Abstract

Hemangioma of retropharyngeal space is a rare entity. It usually regresses spontaneously within nine years of age. However, a symptomatic tumor needs an urgent management. History, digital palpation, computed tomography (CT) and fine needle aspiration cytology (FNAC) clinches the diagnosis. We report a case of in a 1 year 11 month old female child presented with respiratory distress, diagnosed as retropharyngeal hemangioma and treated conservatively with sclerotherapy successfully.

Case Report

A 2-year-old female child from Jharkhand used to have frequent episodes of upper respiratory tract infection until last week of January 2011, when she got first attack of breathing difficulty during sleep. Her parents took her local hospital in Ranchi, where she was admitted and stayed for two days. X-ray and computed tomography (CT) scan of nasopharynx revealed a retropharyngeal space occupying lesion and referred to higher center. Subsequently, they came to Dept. of ENT and Head-Neck Surgery of our hospital. Her parents complained of sleepless nights for a fortnight because of the breathing difficulty, she felt each time she desires to get asleep. Only in prone position she gets some relief of the distress, but unknowingly during sleep her posture changes to supine and again she feels the distress.

On examination, her general condition was good, cry a bit hoarse and she was very much apprehensive. She was admitted and examined under general anesthesia; a cystic retropharyngeal mass was noted; on aspiration from the cystic mass 15 ml of hemorrhagic fluid came out. Cytologic examination of that fluid showed red blood cell (RBC) - 5.5 million/dl, white blood cell (WBC) - 6400/dl, Platelet - 1,50,000/dl and no pus cells. She was kept under observation for 5-6 days; she was relived off all symptoms and discharged on 7th postoperative day. After 40 days, she was readmitted in our department with the same complaints. This time 10 ml of fluid was aspirated and followed by intralesional injection of sclerosant sodium tetradecyl sulfate (2 ml of the sclerosant agent was diluted with 4 ml of  distilled water). She was discharged with no symptoms and asked to come for follow-up monthly. On 5th follow-up (after 4 months) we readmitted her, as she was mild symptomatic with breathing problem. Again examination under general anesthesia was done, at this time only 3 ml fluid came out on aspiration and 2 ml diluted sclerosant injected intralesionally. Followup CT scan after six months and one year was found normal. The patient is under regular follow-up and leading normal life.

Discussion

The retropharyngeal space lies between the buccopharyngeal fascia covering posterior pharyngeal wall anteriorly and cervical vertebra with pre-vertebral muscle covered by pre-vertebral fascia posteriorly. This space is divided into two compartments (right and left) by its attachment with the median raphe. Types of retro-pharyngeal space occupying lesion are: 

1) Congenital (brachial cleft cyst, ectopic thyroid); 2) inflammatory (retropharyngeal abscess and retropharyngeal cellulites); 3) neoplastic (cystic hygroma, neurofibroma, neuroblastoma, hemangioma); 

4) traumatic (foreign body, hematoma) and 5) metabolic (hypothyroidism). Of them the hemangioma is a rare cystic mass in this space.

A hemangioma (Latin: hemangio meaning blood vessel and oma meaning tumor) is a benign self-involuting tumor of endothelial cells. This tumor is most often found on the head or neck. However, they may occur anywhere on the skin or internal organs. In most cases hemangioma appears during the first days or weeks of life and resolve by own at the latest by age 10 years.

But some times hemangiomas can attain a mega size or even in small size can be symptomatic, thus necessities a management. Most approp riate treatment plan needs to be individualized for each patient and each lesion.^1,2 Sclerotherapy is a technique used for children and young adults with vascular or lymphatic malformations, and in adults to treat varicose veins and hemorrhoids. After injection within a vesicle it diffuses away from the site of injection, and vascular endothelium is irreversibly injured; the vessel gradually gets fully sclerosed and eventually replaced by a fibrous tissue. Sclerosants commonly used are polidocanol, 5% phenol, absolute alcohol, hypertonic saline and sodium tetradecyl sulfate.³

In our case, we unanimously thought that sclerotherapy would be the best treatment option because being vascular tumor/malformation hemangiomas can bleed severally, and control of bleeding in retropharyngeal space will be very difficult to manage. A simple solution to this complex situation is to sclerose these vescle. We used sodium tetradecyl sulfate as sclerosant because it is easily available and very economical.

Being a detergent-based chemical, its action is on the lipid molecules on the venous endothelium, causing destruction of the internal lining of the vein and causing them to shed, leading to thrombosis, fibrosis and obliteration (sclerosis). Until now the patient is relieved of the distress with this treatment (1 year follow up) and we expect no recurrence in future.

Conclusion

Hemangioma of retropharyngeal space is a rare entity. CT scan followed by digital palpation and fine needle aspiration cytology (FNAC) can clinch the diagnosis in case of retropharyngeal hemangioma presented with respiratory distress. Repeated aspiration of collected fluid and sclerothraphy in hemangioma may be a simple cost-effective and preferable treatment option.

References

1. Fishman SJ, Mullikin JB. Hemangiomas and vascular malformations of infancy and childhood. Pediatr Clin North Am 1993;40(6):1177-200.
2. McCook TA, Felman AH. Retropharyngeal masses in infants and young children. Am J Dis Child 1979;133(1):41-3.
3. Woods JE. Extended use of sodium tetradecyl sulfate in treatment of hemangiomas and other related conditions. Plast Reconstr Surg 1987;79(4):542-9.