Clinical presentation of congenital neck mass in children

Background: Congenital neck mass is a very common problem in children in Otolaryngology & Head-Neck Surgery and Paediatric Surgery Department but data of our country is inadequate and there is lack of published study. This study will help to diagnose congenital neck masses, evaluate the success of surgical treatment in our country. Objective: To assess the frequency of congenital neck mass in children and to see the clinical presentation of different congenital neck mass. Study design: Cross sectional prospective study. Place of study: Department of Otolaryngology – Head & Neck Surgery, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Dhaka Medical College Hospital (DMCH)l, Dhaka and Mymensingh Medical College Hospital (MMCH), Mymensingh. Methodology: Patients below the age of 18 years with congenital neck mass who presented to three tertiary level hospitals from January 2010 to December 2010 were included in this study. Total thirty six patients were purposively selected. Age, gender, types of swelling and location were examined, investigations were done. Data was analyzed with SPSS software and was presented in the form of tables, diagrams and pie charts. Results: 36 patients with different congenital neck swelling were examined. Out of 36 patients, thyroglossal cysts were commonest21(58.33%). Other congenital lesions were as follows: Branchial cyst 7(19.44), Lymphangioma 3(8.33%), Haemangioma 3(8.33%), Dermoid cyst 2(5.55%). Midline was most common location 21 (58.33%). Conclusion: Congenital neck masses constitute important differential diagnosis for neck masses. These masses constitute diagnostic and therapeutic challenges for many clinicians. Correct diagnosis, safe and complete surgical excision requires sound knowledge of the location and extent of these masses.


Introduction:
Congenital masses are the most common non-inflammatory neck mass 1.They result mainly from defective transformation of branchial arches and their derivatives.They constitute important differential diagnosis for neck masses.These masses constitute diagnostic and therapeutic challenges for many clinicians.Correct diagnosis, safe and complete surgical excision requires accurate knowledge of the location and extent of these masses 2 .These masses are classified as lateral neck masses (including branchial cyst or fistula), midline neck masses (including thyroglossal duct cyst or fistula, thymic cyst, dermoid cyst, and teratoma of the neck), and masses of the entire neck (including haemangioma and lymphangioma) 1 .These include, in descending order of frequency, thyroglossal duct cysts, branchial cleft anomalies, dermoid cysts, and median cervical clefts.The thyroglossal duct cyst is the commonest congenital neck mass, accounting for 70% of congenital neck anomalies and second common benign neck mass, after lymphadenopathy 3 .Branchial anomalies account for up to 17% of all paediatric cervical mass.Dermoid cysts account for up to 25% of midline cervical anomalies 5 .Approximately 7% of dermoid tumour occurs in the head and neck 4 .Lymphangioma are uncommon with only five cases per 3000 admission to a paediatric hospital and only account for four of 152 benign tumours of the neck 6 .Vascular lesions are among the commonest congenital neonatal abnormalities 7 ..In one series 32% were younger than 10 years and 40.5% were older than 30 years 6 .In this series mean age of presentation was 6-8 years.It differs from study to study.

Methods
In different articles it is shown as 7.8 years 2 , 37.6 years 12 , and 15.4 years 13 .
This study showed 19(90.48%)patient present with midline neck swelling and 2 (9.52%) patients present with lateral neck swelling on left side. 1 (4.36%) patient present with painful neck swelling, 2 (9.52%) present with infected cyst and 2 (9.52%) present with recurrent cyst.In published series 90% cyst are located in midline and 10% in lateral portion of neck of which 95% in left and 5% in right side 6 .In another study 89.8% were in midline and 10.2% were in lateral position 13 .
In present series 7 (19.44%)patients of branchial cyst found of them 5 (71.43%) were in second decade and 2 (28.57%) in first decade.One study showed peak age of incidence in third decade 4 and another showed in second decade 2 .Among the patients 4 (57.14%) were male and 3 (42.86%)were female.In most published cases 60% male and 40% female 4 .But one study showed equal ratio of male and female 8 .
Location of branchial cyst were 5 (71.43%) in left side and 2(28.57%) in right side, among them 6(85.71%) in upper neck and 1(14.28%) in lower neck along the anterior border of Sternomastoid muscle.All the branchial cysts were cystic.One patient gave history of previous surgery (14.28%), one patient present with infected cyst (14.28%).In most published series 60% branchial cyst in left side and 40% in right side, 66.67% in upper neck and 33.33% in lower neck, 70% cystic and 30% solid 4 .
Congenital vascular problem is not infrequent in our country.In this present series 6 cases were identified, among them lymphangioma 3(8.33%) and haemangioma 3 (8.33%).
Age of patient of lymphangioma were 2 (66.67%) in 0-4 years and one (33.33%)above 10 years.Two (66.67%) patients present with swelling in right posterior triangle and one (33.33%) in anterior triangle left side.One (33.33%) was capillary lymphangioma and two (66.67%) were cystic hygroma.No case of cavernous lymphangioma was found.Though capillary lymphangioma is more common in most of the study 6 cystic hygroma is common in this series.This variation may be due to small sample size.
In one study showed cystic hygroma is the most commonly encountered problem among lymphangioma; of which 50-60% present at birth or perinatally and 30% by age of 2 years 19 .
Haemangioma is commonest benign tumour of infancy and head-neck is affected by 14% to 20% 6 .In our series there were 3 patients with haemangioma, among them 2(66.67%)present from birth and 1(33.33%) in second decade.2 patients were male and 1 patient was female.Among the three cases, 2(66.67%)cases found in right side of neck and 1(33.33%)case in left side of neck.One of the haemangioma extended to the right parotid region and another one was extended to thorax.Only one haemangioma was confined to neck only.Infantile haemangioma typically appears in the first few weeks of life 20 , usually within the first month of life 21 , proliferates for weeks to several months 20 .A study found male-female ratio was 19:1, was present from birth in 24% children, 75% were located on the scalp and forehead 22 .
Dermoid usually present in 2 nd and 3 rd decade but probably been present since birth 11 .Epidermoid cyst is the most common variety 11 .In this series, 2 patients of dermoid were found.One patient presents with cystic submental swelling and another presents with solid infrahyoid swelling.No case of teratoid cyst or sublingual variety was found.Histopathology shows one (50%) was epidermoid cyst and one (50%) dermoid cyst.
In the series ultrasonography was done in all patients and ultrasonography shows 34 20 (94.44%) patients having cystic swelling and remaining 2 (5.56%) patients having solid swelling.All of the solid swellings found in dermoid.
In this series all patient (100%) with congenital neck mass were treated surgically.In thyroglossal duct cyst Sistrunk's operation was done and others are treated by excision.
No conservative treatment or treatment with sclerosing agent was done.

Conclusion:
Congenital neck masses constitute diagnostic and therapeutic challenges for many physicians.Work-up and management is lesion dependent, and proper preoperative diagnosis is essential for planning and performing appropriate surgical procedure.