PAL #1: Lumps & Bumps

1. PAL #1: Lumps & Bumps By: Natasha Rupani Acknowledgement: Dr. HamadAlkhalaf

2. Objectives • To have some basic knowledge of neck anatomy re:position and drainage of lymph nodes, midline structures. • Develop a systematic approach to the evaluation and management of lymphadenopathy • Recognize worrisome features of lymphadenopathy • Be aware of congenitaletiologies for neck swellings • Recognize common infectious / inflammatory / malignant etiologies of neck swellings in children

3. Clinical Scenario A 3 year old boy is brought to his family doctor because the parents notice swellings in his neck. He has been previously well. For the last few days, he has had a fever, sore throat and general malaise. How would you approach this patient?

4. Context: Introduction • Lymph nodes, in conjunction with the spleen, tonsils, adenoids, thymus, and peyer’s patches, are highly organized centers of immune cells that filter antigen from the extra cellular fluid. • Body has 600 lymph nodes.

5. Introduction (2) • Examining the lymph nodes is an important aspect of the general physical examination of any pediatric patient • Lymph nodes are normal structures and certain lymph nodes may be palpable in healthy children • Because of its association with malignancy, lymphadenopathy can be a major source of parental anxiety.

6. Anatomy of Lymph Node

7. Anatomy of a Lymph Node (2) • Lymph (ultra filtrate of the blood) is collected in lymphatic capillaries present throughout the body except the brain and heart. • Lymph moves slowly under low pressure unidirectionally and ultimately drains into either: • Right lymphatic duct (lymph from pt upper R) • Thoracic duct (rest of the body) • These ducts ultimately drain into the venous system (R and L subclavian veins)

9. Anatomy of the Lymph Node (3) A working knowledge of the nodal basins and the anatomy of the regions they drain is helpful in formulating a differential diagnosis for lymphadenopathy

11. DRAINAGE PATTERNS Ear, scalp Scalp, skin Scalp Scalp, parotid, ear Tonsils teeth Scalp, neck, upper thoracic skin teeth Anterior cervical Larynx, tongue, oropharynx, anterior neck, thyrpid (GI, GU, pulmonary, breast)

13. Pathogenesis Lymph node enlargement can be caused by the following mechanisms: 1. Proliferation of normal cells that comprise the lymph node (in response to antigen stimuli) a. benign hyperplasia b. vascular engorgement and edema secondary to local cytokine release c. suppuration secondary to tissue necrosis

14. Pathogenesis (2) 2. Entry of large number of cells exogenous to the node like: a. neutrophils b. metastatic neoplastic cells 3. Deposition of foreign materials like lipid storage disease

15. Epidemiology • In one review, lymphadenopathy present in: 44% well child visits 64% sick visits (in children < 5 years of age ) Herzog LW, Prevalence of lymphadenopathy of the head and neck in infants and children. ClinPediatr (Phila) 1983; 22:485 • Prevalence of malignancy among the patients seen in primary care setting is relatively rare.

16. Epidemiology (2) • In contrast, prevalence of malignancy in lymphadenopathy biopsies performed in pediatric referred centers ranges from 13 – 27% Lake AM, Oski FA. Peripheral lymphadenopathy in childhood. Ten-year experience with excisional biopsy. Am J Dis Child 1978; 132:357 Soldes OS, Younger JG, Hirschl RB. Predictors of malignancy in childhood peripheral lymphadenopathy. J OediatrSurg 1999; 34:1447

17. Epidemiology (3) • In the largest series studies, 239 children underwent peripheral lymph node biopsies for evaluation of lymphadenopathy • The following etiologies were noted 1. Idiopathic reactive hyperplasia (52%) 2.Granulmatousdisease (cat scratch, atypical mycobacterium, TB, fungal, histocytosis in (33%) 3. Malignancy (13%), 2/3 of them has Hodgkins disease 4. Chronic dermatopathic or bacterial infections (3%)

18. Epidemiology (4) • The prevalence of lymphadenopathy varies with age and site • Small occipital and post auricular nodes, for example, are common in infants, but not in older children • In contrast, cervical and inguinal nodes are more common after 2 yrs of age • Epitrochlear and supraclavicularadenopathy are uncommon at any age

19. Epidemiology (5) • In neonates, lymph nodes are barely palpable • M = F • Race is not a factor in most lymphadenopathy • Uncommon causes of lymphadenopathy should be considered in certain areas • e.g. HIV in Africa, TB and other tropical diseases in developing nations

20. Important definitions • Lymphadenopathy Pathologic swollen lymph node (regardless of the cause) • Lymphadenitis Inflamed lymph node usually by infectious cause

21. Important definitions (2) • Localized lymphadenopathy Abnormal enlargement of one (or two contiguous) LNs • Generalized lymphadenopathy Abnormal enlargement of two or more noncontiguous LNs

22. Important definitions (3) • Acute lymphadenopathy < 2 weeks duration • Subacute lymphadenopathy 2 – 6 weeks duration • Chronic lymphadenopathy > 6 weeks duration

23. First evaluation… Stepwise approach: • History • Physical Exam • +/- appropriate investigations for selected patients requiring further work-up

24. History • HPI: • Duration and location of lymphadenopathy • Local symptoms • cough, pharyngitis, dental problems, recent onset of fever, contact with sick pts, skin lesions • Associated constitutional symptoms • prolonged fever, wt loss, night sweats, skin rash, bone or joint symptoms

25. History (2) • Exposure hx (food, animal, travel) a. Ingestion of unpasteurized animal milk, uncooked meats (brucella,TB) b. Animal contact: cats (cat scratch disease & toxoplasmosis), goats (brucellosis), rabbits (tularemia), prairie dogs (bubonic plague), tick bites, flea, and mosquito bites (lyme disease, bubonic plague, tularemia) c. Travel hx(tularemia, TB, measles, rubella, leishmaniasis, typhoid fever)

27. History (3) • Past Medical History • Recurrent infections, skin abscess, supporative adenitis (CGD, HIV) • Autoimmune disease (autoimmune lymphoproliferative syndrome) • Asthma (Churg–Strauss syndrome- mediastinal LAD) • General health: previous hospitalizations, surgeries, visits to ED

28. History (4) • Immunization status - diphteria, measles, rubella • Medications - Amoxicillin rash in EBV - Recent steroid therapy • IV drug use, sexual history (in adolescents)

29. History (5) Drugs that cause lymphadenopathy

30. History (6) • Family hx malignancy, autoimmune, inflammatory, storage diseases • Social hx recent immigration

31. Physical Exam General appearance: A: appearance B: basics (i.e. vitals) C: colour D: dysmorphic features E: equipment Growth parameters (wt loss of > 10% is a red flag)

32. Physical Exam (2) Head and Neck: • Scalp infection • seborrheicdermatitis, tineacapitus • Conjunctivitis injection • oculoglandular syndrome, Kawasaki disease • Nasal obstruction • rhabdomyosarcoma, nasopharyngeal carcioma, URTI • Oropharynx • pharyngitis, dental problems, HSV ginivostomatitis • Ears • otitis media • Neck • ROM, other LN involvement, transillumination

33. Physical Exam (3) Abdomen: • Hepatosplenomegaly (*part of lymph node exam) • Abdominal masses (e.g. neuroblastoma) Skin: • Any rashes • Petechiae, purpura, ecchymoses (e.g. thrombocytopenia)

34. Lymph Node Exam: • Size (measure them), Location, Fixation, Consistency, Tenderness

35. Exam all lymph nodes: - pre-auricular • post-auricular • tonsillar • submental • submandibular • anterior cervical • posterior cervical • occipitial • supraclavicular • axillary • epitrochlear (antecubitalfossa) • inguinal, popliteal

36. Special Considerations Formulating your DDx requires consideration of several important clinical features: • Age of the patient • Size of the nodes • Location of the nodes • Quality of the nodes • Localized vs. Generalized • Time course • Worrisome features

37. 1. Patient Age • Normal size of lymph nodes vary with age • Rarely palpable in newborns • “Shotty” lymphadenopathy: small mobile benign lymph nodes common in young children 3 to 5 years, seen frequently in setting of viral illness.

38. Cont’d • Differential dx changes with age: • Eg: Hodgkin lymphoma in adolescents and adults (rare before age 10) • Eg: Sexually transmitted infections common cause of inguinal lymphadenopathy in late adolescent/ adulthood • Eg: URTI infections, otitis, conjunctivitis frequently lead to chronic reactive cervical lymphadenopathy in preschoolers and school age

39. 2. Size of Lymph Nodes Rule of thumb: Cervical/ Axillary: up to 1cm Inguinal: up to 1.5cm Epitrochlear: up to 0.5cm Size limits differ somewhat with age- less stringent in young children than adolescents/adults because of frequent antigenic exposure in early childhood illnesses and the gradual acquisition of Abs and immunity. Clinical Pearl: Increasing suspicion for malignancy with nodes >2cm

40. 3. Location of Lymph Nodes Refer back to Table 1: location can direct source of infection based on drainage patterns. Eg. Anterior cervical lymphadenopathy related to pharyngitis. Eg. Enlarged axillary node in the setting of catch-scratch disease from scratch on arm. Clinical Pearl: Palpable lymph node in the supraclavicularfossa is worriesome and should prompt a thorough evaluation for a cause…

41. 4. Quality of Lymph Nodes • Tender nodes likely infection, especially if associated with erythema, warmth, induration, or fluctuance. • Occasionally malignancy can cause node tenderness because of hemorrhage into the node and subsequent stretching of the capsule. • Nodes that are soft, easily compressible, and freely mobile are benign.

42. Cont’d • Hard nodes found in cancers are from fibrosis post inflammation. • Lymphomatousnodes are firm and rubbery. • Fixed or matted nodes usually from invasive cancers or from inflammation surrounding nodes as in tuberculosis or sarcoidosis.

43. 5. Localized vs. Generalized Localized: single area • most common in cervical and inguinal regions occurring from lymphadenitis (infection of the node itself) or from an infection in its drainage area • common presentation in primary care

47. Cont’d Generalized: 2+ nodal groups involved • usually systemic disease • associated hepatosplenomegaly or rash

49. Causes of generalized lymphadenopathy

50. Causes of generalized lymphadenopathy