eCommons@AKU eCommons@AKU

Haemangioendotheliomas (HAE), although rare but are the most common parotid gland tumours in children. We report a 4-month-old girl who presented with a progressively enlarging right sided facial swelling overlying the angle of the mandible. An Ultrasound of the lesion and a computed tomography (CT) scan of the head and neck was carried out which revealed a large lesion within the right parotid gland. CT scan further demonstrated a direct communication with the right external carotid artery and external jugular vein. Considering the clinical course and radiological findings, there was sufficient evidence to avoid any invasive testing. Due to the self-limiting nature of the disease, patient was managed expectantly.


Introduction
2][3][4][5][6][7] They constitute 1-5% of all salivary gland neoplasms. 8These present in infancy as a painless large rapidly growing mass. 4It is a benign and self-limiting condition. 4,5It is therefore of paramount importance that early and accurate diagnosis be made to avoid invasive procedures and alleviate undue anxiety among the caretakers.
We report the case of a infantile haemangioendothelioma of the parotid gland that was managed conservatively at our centre.Although, there have been previous case reports on infantile haemangioendotheliomas, [1][2][3][4][5][6][7][8][9][10] this is the first time such a case is coming to light from our part of the world.
Prior permission from the patient's guardians was acquired before the preparation of this manuscript.

Case Report
A 4-month-old girl was brought to the paediatric surgery outpatient clinic on the 22nd of July 2018, with a progressively enlarging right sided facial swelling.Swelling was first noticed at one month age by her parents.It was located at the right side of angle of the mandible and had no overlying erythema or discharge.There was no demonstrable tenderness on palpation.Parents denied any presence of fever, signs of infection or feeding issues and the patient was otherwise healthy with adequate growth and no developmental delay.Patient was born to non-consanguineous parents.She was delivered spontaneously at term without any natal or peri-natal complications.She had no relevant past medical, family or genetic history.

Discussion
Haemangioendotheliomas (HAE) are neoplastic growths of vascular endothelial cells. 11Juvenile haemangiomas may occur on any part of the body but most commonly occur in the head and neck region particularly, the parotid gland. 6These are benign in nature and most cases of HAE appear in the early months of the life. 5Median age of the presentation is around 4 months. 1,3,4There is a female predominance with a 3:1 ratio compared to males. 3,4,8Bluish/purplish discolouration of the overlying skin is a suggestive clinical sign. 1,8The diagnosis may also be supported by a cutaneous strawberry haemangioma either locally or elsewhere. 5,8fantile parotid haemangiomas have a natural history that includes rapid growth during infancy followed by gradual involution at around 8-18 months. 1,4They are usually not noticeable in the neonatal period but become prominent in the first few months of life. 1,5aracteristic imaging findings aid diagnosis so as to avoid invasive biopsies in the infants.Sonographic findings include a homogenous mass arising in the parotid gland with a lobular structure, fine echogenic internal structure and a lobulated contour. 7,8The presence of large blood vessels within the tumour is highly suggestive. 4CT scan shows a soft tissue mass that enhances with contrast material.On MRI these lesions appear isointense when compared to muscle on T1weighted MRI and they show hyperintense or intermediate signal on T2-weighted MRI.The lesions exhibit homogenous contrast enhancement on MRI. 11RI is the best imaging modality. 1,3 Red cell scintigraphy can diagnose head and neck haemangiomas with very high accuracy.It shows uniform, well defined uptake similar to the heart and great vessels. 4 came across three cases that reported an association between infantile parotid haemangioendothelioma and a cytomegalovirus (CMV) infection. 2,6,10This suggests that there might be some aetiological role of CMV in this pathology.However, it is equally possible that these findings may have been purely incidental.
Since, many tumours regress on their own, no active management is required.Surgery is not recommended due to the risk of damage to the underlying facial nerve. 4,8There is reported success with oral propranolol, it has been proven an effective, safe and well tolerated treatment option. 1,9,12ropranolol facilitates vasoconstriction, decreases expression of vascular growth factors and activates apoptosis of capillary endothelium cells. 1 Minor side effects include agitated sleep, diarrhoea and hypoglycaemia. 12These are all easily controlled. 12re but significant systemic complications of parotid HAE include cardiac failure and Kasabach Merrit syndrome. 4,5Kasabach Merrit syndrome involves a vascular tumour and developing subsequent thrombocytopenia.Treatment of choice in such cases are oral steroids and interferon alpha. 4,5Our patient had normal platelet levels, as previously mentioned and so was not currently at risk of developing such a condition.
The Table , summarises case reports published in the last 10 years that were accessible to us as full text English articles.

Conclusion
Suggestive clinical course and characteristic findings on radiology provide sufficient evidence for confident diagnosis of infantile haemangioendotheliomas of the parotid gland.There is no need for invasive biopsy procedures.Prognostically the disease shows resolution with no medical or surgical intervention.
Disclaimer: None to declare.

Table:
Case reports on infantile haemangioendothelioma of the parotid gland, published in the last 10 years that were accessible as full text, English articles.

Figure- 2 :
Figure-2: Head CT showing a large right parotid swelling with intense contrast uptake demonstrating vascularity.

Figure- 3 :
Figure-3: Coronal CT Sequence demonstrating communication of Right Parotid mass with internal carotid artery.
Non contrast images showed diffuse enlargement of superficial and deep lobes of parotid with lobulated contour.Post contrast images showed intense enhancement of the entire left parotid gland.USG: Large lobulated tumour USG: Enlarged parotid (3.1 x 2.2 x 2.5 cm) with increased vascularity MRI: well-defined, lobulated homogenously enhancing lesion diffusely involving the superficial and deep layers of the parotid gland USG: Large lobulated mass with echogenic septations replacing the right parotid gland Colour Doppler showed multiple intratumoral vessels with both arterial and venous components MRI: lobulated mass with flow voids replacing superficial and deep lobes of right parotid gland.The lesion is isotense to muscle on T1 and hyperintense on T2.USG: Colour Doppler showed enlarged and heterogenous right parotid gland MRI: Hyperintense right parotid mass containing vascular flow voids on T2.