Abstract
Vallecular cysts are infrequent causes of supraglottic obstruction causing stridor and swallowing difficulty in infants. When detected early in life, the management consists of marsupialization or resection. Supraglottic lymphangiomas of the tongue base and vallecula present with similar symptoms and time of presentation. Endoscopic visualization is traditionally considered to be sufficient in identifying and differentiating these. When a vallecular cyst is visually diagnosed by the surgeon during endoscopy, surgical treatment is provided at the same time. Obtaining a specimen is rarely considered for histopathologic diagnostic verification. However, the natural presentation of a cystic lymphangioma may be indistinguishable from a solitary vallecular cyst by endoscopy alone. This case presentation argues in favor of histopathologic diagnosis in vallecular cysts because the 2 may represent a continuum of disease. A vallecular mass with a single large mucus-filled cyst and adjoining edematous soft tissue extension into the tongue base and piriform sinus diagnosed as lymphangioma through D2-40 immunoreactivity is presented.
1
Introduction
Vallecular cysts and supraglottic lymhangiomas are considered distinct entities. The former is defined by its location and appearance. A cystic lymphangioma may occur in the vallecula and will be given the same diagnosis if the cystic component is predominant and the lymphangioma is not very extensive. The clinical presentation will not aid in the differential, and the usual workup does not include imaging studies such as a magnetic resonance imaging (MRI) in the absence of extenuating circumstances. Many times the clinical finding of stridor prompts an airway evaluation, and flexible endoscopy is the preferred diagnostic tool. However, flexible endoscopy can be inconclusive or fail to identify the pathology , and for some children with compromised airway, it may not be safe enough to perform in the office. For diagnosis, treatment, and a better controlled airway, the next step is direct laryngoscopy and bronchoscopy under general anesthesia. The diagnosis of the solitary vallecular cyst is visual, and a variety of surgical treatment options are readily available including the use of laser(s), marsupialization, and dissection techniques . Recurrence rates are variable with different surgical techniques. Better control of recurrences are reported with marsupialization and the use of laser(s) . The recurrences may be due to surgical technique used, accessibility, and presumably to multifocal or multilocular presentation initially obscured. Multifocal or multilocular presentation is very common with cystic lymphangiomas due to residual disease. With lymphangiomas, staged removals and revisions are expected because recurrences are common and local control may be difficult . For accurate diagnosis and prognostic evaluation, it is of utmost importance to differentiate between a solitary vallecular cyst and a cystic lymphangioma. Endoscopic visualization and imaging studies including ultrasound, plain radiographs, MRI and computed tomographic scans may not accurately provide this information when a lymphangioma is obscured by its predominant cystic component, thus presenting as a solitary vallecular cyst. Histopathology is required but may need to use additional diagnostic markers for the differential diagnosis. Monoclonal antibody D2-40 is a new marker of lymphatic endothelium and can provide the required detail. D2-40 is a highly specific marker of variable sensitivity for lymphatic endothelium in normal tissue and a subset of vascular lesions, including lymphangiomas, and is valuable for studying benign and malignant vascular disorders in routinely processed tissue specimens. Previous histopathologic classifications may have been inadequate in identifying lymphangiomas, and it is plausible that reported recurrences of vallecular cysts are in actuality undiagnosed lymphangiomas.
2
Case report
A 15-month-old boy was evaluated for intermittent noisy breathing that had been present for the past 5 months. He had been treated with various inhalers, antibiotics, and steroids for presumed bronchiolitis without effect. An Ear, Nose, Throat (ENT) physician performed flexible endoscopy and observed epiglottic swelling, which was attributed to laryngopharyngeal reflux. Referral to pediatric ENT was made when antireflux treatment failed to make a difference. He has a strong cry, and his noisy breathing was absent on the day of evaluation by the pediatric ENT specialist. Chest x-rays were normal. No apneas, cyanosis, or life-threatening events are reported. No developmental delay is recorded. Choking and difficulty swallowing are not observed. Endoscopic examination in the operating room was scheduled.
Mask ventilation proved to be very difficult, and the airway was lost at the early phase of the induction. Intubation attempts were unsuccessful. A size 3.5-mm rigid bronchoscope was used to establish the airway. During rigid endoscopy, a large cystic mass of the vallecula and tongue-base was discovered. The mass was displacing the epiglottis posteriorly and caused considerable difficulty in visualizing the airway. Once the airway was established with the bronchoscope, subsequent intubation was facilitated by the use of a 0° Hopkins telescope inserted through a 4.0 endotracheal tube for guidance. Suspension microlaryngoscopy using a Lindholm laryngoscope followed. The large vallecular cyst was about 3 cm in size in its largest diameter and typical in appearance and location, distorting the epiglottic position and obstructing the view to the larynx to such extent, that only the posterior surface of the arytenoids was visible under suspension ( Fig. 1 ; with endotracheal tube in place). The cyst had a preference toward the left of the midline and followed with edematous, irregular, extensions toward the piriform sinus. Microscissors and a cup forceps were used to obtain a biopsy of the cyst wall. This caused thick mucus to pour out of the cyst as was expected ( Fig. 2 ; with suction in place and partial laser coagulation). NdYAG laser at low power was used to remove and coagulate the external visible walls of the cyst completely and, moreover, to reduce the irregular edematous tissues toward the left piriform sinus. To address the associated laryngomalacia, the aryepiglottic folds were trimmed with microscissors. This intervention moved the epiglottis in a favorable anterior position now allowing full visualization of the vocal cords including the anterior commissure. Patient was kept intubated overnight in the pediatric intensive care unit and was extubated the next morning. He remained asymptomatic since. Pathology report was positive for lymphangioma supported by immunoreactivity with D2-40 monoclonal antibody. An MRI with contrast was obtained 8 weeks after laser removal and showed no evidence of lesion or of any extension into surrounding soft tissue. Flexible endoscopy 9 weeks after laser removal showed normal anatomy with no evidence of recurrence.
