Cystic hygroma may be associated with:
Correct Answer: Turner's syndrome
Description: Cystic hygroma Cystic hygromas are multiloculated cystic spaces lined by endothelial cells It results due to sequestration of a poion of jugular lymph sac from the lymphatic system Cysts are filled with clear lymph and are lined by endothelium Turner's syndrome is associated with cystic hygroma Most cystic hygromas involve the lymphatic jugular sacs Cystic hygroma MC site: Posterior neck region Other common sites: Axilla, mediastinum, inguinal & retroperitoneal regions Approximately 50% of them present at bih It may show spontaneous regression Clinical features Usually present at soft cystic masses that disto the surrounding anatomy, can result in acute airway obstruction. Usually manifests in the neonates or in early infancy (50% present at bih) Prone to infection & hemorrhage within the mass Swelling is soft & paially compressible and invariably increases in size when the child coughs or cries. Characteristic features: Brilliantly translucent Diagnosis MRI play a crucial role in preoperative planning Treatment Complete surgical excision is the preferred treatment Injection of Sclerosing agents such as bleomycin or OK-432 (Picibanil), derived from streptococcus pyogenes may eradicate the cystic hygroma.
Category:
Surgery
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