Sudden Onset, Rapidly Expansile, Cervical Cystic Hygroma in an Adult: A Rare Case with Unusual Presentation and Extensive Review of the Literature

Abstract

Cystic hygroma (CH) is a benign infiltrative malformation of the lymphatic channels. We report a case of a 28-year-old Indian female who presented with rapidly enlarging right sided neck swelling over the posterior triangle since 5 days. Complete resection of CH is sometimes not amenable because of its infiltrative nature and involvement of surrounding vital structures. However, in our patient successful complete surgical resection was undertaken. The MRI findings of our patient were consistent with brachial cleft cyst; this posed a challenge in the diagnosis of CH. The histopathological analysis of the resected mass confirmed CH. CH is rare in adults and such an acute presentation is exceptionally atypical. History of prior trauma and infection are known etiological factors for adult CH; these were conspicuously absent in our patient. CH should be considered in the differentials of rapidly enlarging cystic swelling of posterior region of neck in adults. Optimal and timely management is necessary to achieve a favorable prognosis. Therefore, we report a case of rapidly enlarging cervical CH in an adult along with extensive literature review to have a better understanding regarding epidemiology, etiopathogenesis, clinical presentation, optimal management, and prognosis of such a rare entity in adults.

Cystic lymphangioma in adult: a case report and a review of the literature

“…Cystic lymphangioma (CL) is a benign congenital lymphatic malformation [ 1 ]. Children under 2 years of age are mostly affected.…”

Adult-onset giant cervical cystic hygroma with pressure manifestations on aerodigestive tract, managed surgically: reporting of a rare case

“…Extensive lesions in the floor of mouth, oropharynx or in the neck can lead to pressure manifestations such as dysphagia when compressing the oesophagus or dyspnoea due to tracheal or laryngeal compression. 5 The most distressing symptoms in our patient were caused by the pressure effect of the hygroma in the form of dysphagia and postural shortness of breath.…”

“…Imaging methods such as ultrasound, magnetic resonance imaging and computed tomography (CT) have been used prior to treatment, although imaging preferences vary according to their cost, convenience and resolution. 5 Preoperative imaging is important to look for intrathoracic extension, which is present in 10% of cases. 13 Although in our patient the diagnosis was made clinically and supported by the ultrasound, we performed CT for better anatomical mapping and planning for the surgical approach.…”

“…Several staging and classification systems have been employed for better diagnosis and management. 5 Smith et al described lymphatic malformation as microcystic, macrocystic or mixed variant, with macrocystic containing cysts more than 2 cm in diameter. 14 A classification based on CT, anatomical location and histology was proposed by McGill and Mulliken in 1993 and is clinically relevant.…”

“…Clinical presentation depends on the location, size and rate of growth of malformation. [3][4][5] Diagnosis in adults is considered to present a greater challenge than in children, and final diagnosis is usually based on postoperative histology. There are few reports of adult cervicofacial cystic hygroma in the English language literature and the optimum management of these lesions is still a matter of debate.…”