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Case report: cystic hygroma.

Background

Cystic hygroma (CH) is a benign, congenital malformation of the lymphatic system with an incidence of 1:6,000 [Gedikbasi et al., 2007]. 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 [Mirza et al., 2011] The majority of CHs are present at birth, with 90% becoming evident by the age of two years [Rautio et al., 2003]. Clinical presentation is as a soft, enlarging, painless mass, which may undergo spontaneous regression [Velez and Mintz, 2006]. Aetiological factors include environmental factors, maternal viral infection and maternal substance abuse. CH can also be associated with genetic abnormalities, most commonly Turners syndrome [Mosca et al, 2008]. Histopathology characteristically reveals multiple, interconnecting, epithelial-lined spaces [Karkos et al., 2005]. 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 [Padwa et al., 1995].

Complications are frequent, and may be multiple, including airway compromise, infection, bleeding and dental caries [Padwa et al., 1995]. Management often involves debulking surgery or sclerotherapy [Ben Gamra et al., 2006]. Spontaneous regression has been noted in the literature [Kumar et al., 2010]. Post-resection recurrence may be seen in approximately 20% of cases [Mirza et al., 2010].

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 (Fig 1). 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.

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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 (Figs 2 and 3).

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 (Fig 4). 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.

Discussion CH affecting the tooth-bearing area is uncommon, and this is the first report to our knowledge describing dental extractions in its immediate vicinity. There are numerous factors that may contribute to this patient's high caries rate. The patient's poor oral hygiene was a major factor. Due to her glossectomy, the patient was limited to a soft diet, with a prolonged oral clearance, and buccal holding of food. She was unable to chew, as a consequence of multiple dental extractions and only eat foods that dissolved in her mouth. The patient's saliva was extremely viscous, which may be as a result of multiple surgeries in the area of her left parotid gland. She was unable to achieve a lip seal, resulting in a profound xerostomia, which has been exacerbated by prolonged antihistamine use.

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It has been suggested in the literature that a history of bleomycin chemotherapy is a contraindication to the use of nitrous oxide-oxygen inhalational sedation. Bleomycin is an anti-neoplastic antibiotic, and can lead to an interstitial pneumonitis and progressive pulmonary fibrosis, in the presence of nitrous oxide [Fleming et al., 1998]. However, research has confirmed that the low dose of bleomycin utilised for sclerotherapy of CH is not associated with pulmonary fibrosis [Okada et al., 1992].

The reasons for failure of local analgesia in this case are unclear, but may be due to uptake of the anaesthetic solution into the CH, altered innervation of the area secondary to multiple surgeries or local inflammation. Certainly, a more profound analgesia was achieved when using articaine 4% with 1:100,000 adrenaline than with lignocaine 2% with 1:80,000 adrenaline. This is consistent with a recent study, which found a significantly increased efficacy of articaine 4% over lidocaine 2% for maxillary infiltration in molar teeth. This increased efficacy has been attributed to the greater degree of lipid solubility of articaine, which increases its diffusibility through soft tissues, and its increased concentration [Srinivasan et al., 2009]. Inhalational sedation, using up to 60% nitrous oxide contributed both analgesic and sedative effects. This, along with a maximum dose of articaine allowed extraction of the teeth with minimum discomfort for the patient.

Conclusion

A potential management strategy for patients with CH, with reference to the myriad of inherent difficulties, which may complicate such treatment is described.

References

Ben Gamra O, Mbarek C, Neji R et al. Cervico-facial lymphangiomas. What's the appropriate management? Tunis Med 2006; 84:721-723.

Fleming P, Walker PO, Priest JR. Bleomycin therapy: a contraindication to the use of nitrous oxide-oxygen psychosedation in the dental office. Paediatr Dent 1998; 10:345-346.

Gedikbasi A, Gul A, Sargin A, Ceylan Y. Cystic hygroma and lymphangioma: associated findings, perinatal outcome and prognostic factors in live-born infants. Arch Gynecol Obstet 2007; 276:491-498.

Karkos PD, Spencer MG, Lee M, Hamid BN. Cervical cystic hygroma/lymphangioma: an acquired idiopathic late presentation. J Laryngol Otol 2005; 119:561-563.

Kumar N, Kohli M, Pandey S, Tulsi S.P.S. Cystic Hygroma. Natl J Maxillofac Surg 2010; 1:81-85.

Mirza B, Ijaz L, Saleem M, Sharif M, Sheikh A. Cystic Hygroma: An Overview. J Cutan Aesthet Surg 2010; 3:139-144.

Mirza B, Ijaz L, Iqbal S et al. Cystic Hygroma of unusual sites: Report of two cases. Afr J Paediatr Surg 2011; 8:85-88.

Mosca R, Pereira G, Mantesso A. Cystic hygroma: characterization by computerized tomography. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008; 105:e65-e69.

Okada A, Kubota A, Fukuzawa M, Imura K, Kamata S. Injection of bleomycin as a primary therapy of cystic lymphangioma. J Pediatr Surg 1992; 27:440-443.

Padwa BL, Hayward PG, Ferraro NF et al. Cervicofacial lymphatic malformation: clinical course, surgical intervention and pathogenesis of skeletal hypertrophy. Plastic Reconstruct Surg 1995; 95:951-960.

Rautio R, Keski-Nisula L, Laranne J et al. Treatment of Lymphangiomas with OK-432 (Picibanil). Cardiovasc Intervent Radiol 2003; 26:31-36.

Srinivasan N, Kavitha M, Loganathan CS, Padmini. Comparison of anesthetic efficacy of 4% articaine and 2% lidocaine for maxillary buccal infiltration in patients with irreversible pulpitis. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009; 107:133-136.

Velez I, Mintz S. Cystic Hygroma Case Report. N Y State Dent J 2006: 72:51-52.

Barry S *, Allotey J **, Brundler A.M **, Duggal M.S *

* Department of Paediatric Dentistry, Leeds Dental Institute, Leeds.

** Histopathology Department, Birmingham Children's Hospital, Birmingham, UK

Postal address: Dr. S. Barry, Department of Paediatric Dentistry, Leeds Dental Institute, Clarendon Way, Leeds, LS2 9LU

Email: dnsmb@leeds.ac.uk
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Article Details
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Author:Barry, S.; Allotey, J.; Brundler, A.M.; Duggal, M.S.
Publication:European Archives of Paediatric Dentistry
Article Type:Report
Geographic Code:4EUUK
Date:Dec 1, 2012
Words:1617
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