CYSTIC HYGROMA CASE REPORT

Case Report                                                                                                                                                                                                                  

Cystic hygroma (CH) is a benign, congenital malformation of the lymphatic system with an incidence of 1:6,000. It usually affects the posterior triangle of the neck, with a predilection for the left side. Additional sites may include the forearm and anterior sternum. The majority of CHs are present at birth, with 90% becoming evident by the age of two years. Clinical presentation is as a soft, enlarging, painless mass, which may undergo spontaneous regression. Aetiological factors include environmental factors, maternal viral infection and maternal substance abuse. CH can also be associated with genetic abnormalities, most commonly Turners syndrome. Histopathology characteristically reveals multiple, interconnecting, epithelial-lined spaces. Skeletal hypertrophy is a common finding associated with cervicofacial CH, and the mandible is frequently affected, resulting in a high incidence of anterior open bite and class III malocclusion. Complications are frequent, and may be multiple, including airway compromise, infection, bleeding and dental caries.

Case Report

The case of a 13-year-old female, who presented with spontaneous dental pain affecting the maxillary left first and second permanent molar teeth, on a background of a large left-sided cervico-facial-thoracic CH is described.

Medical history The patient’s medical history was complex, with a history of subtotal CH resection with tracheostomy at 4 days old, followed by multiple debulking operations and a glossectomy over the following four years. The locally infiltrative nature of the lesion rendered complete excision difficult. The patient had undergone bilateral mandibular ostectomies and a genioplasty for the purpose of reducing mandibular size, and was gastrostomy fed until the age of 11 years. Following decannulation of her tracheostomy at age 12 years, attempts at intubation were unsuccessful, due to laryngeal infiltration by the CH. 

The patient had significant respiratory co-morbidities, with a history of recurrent respiratory tract infections, asthma and obstructive sleep apnoea. She reported recurrent otitis media (due to infiltration of the CH into the eustacian tube), in addition to well-controlled epilepsy and gastro-oesophageal reflux disease. She also suffered from Frey’s syndrome following multiple surgeries, and her left-sided facial nerve was non-functional.

Previous treatment of the CH included multiple debulking surgeries, intra-lesional sclerotherapy with bleomycin and OK432 and laser debulking of the lesion. The patient suffered from psychological issues, due to her appearance and had considerable difficulty in settling at school. She had a history of suicidal ideation, and attended the paediatric psychiatric services.

Dental history The patient had a history of regular dental examinations and intensive prevention, with dental restorations under local analgesia and dental extractions under general anaesthesia without any problems. She had, however,failed to attend multiple dental appointments over the previous two years. 

Clinical examination Examination revealed a CH involving the left face, neck, ear, tongue, larynx, oropharynx, neck and submandibular area. Recent imaging revealed that the lesion involved the mediastinum, circling the trachea and great vessels. The mandible was hypertrophic, with macroglossia and gross tongue protrusion, which prohibited mouth closing.

Mouth opening was limited Oral hygiene was extremely poor, with plaque accumulation, extensive decalcification and multiple carious permanent teeth. Teeth number 17 and 16 were restorable, but 26 and 27 were grossly carious with associated buccal swelling and the diagnosis was periapical periodontitis.

Treatment Initial management included the placement of obtundant dressings with resolution of dental pain. Intensive prevention was instigated, and teeth number 16 and 17 were restored with composite resin under local analgesia (LA) without incident. Extraction of teeth 26 and 27 was complicated by significant trismus, the extent of carious destruction of the crowns of the teeth, and the unacceptably high risk associated with general anaesthesia, due to intubation difficulties. Infiltration of CH into the patient’s oro-pharyngeal tissues prevented intubation. It proved impossible to achieve satisfactory analgesia using 2% lignocaine with 1:80,000 adrenaline. Due to her difficult airway, the decision was taken to treat the patient with inhalational sedation (IS), but administered in an operating theatre by a consultant anaesthetist. The patient was sedated using up to 60% nitrous oxide, and teeth were extracted using 4% articaine with 1:100,000 adrenaline LA. Access was difficult, but the teeth were elevated intact.

Follow-up The patient recovered well, and was discharged home on the same day. On recall after one week, the extraction sockets were found to be healing well. Subsequent to this, the patient was reviewed on a regular basis, with intensive prevention. Fluoride trays were fabricated for the patient (Fig 5) and worn for one-hour daily, alternating between one week using 1% chlorhexidine gel and one week using 2,800 ppm fluoride toothpaste in the trays. Two year follow-up showed improved oral health (Fig 6), with no new carious lesions detected. However, generalised decalcification was still evident, particularly affecting the anterior teeth.