presentation of benign neck diseases by dr. falaq faiyaz

presentation regarding neck diseases

1. Presentor - dr.falaq faiyaz Moderator- dr.pankaj kumar verma Benign neck disease

2. Introduction Neck masses are common occurrence in adult population ,approx 75 % of non-thyroid neck masses are neoplastic & of these 80% are metastatic. 75% of these are caused by primary tumour located above clavicle. In children 90% of neck swelling are benign of which 55% is congenital. FNAC with or without USG is gold standard investigation for neck swelling

3. Congenital neck masses Lymphangiomas – degenrative lesions arising from lymphatics ,classified as simple, cavernous & cystic hygromas . Lymphangioma simplex- k/a capillary lymphangiomas ,composed of thin-walled capillary sized lymphatic channels.presenting as pale , small vesicle like lesions . Cavernous lymphangioma - 40% of all lymphangiomas ,composed of dilated lymphatic spaces with fibrous adventitia,typically occuring in tongue,cheeks & lips

4. Cystic hygroma - composed of cyst & sinuses,varying size from few mm to several cms,presenting as a cystic mass containing eosinophilic acellular lymph fluid. Aetiology - lymphatic system arises from 5 primitive sacs (2 jugular,2 post. Sciatic sacs & single retroperitoneal sac) developed from venous system. Theories of origin- 1)sequestration of lymphatic tissue derived from segments of the primitive sacs,which retain proliferative growth potential & bear no connection with normal lymphatic system.

5. 2) endothelial fibrillar membrane proliferation from walls of cyst ,which penetrate surrounding tissue along line of least resistance b/w muscles & vessels,canalize & produce more cysts. Clinical features- mostly congenital, appears anywhere in head & neck, on palpation feel cystic & transilluminate.can grow rapidly potentially risking life threatening airway obstruction. Investigation- CT & MRI accurately determine location & relationship toh surrounding structures.

6. Treament Many treatment modalities depending upon size ,location & involvement of surrounding structures. Injection of sclerosants - bleomycin,tertacyclines & alcohol Intra-lesion injection of OK-432 ( pincinabil )- causes an inflammatory reaction & thrombosis with subsequent necrosis. Surgical excision remains tratment of choice.

7. Dermoid cyst Classified as epidermoid , true dermoid & tetroid cysts , depending on types of tissues identified pathologically. They make upto 28% of all midline cysts with no sex predominance Epidermoid cysts- contains only skin & no other adnexal structures,lined by squamous epithelium with or without keratin material True dermoid cyst- lined by squamous epi . ,contains skin with appendages such as hair,hair follicles,sebaceous glands & sweat glands.

8. Teratoid cysts- lined either with squamous or respiratory epithelium,contain all 3 embryological elements- ectodermal , endodermal & mesodermal elements such as – nails,teeth ,brain & glandular tissue. Aetiology – ectodermal differentiation of multipotential cells tapped at time of closure of ant. Neuropore ,especially along lines of fusion hence located in midline

9. Clinical features- peak age of incidence is usually second & third decade,they present as cystic or solid painless mass ,usually submental ,they may manifest as acute inflammatory swelling associated with infection. Diagnosis- USG guided fnac , cross-sectional imaging such as CT & MRI Treatment- surgical excision

10. Thyroglossal duct cyst Mc upper neck midline lesion accounting for almost 1/3 rd of all congenital neck masses . Present as a mass at any level b/w foramen caecum & upper mediatinum,majority presenting abt level of hyoid bone Aetiology – embryologically thyroid gland originates from floor of primitive pharynx b/w 1 st & 2 nd pharyngeal pouches.thyroglossal duct cyst arise from epithelial cells when they cease to remain inactive .

11. Stalk usually atrophies during 6 th week but should it persist as a patent tract, it becomes thyroglossal duct along which cysts can develop . It may run from thyroid gland inf. Upwards & in region of hyoid bone ,tract may be located behind through or in front of hyoid. Ending deeply into junction of ant. 2/3 rd & post. 1/3 rd of tongue at foramen caecum . Fistula usually arises from spontaneous drainage of an abscess or more commonly following attempted drainage of a misdiagnosed midline neck abscess/incomplete excision

12. Clinical features- approx 95% of thyroglossal cyst present as asymptomatic cystic mass at level of hyoid bone.moves on swallowing or on protrusion of tongue. Upto 5% present as acute inflammatory episode with an infection & 15% with discharging fistula.mean presentation age is 5 yrs Diagnosis - TFT,usg -FNAC,USG- helps locate ,diagnose & also confirm pesence of normal thyroid gland in its expected position .

13. Treatment- sistrunk operation – excision is performed through a transverse midline- neck incision just below cyst , lesion is dissected from infra-hyoid strap muscle & laryngeal cartilages. Treatment of reccurent cysts & fistulae- Previous incision scar should be excised & central compartment neck dissection performed with excision of scar tissue up to foramen caecum .

14. Branchial cysts Brachial cysts appears as developmental failure of brachial apparatus. Account for 19% of paediatric cervical neck masses. Cysts are usually lined by stratified squamous epithelium except a few cases may have respiratory epithelium,80% have lymphoid tissue in outer wall & contain straw coloured fluid with cholestrerol crystals are found. Aetiology - brachial apparatus theory , cervical sinus theory , thymopharyngeal theory & inclusion theory .

15. Branchial apparatus theory – brachial cysts represents remains of pharyngeal pouches or brachial clefts or fusion of 2 elements. Development of branchial apparatus extends from 3 rd to 8 th week of gestation . Cervical sinus theory – cysts occurs due to remains of cervical sinus of His, formed by 2 nd arch growing down to meet 5 th arch. Thymopharyngeal duct theory – cyst are remnants of original connection b/w thymus & 3 rd brachial pouch from which it takes origin

16. Contd … Inclusion theory – branchial cysts are epithelial inclusions within lymph node. Clinical features- 60% are located in upper 3 rd of neck at ant. Margin of SCM although occur at any side of neck including parotid gland. Diagnosis- usg – fnac – yields acellular fluid with cholesterol crystals on microscopy. Treatment - surgical excision- as brachial cyst have a tendency to become infected , can increase to a large size causing discomfort, pressure symptoms. Transverse cervical incision overlying cyst

17. Branchial fistulae & sinuses Congenital defects consisting of a skin-lined tract,opening internally as a slit on ant. Aspect of tonsil fossa , if it is of 2 nd arch origin . External opening is on ant. Border of SCM at junction of its middle & lower thirds. Branchial sinuses – branchial pits open along line b/w tragus & SCM joint at ant. Border of SCM but with no internal opening.

18. Aetiology - arise from failure of complete development of branchial apparatus including 1 st ,2 nd 3 rd & 4 th arches. During 4 th week of IUL ,6 branchial arches develop as neural crest cells migrate cranially .during 5 th week , 2 nd branchial arch grows over 3 rd & 4 th branchial clefts ,forming a cervical sinus .failure of cervical sinus to close allows potential communication with 2 nd branchial pouch in area of tonsil fossa . 3 rd pouch in area of larynx & 4 th pouch opening in pyriform fossa .

19. Clinical features- mostly infants with discharging sinus, which may or may not have an internal fistulous opening . Clinically 2 nd branchial cleft fistulae are mc , they have a cutaneous opening along ant. Border of SCM, usually at junction of middle & lower 3 rd ,tracking up b/w int. & ext. carotid arteries ending in tonsillar fossa . 3 rd & 4 th branchial fistulae are rare , opening low in neck & ending in pyriform sinus.

20. Diagnosis- contrast swallow or CT fistulogram may be helpful to determine pathway of tract ,differentiate a sinus from a fistula & aid surgical planning. Treatment- surgical excision in stepladder fashion , removing mouth of sinus with ellipse of neck skin.tract is followed upwards as high as possible & then another transverse cervical or cervico -facial incision is made.

21. Acquired neck diseases & masses Non- inflammatory neck masses-sebaceous cysts- skin appendages lesions occuring mainly where there are sebaceous glands & are commonest in hairy skin, particularly in beard region & scalp. Aetiology - cysts develop when duct of sebaceous skin gland , becomes blocked . retained secretions distend gland causing progressive enlargement of cyst. clinical features- lesions can be single or multiple & of diff. sizes, usually spherical , smooth & well defined,stretching overlying skin ,with punctum

22. Acquired dermoid cyst(implantation dermoid ) Solitary ,with solid & cystic areas containing skin appendages. Aetiology - occur due to penetrating injury or following surgery resulting in implantation of dermal or skin structures deep into subcutaneous tissue. Clinical features- present at site of penetrating injury & display solid & cystic areas containing sebaceous material.

23. Pilomatrixoma or calcifying epithelioma of malherbe Benign tumour of prickle cell layer of skin ,mc in 1 st 2 decade of life.mc in females. Aetiology – unknown Clinical features- solitary ,firm nodular superficial lesions measuring up to 3 cm in size.usually no discolouration but if situated superficially a blue-red colour may be seen. Diagnosis- cytopathological features- identification of basaloid & squamous cells , calcium deposits & foreign body giant cells.

24. lipomas Benign lesions of adipose layer,organized into large lobules divided by loose fibrous septa.can be multiple & painful ( dercum ds ) Mc lipomatosis in head and neck region is madelug’s lipomatosis.usually may be subfascial or arising within muscles. Aetiology – h/o preceding trauma leads to breakdown of adipose layer & abnormal growth .

25. Inflammatory neck masses acute cervical lymaphadenitis – occurs due to viral /bacterial infection in upper aerodigestive tract ,ears or skin of head & neck. Clinical features- painful mass , may result into an abscess becoming fluctuant. Treatment – initially empirical

26. Infectious mononucleosis Glandular fever is viral infection caused by EBV ,affects adolescents & younger adults.also asso . With lymphoid or epithelial cell malignancies such as hodgkin lymphoma,burkitt lymphoma, nasopharyngeal carcinoma & multiple sclerosis. Aetiology – EBV is gamma herpes virus infects 90 % of human population ,transmitted via saliva ,life long latent inf. Of B cells Clinical features- asymptomatic /self limiting illness

27. Diagnosis- DLC - > 50% mononuclear cells & 10% atypical lymphocytes.ESR & CRP high . monospot paul bunnell test – positive.raised lft . Treatment – supportive & conservative treatment with avoidance of contact sports for 6 wks.corticosteroids are given for airway obstruction ,thrombocytopenia ,cranial nerve paralysis.

28. Cat scratch disease Peak incidence b/w 2- 14 yrs of age , more common in USA. Aetiology – caused by ricketssia bartonella . Clinical features- cervical lymphadenopathy with erythmatous papulae at site of inoculation , fever ,malaise ,anorexia , headache & splenomegaly . Diagnosis- PCR RNA Treatment – antibiotics gentamicin , rifampicin & ciprofloxacin

29. Cervical necrotizing fasciitis Rare odontogenic / tonsillar bacterial infection may be complication following deep space neck infections- streptococcus pyogenes / viridans . Clinical features- cutaneous cellulitis with disproportionate pain that progresses to necrosis of subcutaneous tissue & skin. Diagnosis- high WBC , usg /CT may show oedema & air pockets of skin Treatment – high dose IV antibiotic with debridement of all necrotic areas.

30. Chronic cervical lymphadenitis Often asso.with systematic symptoms such as malaise , weight loss , anorexia & night sweats. Conditions include – HIV , sarcoidosis , toxoplasmosis , actinomycosis , & TB. HIV-AIDS- classified into – acute infection or seroconversion , asymptomatic infection , persistent generalized lymphadenopathy & full blown AIDS. CLINICAL FEATURES- 30 % seroconversion pt develop PGL –tends to be non- specific & it is characterized by multiple diffuse lymph adenopathy involving more than 2 inguinal sites <3 months.

31. Investigation- HIV serology with FNAC – it will also help differentiate other causes such as TB , NON-HODGKIN LYMPHOMA & kaposi sarcoma. TUBERCULOUS ADENITIS- OLDEST DOCUMENTED INFECTIOUS DS.tuberculous adenitis / scrofula – can affect any LN of head and neck including thyroid gland & salivary gland. Aetiology - mycobacterium TB –obligate Anaerobe .humans are biggest reservoir, after a short period of replication in lungs ,silent dissemination occurs via lymphohematogenous system to extra pulmonary sites .

32. Clinical features- 90 % of pts have unilateral involvement of jugular chain of LN f/b submandibular triangle & post. Triangle . Ln - usually firm ,painless & present with a characteristic erythmetous skin discolouration , if ds is undiagnosed they tend to fistulate or form cold abscess. diagnosis - difficult to diagnose , cbc –may be absolutely fine , with raised ESR, X-ray chest is mandatory ,soft tissue xray of neck may show dystrophic calcification , usg - multiple matted LN ,FNAC ,PCR & EXCISION BIOPSY

33. NON-TUBERCULOUS ATYPICAL MYCOBACTERIAL ADENITIS Common in west , differ from TB as it tends to infect otherwise healthy immunocompetent children . Aetiology - mycobacterium avium ,route of entry is through oropharynx & eyes. Clinical features- cervical lymphadenopathy with out systemic involvement. Diagnosis- culture of microbiological swab , FNAC or incisional biopsy. Treatment- eventually develop immunity but macrolides can be given , surgical excision is ultimate treatment of choice.

34. Miscellaneous rare causes of neck masses Organised haematomas - aetiology – blunt /penetrating trauma. c/f – neck mass with local discomfort & constant pain. Diagnosis & Inv- usg guided fnac , sonographic image may differ acc. To age of haematoma ,CT & MRI display radiological features & anatomical extent. Treatment- surgical excision

35. Contd … Castleman disease- rare entity characterised by hyperplasia of LN & capillary proliferation affecting adolescents & young adults. Histologcal division- hyaline vascular type,plasma cell type & mixed type Clinical type- localized types (ECL) benign clinical course & multicentric (ECM) worst prognosis leads to non- hodgkin lymphoma.

36. Aetiology – unknown ,but 50% multicentric variants are cuased by kaposi sarcoma- associated virus (KSHV) c/f- progressively enlarging lat. Cervical lymphadenopathy asso . With an autoimmune iron deficiency anaemia .

37. Kikuchi disease Self limiting disease of lymph node affects young women a/k/a- kikuchi fujimoto ds . Histologically – characterised by histiocytic necrotizing lymphadenitis. Aetiology - autoimmune aetiology has be proposed by human leucocyte antigen class 2 gene.ass. With SLE c/f- persistent intermittent fever with tender enlarged cervical lymph node. Treatment- self limiting

38. Kawasaki disease Acute self limiting vasculitis in children. c/f- characterized by fever , b/l non- exudative conjuctivitis , erythema of lips & oral mucosa , changes in extremities,rash & cervical lymphadenopathy ,untreated children may lead to MI . No laboratory test available . Treatment- intravenous gammaglobulin therapy & high doses of aspirin.

39. Thank you