Case Presentation

1
Case Presentation

• 2 year old boy c/o 3 weeks of left ear pain,
  fevers.
• Seen by pediatrician, started on amoxicillin, w/o
  improvement.
• Parents now concerned about area of swelling
  behind left ear, which pushes ear forward.
• No otorrhea.

2
Case Presentation

• No significant PMHx, PSHx, meds, allergies.
• Immunizations not UTD.
• Hearing assessment passed.

3
Case Presentation

• Awake, unkept child, irritable, febrile.
• Left ear auricle anteriorly pushed, 2-3 cm
  retroauricular mass above mastoid tip, firm,
  non-fluctuant, somewhat tender, well-defined
  borders, no overlying erythema.
• EAC impacted cerumen.
• Right ear wnl.
• CNs grossly intact, including VII B.
• Neck supple.
• Rest of exam wnl.

4
Case Presentation

• Awake, unkept child, irritable, febrile.
• Left ear auricle anteriorly pushed, 2-3 cm
  retroauricular mass above mastoid tip, firm,
  non-fluctuant, somewhat tender, well-defined
  borders, no overlying erythema.
• EAC impacted cerumen.
• Right ear wnl.
• CNs grossly intact, including VII B.
• Neck supple.
• Rest of exam wnl.

5
(No Transcript)
6
Differential Diagnosis andWork-up
7
HE
60x
8
S100
9
CD1A
10
Langerhans Cell Histiocytosis

• Unknown etiology.
• Rare 1-5/million/year.
• Idiopathic proliferation of dendritic histiocytes
  (Langerhans cells).
• Other names histiocytosis X, eosinophilic
  granuloma, Hand-Schuller-Christian dz,
  Letterer-Siwe Dz.
• MF.
• Risk factors solvents, lack of vaccinations,
  infections in neonatal period.

11
Langerhans Cell Histiocytosis

• Eosinophilic granuloma localized, solitary or
  multiple ulceration on the gingiva and palate,
  bone destruction, tooth loosening/loss.
• Hand-Schueller-Christian dz disseminated form,
  bone lesions, diabetes insipidus, exophtalmos.
• Letterer-Siwe dz disseminated form, oral
  ulcerations, ecchymosis, edema, gingivitis,
  periodontitis, jaw bone involvemente, tooth loss.
• Also skin rash, OM, lung/bone/lymph nodes.
• Hashimoto-Pritzker dz congenital form.

12

• Found in epidermis.
• Have Birbeck granules.

13
Langerhans Cell Histiocytosis

• No evidence of malignancy, but locally
  destructive.
• 60 of cases in children
• Can range from isolated mass to multi-system
  disease.
• Isolated good prognosis, sometimes self-limited
  (type 1).
• Multi-system chronic but indolent (types 2A and
  B).
• Disseminated pancytopenia, BM infiltration,
  fever, rash progressive and fatal (type 3).
• Up to 70 have HN dz.

14
(No Transcript)
15
Clinical Presentation

• Highly variable.
• Solitary bony/visceral involvement to diffuse
  disease w BM infliltration.
• HN frontal bone, temporal bone, skin rash in
  EAC and peri-auricular, maxilla/mandible, oral
  manifestations.

16
Clinical Presentation

• Otologic manifestations are common (13-61).
• There is usually delay in Dx.
• Usually treated as OE, OM, mastoiditis.
• Work-up should include CT, MRI, biopsy.
• Ear involvement has been associated with worse
  outcome.
• More difficult to cure?

17
Treatment

• Chemotherapy vinblastine, etoposide, prednisone,
  methotrexate, mercaptopurin, cyclosporin.
• Radiation therapy (for recalcitrant dz).
• Surgery (for localized lesions).
• Steroids.
• Treatment dictated by clinical presentation.
• Many reports of spontaneous resolution.
• Common complications diabetes insipidus,
  hypothyroidism, hearing loss, NLD obstruction,
  vertebral compression fractures.
• Current trial by the Histiocyte Society to
  establish guidelines of Tx.
• 70 disease-free outcomes in several series.

18
(No Transcript)
19
(No Transcript)
20
(No Transcript)
21
(No Transcript)