College of Medicine
Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery
Mail: One Baylor Plaza, NA102, Houston, TX 77030
Phone: 713-798-5906
E-mail: oto@bcm.tmc.edu

Last modified: December 15, 2005