CASE CONFERENCE Ashmita Monga, M.D.

1. CASE CONFERENCE Ashmita Monga, M.D.

2. 19 day/o female with swelling on neck. 2

3. HPI Swelling on the R ant neck for 5 days Not increasing in size No history of trauma No history of fever No history of redness No difficulty in breathing No history of keeping neck to one particular side Has good po intake No excessive crying, no abnormal behavior 3

4. BIRTH HISTORY FT, NSVD with no complications with normal nursery course Maternal labs – RPR neg, HIV neg, Hepatitis B neg, GBS neg, PPD neg Neonatal labs – RPR neg, no ABO incompatibility No history of forceps delivery or vacuum assisted delivery 4

5. SOCIAL HISTORY Baby lives with mother, grand-mother, uncle. No smokers. Has two dogs and two birds. Father recently travelled to DR. 5

6. PHYSICAL EXAMINATION VS – T 98.6 F, P 120, R 38, O2 Sat 99 % Alert, active, in NAD Neck – firm, irregular mass ~ 2 cm in diameter on distal part of right sternocleidomastoid muscle. Non-erythematous, Non-tender, movable, not fixed. Rest of PE - WNL 6

8. DIFFERENTIAL DIAGNOSIS OF NECK MASSES 8

9. CONGENITAL MASSES VASCULAR AND LYMPHATIC MALFORMATIONS INFECTIOUS AND INFLAMMATORY LESIONS OTHER BENIGN LESIONS

10. CONGENITAL MASSES VASCULAR AND LYMPHATIC MALFORMATIONS INFECTIOUS AND INFLAMMATORY LESIONS OTHER BENIGN LESIONS

11. BRACHIAL CLEFT CYST Formed along course of 1st, 2nd, 3rd or 4th brachial clefts as a result of improper closure during embryonic life. 2nd Brachial cleft cysts are most common. May be inherited as autosomal dominant trait. May be unilateral or bilateral. May open into the cutaneous surface or drain into pharynx. Secondary infection– treat with antibiotics. 11

12. THYROGLOSSAL DUCT CYST Most common midline mass. Failure of involution of any portion of thyroglossal duct can cause this cyst. Presents as asymptomatic midline mass or below the hyoid bone which elevates with tongue protrusion. Can be associated with ectopic thyroid tissue. Preoperative workup- TFT’s vs ultrasound. Thyroid scan - In hypothyroid individuals or if abnormal result on US. Management- Excision ( Sistrunk Procedure). 12

13. THYROGLOSSAL DUCT CYST

14. THYMUS GLAND ANOMALIES Failure of involution of thymopharyngeal ducts may persist as cervical thymic cysts. Firm, mobile masses found in lower aspect of the neck. Presents during first decade of life. Work up- CXR and CT. Management- surgical excision. 14

15. DERMOID AND TERATOID CYSTS Developmental anomalies composed of different cell layers. Dermoid cysts are composed of mesoderm and ectoderm and may contain hair follicles, sebaceous glands and sweat glands. Often midline or paramedian, do not elevate with tongue protrusion. Treatment- Simple excision. Teratoid cysts- Contain all 3 cell layers. Present within first year of life. Larger midline or paramedian masses than Dermoid cysts. Distinguished by cellular differentiation enough to have recognizable organs or structures.  Large size could cause aerodigestive compressive symptoms. 15

16. DERMOID CYST 16

17. LARYNGOCELES Lateral neck mass form congenitally as a result of enlargement of the laryngeal saccule with distension and entrapment of air.  Laryngoceles are classified as either internal, external, or combined. Internal laryngocele is -confined solely to the larynx and is caused from distension of the saccule, typically to the false vocal cord and aryepiglottic fold. Presents as hoarseness and respiratory distress. External laryngoceles will protrude through the thyrohyoid ligament at the entrance of the superior laryngeal nerve.  Presents as a soft, compressible, lateral neck mass that may distend with increases in intralaryngeal pressures.  If infected—larynopyoceles Combined type has features of both internal and external laryngoceles. Work up- CT scan. Treatment- If asymptomatic, no treatment needed. Symptomatic ones and larynopyoceles should be excised.

18. CONGENITAL MASSES VASCULAR AND LYMPHATIC MALFORMATIONS INFECTIOUS AND INFLAMMATORY LESIONS OTHER BENIGN LESIONS

19. HEMANGIOMAS The most common tumor of infancy. Typically present within the first few months of life. Demonstrate a rapid growth and then a period a quiescence followed by a period of involution. Work up- CT with contrast or MRI with gadolinium. Treatment- Parental reassurance and periodic photo documentation. Cavernous hemangiomas Larger and are associated with deeper tissue involvement. Less likely to spontaneously resolve.  Subglottic hemangiomas may be associated with cutaneous hemangiomas.  Perform airway evaluation if cutaneous vascular lesions associated with stridor. Hemangiomas causing complications-- high dose steroids can be used. Surgical excision if unresponsive – Subtotal or staged excision.

20. HEMANGIOMAS

21. LYMPHANGIOMAS Lymphatic cysts that are isolated from their normal route of drainage into the venous system. Classified into capillary, cavernous, and cystic types based on the size of the lymphatic spaces. Cystic hygromas Large, soft, painless, and compressible masses, Usually present by the age of 3.  Most commonly present in the posterior triangle. When they present in the anterior triangles, airway obstruction is more of a concern.  Work up - CT.  Spontaneous regression is rare.   Treatment of choice for obstructing lesions - Surgical excision. Recurrence rates are generally high because of the poor encapsulation and dissection planes.

22. CYSTIC HYGROMA

23. VASCULAR MALFORMATIONS Present during birth and grow proportionately with the child.  Progressive dilation of the venous structures.  Vascular malformations can cause significant bony distortion and destruction.  Port wine stain (capillary type vascular malformation) is the most common vascular malformation.  Arteriovenous malformations are clinically distinguishable from other head and neck masses as they are pulsatile on palpation and may be associated with an audible bruit. 

24. PORT WINE STAIN

25. CONGENITAL MASSES VASCULAR AND LYMPHATIC MALFORMATIONS INFECTIOUS AND INFLAMMATORY LESIONS OTHER BENIGN LESIONS

26. CERVICAL ADENITIS Infection and inflammation of the cervical lymph nodes is the most common cause of pediatric neck masses.  Palpable cervical lymph nodes are present in 40% of infants.  Cervical nodes that are asymptomatic and < 1 cm in diameter may be considered normal in children under 12.  Most common site of cervical adenitis is the submandibular or deep cervical nodes.  Lymphadenitis may be of viral, bacterial, fungal, parasitic, or noninfectious etiology.

27. CERVICAL ADENITIS Most common bacterial cause of cervical adenitis is Staphylococcus aureus and Group A Streptococci.  The cervical lymphadenopathy is usually tender . Associated with malaise and fever.  Majority of the cases respond within 4-6 weeks to a ten day course of a beta-lactamase resistant antibiotic.  If there is failure to respond, a fine needle aspiration is warranted to exclude other diagnoses.  Node suppuration may occur, and needle aspiration or I and D of the necrotic lymph node may be required. 

28. RETROPHARYNGEAL ABSCESS The parapharyngeal and retropharyngeal spaces are the most common neck spaces to be involved in pediatric neck abscesses.  Occurs in around 3-4 years of age. Usually polymicrobial --aerobic organisms (Staphylococcus, Streptococcus, Niesseria) anaerobes (Fusobacterium, Peptostreptococcus, and Bacteroides). Clinical manifestations- fever, decreased po, drooling, difficulty to move the neck. Muffled voice, stridor or respiratory distress.  Work up– CT scan with contrast  It allows for precise anatomical localization and relation to such structures as the major cervical vessels can be assessed . Treatment—IV antibiotics with/without surgical drainage (either intraoral or external).  Abscesses that are confined within a capsule and that do not extend posterior or lateral to the great vessels may be safely drained via a transoral approach. 

29. RETROPHARYNGEAL ABSCESS

30. LEMIERRE’S SYNDROME Septic thrombophlebitis of the internal jugular vein and metastatic abscesses in lungs.  Causative organism- Fusobacterium necrophorum. Was classically seen as a complication of inadequately treated tonsillitis, but may also be seen with neck abscesses.  Presentation with spiking fevers and fullness to one side of the neck. CXR- Multiple cavitary nodules, often B/L associated with pleural effusion. Treatment is with prolonged course of intravenous penicillin or cefoxitin.  Recurrent septic emboli occurrence may require surgical ligation or excision of the internal jugular vein.

31. TUBERCULOUS AND NON TUBERCULOUS MYCOBACTERIA NTB More common than TB in children. Mycobacterial infection - Single large cervical lymph node.  Later, the overlying skin may turn a characteristic violaceous color.  For tuberculous adenitis, a CXR is needed to rule out active pulmonary disease.  A PPD is usually strongly reactive.  Treatment with combinations of isoniazid, ethambutol, streptomycin, and rifampin.  NTM – Rarely exhibit fever or systemic symptoms.   CXR is usually normal. PPD reactions - normal or only intermediate in reactivity.  The treatment is less definitive with high resistance to traditional anti-tuberculous agents alone and requires a combination of typical and atypical agents.  Unless total surgical excision can be obtained, chronic draining sinuses often develop and persist until spontaneous resolution occurs.

32. VIRAL ADENITIS Viral adenitis is likely the most common infectious process in the neck.  Common pathogens include rhinovirus, adenovirus, and enterovirus.  Usually self-limited and are associated with upper respiratory symptoms.  Infectious Mononucleosis is caused by Ebstein Barr Virus (EBV).  Exudative, almost necrotic tonsillitis, impressive cervical lymphadenopathy and occassional hepatosplenomegaly.  Work up Heterophile antibodies (monospot) test may remain negative early in the disease, otherwise confirmatory.  IgM and IgG titers are most specific.  Treatment is usually supportive.  If adenotonsillitis is so severe that airway symptoms emerge, steroid and antibiotic therapy may be necessary.  Ampicillin and Amoxicillin have been associated with a rash in 90% of EBV patients and should be avoided.  CMV and HIV cervical lymphadenitis present with similar symptoms to EBV.  Treatments for the cervical lymphadenopathy are supportive as well.

33. CAT SCRATCH DISEASE Caused by Bartonella henselae.  Can be transmitted to humans by a Cat scratch.  Presents as self limited, unilateral, regional lymphadenopathy.  May have low grade fevers and malaise.  Work up Warthin-Starry stain-shows small pleomorphic gram-negative rods.  Cultures may take several weeks. Serologic testing of IgM and PCR of Bartonella DNA.  Symptoms usually resolve over several weeks to months, but in 10% of the patients, the nodes may progress to suppuration and require incision and drainage.  Antibiotic therapy not helpful but some anecdotal evidence has suggested benefit from macrolides and aminoglycosides. 

34. CONGENITAL MASSES VASCULAR AND LYMPHATIC MALFORMATIONS INFECTIOUS AND INFLAMMATORY LESIONS OTHER BENIGN LESIONS

35. PLUNGING RANULA Mucus retention cyst or a mucus extravasation pseudocyst arising from an obstructed sublingual gland. Simple ranula is confined to the oral cavity- cystic unilateral mass of the floor of the mouth.  Plunging ranula may pierce the mylohyoid and present as a paramedian or lateral neck mass with or without an obvious oral cavity ranula.  Work up Cyst aspiration - fluid with high levels of protein and salivary amylase.  CT or MRI - uniloculated cystic mass arising from the sublingual space with extension into the submental, submandibular, or parapharyngeal space.  Surgical treatment - excision in continuity with the sublingual gland of origin.

36. PLUNGING RANULA

37. STERNOMASTOID TUMOR OF INFANCY Fibrotic lesion of the distal sternocleidomastoid.  Present within the first 7 to 28 days of birth as a firm mass within the SCM.  Head tuck to the ipsilateral side and chin pointed away from the lesion may be present.  Possible association with breach delivery and forceps delivery.  Work up Clinical Ultrasound - solid mass that is intrinsic to the SCM.  Treatment - aggressive physical therapy and range of motion exercises (80-100% regress by the first birthday). Rarely an untreated case can lead to long term torticollis and resultant dysmorphosis.   Surgical division of the SCM is only recommended if physical therapy fails.

38. CONGENITAL MUSCULAR TORTICOLLIS

39. BACK TO OUR PATIENT Physiotherapy and massage. At 1 month of age with improving range of motion. At 5 months, swelling totally disappeared.

40. THANK YOU !! 40