cystic hygroma

Cystic Hygroma

Anuja Sriparameswaran, MD
Volume 15 - Issue 2 - February 2016

The patient had a soft mass on the right lateral neck.A 28-year-old gravida 2 now para 3 mother delivered a term male infant by scheduled cesarean. The mother had an uncomplicated pregnancy. At birth the infant was noted to have a soft mass on the right lateral neck, but was otherwise well without any other dysmorphic features. The infant was initially observed in the neonatal intensive-care unit and had an uneventful stay; he was then observed in the wellborn nursery for 2 additional days. The patient did well without any respiratory distress and was breast-feeding successfully. 

An ultrasound was performed and found a multiseptated anechoic lobulated lesion in the right lateral neck that measured 4.8 × 3.1 × 2.9 cm and was diagnosed as lymphatic malformation/cystic hygroma. The infant was seen in the surgery clinic on day 5 after birth and was doing well; repeat ultrasound measurements were consistent with previous measurements. Magnetic resonance imaging performed later found a small macrocystic lymphatic malformation in the posterior triangle on the right side of neck. 

Cystic hygroma is a congenital malformation of the lymphatic system, where failure of communication between lymphatic and jugular veins leads to dilation of the lymphatic vessels.1,2 They are mobile, nontender fluctuant masses that transilluminate and are commonly found laterally in the area of the posterior triangle of the neck.3-5 It is a benign tumor and has the potential to infiltrate adjacent structures.6 They can develop anytime; however, 50% of them are present at birth and 90% become apparent by 2 years of age.7-9 The lesions rapidly enlarge because of infection with upper respiratory virus or with trauma.2,3,10

The most common location is in the neck (75%) followed by axilla (20%) and less commonly in the mediastinum, groin, and retroperitoneum.6 In the differential diagnosis of neck masses, thyroglossal duct is the most common congenital lesion that may present, followed by brachial cleft cyst, and cystic hygroma or hemangioma.9

Syndromes associated with cystic hygromas are Turner’s syndrome, Noonan’s syndrome, fetal alcohol syndrome, distichiasis-lymphedema syndrome, and familial pterygium colli and aneuploidies; trisomy 21, 18, and 13.2,11,12 Chromosome abnormalities are associated with a poor prognosis.12 Cystic hygromas that develop in the third trimester or in the postnatal period are usually not associated with chromosome abnormalities.12  

Surgery is favored as a primary treatment in most published studies, and a good outcome relies on the location of the lesion and complete excision.10 Incomplete excision of the lesion could result in its recurrence.4 Alternatives to surgery include needle aspiration, diathermy, radiation, and injection of sclerosing agents. Sclerosing agents used are steroids, alcohol, bleomycin sulfate, tetracycline, and OK-432.10,13

Some studies recommend close observation of asymptomatic patients with no airway compromise, feeding difficulties, or recurrent infection. If the mass is the only presenting symptom, waiting for at least 18 months to 2 years of age is suggested. If no regression is noted by 5 years of age, a surgical approach or injection of sclerosing agent is recommended.10,13

In this case, our patient was referred by a surgeon to an interventional radiologist to treat with sclerotherapy. However, the decision was made to observe due to the asymptomatic nature and size of the lesion, until the child was 1 year of age, because this will minimize the risk of the procedure from an anesthetic and procedural standpoint.

REFERENCES

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  11. Cystic hygroma, 2008. http://www.genetics.emory.edu/documents/resources/Emory_Human_Genetics_Cystic_Hygroma.PDF. March 25, 2015. Accessed August 11, 2015.
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  13. Keneddy TL, Whitaker M, Pellitter P, Wood E. Cystic hygroma/lymphangioma: a rational approach to management. Laryngoscope. 2001;111:1929-1937.