PROPTOSIS            children    Dr. Yousaf Jamal      FCPS Resident   Ophthalmology UnitHayatabad Medical Complex       0...
CONTENTS• Recap• Causes in children• Differentiating features• Common causes discussion• Summary• Take home message• MCQs ...
Recap• Causes of Proptosis• Common causes in adults• Examination of proptosis Page 3        Proptosis in Children   Mar 25...
CONTENTS• Recap• Causes in children• Differentiating features• Common causes discussion• Summary• Take home message• MCQs ...
Causes in children• Orbital cellulitis        • Retinoblastoma• Dermoid cyst              • Leukemia• Capillary           ...
CONTENTS• Recap• Causes in children• Differentiating features• Common causes discussion• Summary• Take home message• MCQs ...
CAPILLARY                 ORBITAL LYMPHANGIOMA               HEMANGIOMAPathology      Vascular hemartoma        Isolated v...
ON GLIOMA                               ON MENINGIOMAPathology      Fusiform enlargement of ON, origin -    Tubular enlarg...
RHABDOMYOSARCOM                        ORBITAL CELLULITIS               APathology      Undifferentiated mesenchymal      ...
CONTENTS• Recap• Causes in children• Differentiating features• Common causes discussion• Summary• Take home message• MCQs ...
ORBITAL CELLULITIS• Bacterial orbital cellulitis – life-threatening• Infection of soft tissues behind septum• More common ...
Etiology• Sinus-related                • Post-trauma   – Ethmoidal (90%)                – Penetrating septum (48-• Extensi...
Presentation…• Malaise• Fever• Pain• Visual impairment• Tender, warm, red periorbital edema• Proptosis Page 13       Propt...
… Presentation• Painful Ophthalmoplegia• Chemosis• Headache• Rhinorrhea• Elevated IOP Page 14         Proptosis in Childre...
Courtesy: MedlinePage 15   Proptosis in Children   Mar 25, 2013
Courtesy: MedlinePage 16   Proptosis in Children   Mar 25, 2013
Work-up• Hx• Examination• Vital signs record• WBC/blood culture• Lumbar puncture• CT orbit• MRI  – For cavernous sinus thr...
Complications• Ocular                  • Intracranial  – Exposure                 – Meningitis (2%)    keratopathy        ...
Treatment                   MEDICAL…• Hospital admission + ENT consultation• I.V. antibiotics  –   Ceftazidime, Cefotaxime...
• Metronidazole• Nasal decongestant  – Phenylephrine  – Oxymetazoline• Antibiotic eye ointment Page 20        Proptosis in...
SURGICAL…• Indications   – Decreasing vision   – Afferent pupillary defect   – No response to antibiotics in 48-72 hrs   –...
• Canthotomy/Cantholysis in emergency• Surgical drainage of fluid• Orbital surgery + sinusotomy• For fungal   – Debridemen...
Doses• Ceftazidime (Fortum® )  – Adults=1-2g/8hr I.V.  – Pediatric     • Neonates=30mg/kg IV     • 1 month-12 years=30-50m...
• Cefotaxime (Claforan® )  – Adults=1-2g/10ml/4hr IV in 3-5 min (max=12g/day)  – Pediatric      • 0-1 week=50mg/kg/12hr IV...
• Vancomycin (Vancocin® )  – Adults=1g/12hr IV (over 1 hour with rate <    10mg/min at concentration <5mg/ml)  – Pediatric...
• Ampicillin-Sulbactam (Unasyn® )  – Adults=3g/6hr IV  – Children=300mg/kg/day 4 divided doses• Piperacillin-Tazobactam (Z...
Tx combinations• Metronidazole to all• Vancomycin + Ceftriaxone (3rd gen ceph)  – Allergy to penicillin• Fluoroquinolone  ...
Follow-up• Daily monitor  – Symptoms  – Temperature  – Visual acuity  – Ocular motility  – Proptosis  – Cornea  – IOP  – R...
• Change to oral to complete 14 days course if  – Afebrile for 48 hours• Oral Drugs  – Amoxicillin-clavulanate (Augmentin®...
DERMOID CYST• A choristoma, found adjacent to suture lines• Connected to periorbita by fibrovascular  tissue• Contain…  – ...
Presentation…• Mostly in children• Mostly…Superotemporal aspect of orbit• A painless mass• Slowly growing• Usually < 1 cm ...
… Presentation• Globe displacement• Inflammation if ruptures• May  – Compress optic nerve  – Diplopia Page 32        Propt...
Courtesy: MedlinePage 33   Proptosis in Children    Mar 25, 2013
Page 34   Proptosis in Children   Mar 25, 2013
Courtesy: MedlinePage 35   Proptosis in Children     Mar 25, 2013
Work-up                      CT/MRI – Indicated if posterior extent not palpated – Cystic appearance – Well circumscribed ...
Courtesy: eyetext.orgPage 37   Proptosis in Children     Mar 25, 2013
Courtesy: eyetext.orgPage 38   Proptosis in Children        Mar 25, 2013
Treatment                     Surgical• Excision in Toto Page 39       Proptosis in Children   Mar 25, 2013
CAPILLARY HEMANGIOMA• Most common orbital tumor of infancy• Benign endothelial cells neoplasms• Absent usually at birth• R...
Pathophysiology• Thought … of placental origin• 02 phases of growth   – Proliferative            • Rapid growth…8-18 month...
Mortality/Morbidity• Kasabach-Merritt syndrome  – Coagulopathy, thrombocytopenia  – large visceral / Nasopharyngeal hemang...
Presentation• F:M…3:1• 1-2% in neonates• All develop by 6 months• Typically…red spot growing in size in  Periorbital area•...
Classified as…  1.   Cutaneous  2.   Purely preseptal  3.   Preseptal with extraconal element  4.   Combination of presept...
Signs• Usually…superonasal, brow or lid lesion• Typically blanches with pressure• Ptosis & proptosis if posterior extensio...
Page 46   Proptosis in Children   Mar 25, 2013
Work-up                       CT – poorly circumscribed mass with no bony erosion – Homogenous enhancing lesion           ...
Treatment                       Medical• Observation• Steroids  – Clobetasol propionate cream  – Injectable steroid formul...
Surgery• Laser surgery   – carbon dioxide laser   – Argon laser   – Nd:YAG laser• Primary excision Page 49          Propto...
OPTIC NERVE GLIOMA• Most common primary tumor of ON  – In children…mostly benign  – In adults…Aggressive glioma• WHO class...
• 10-38% of pediatric pts with ON glioma have  NF-1• Bilateral ON gliomas…almost pathognomonic  for NF-1  Listernick R, Ch...
Presentation…• ON gliomas are…*  – 4% of orbital tumors  – 4% of intracranial gliomas  – 2% of intracranial tumors  – 2/3r...
… Presentation…•       F>M•       In pediatrics      –       Median age…5 yrs      –       80% pts present before age 15• ...
… Presentation• Painless proptosis with inferior dystopia• Decreased vision• Nystagmus• Changes in appetite or sleep• Larg...
Page 55   Proptosis in Children   Mar 25, 2013
Work-up                      CT• Diffuse Fusiform enlargement of the optic  nerve                      MRI• T1-weighted im...
Page 57   Proptosis in Children   Mar 25, 2013
Treatment• Not required if  – Not growing  – Good vision  – No cosmetic blemish• Surgical excision  – Large & growing lesi...
EMBRYONAL SARCOMA• Most common primary orbital malignancy of  childhood• Origin…undifferentiated mesenchymal cells• Called...
Presentation…• Usually first decade (average…7yrs)• Boys > girls• Proptosis  – Rapid (days to weeks)  – Unilateral  – Pain...
… Presentation…• Swollen lids but bluish appearance• Ptosis• Location   – Superonasal   – Retro bulbar   – Superior   – In...
… Presentation• Other findings  – Disc edema  – Choroidal folds  – Decreased vision  – Cervical lymph nodes involvement Pa...
Courtesy: medlinePage 63   Proptosis in Children     Mar 25, 2013
Courtesy: medlinePage 64   Proptosis in Children   Mar 25, 2013
Page 65   Proptosis in Children   Mar 25, 2013
Work-up                         USG•   Relatively well-circumscribed mass•   Low-medium amplitude echoes                  ...
Treatment• Referred to pediatric oncologist• Radiotherapy• Chemotherapy• Surgery  – Exenteration for recurrent & resistant...
LYMPHANGIOMA• Introduction   – Non-Neoplastic   – Non-functional   – Abortive   – Vascular malformation• Hemodynamically i...
Presentation…• Early childhood (average=5-6 yrs)• Multiple bluish soft masses• Upper nasal quadrant• Cystic conjunctival c...
… Presentation• Worsened by RTI• Palatal involvement• Ptosis• Restricted eye movements• Optic nerve compression• Papillede...
Page 71   Proptosis in Children   Mar 25, 2013
Page 72   Proptosis in Children   Mar 25, 2013
Work-up                        CT• Multilobulated cystic mass within the orbit  and/or intralesional calcifications       ...
Page 74   Proptosis in Children   Mar 25, 2013
Treatment• Conservative             • Intralesional• Partial surgical            injection of  resection of the           ...
• Sodium tetradecyl sulfate (Sotradecol®)•   An anionic surface-active agent used for its wetting properties in    industr...
• OK-432 (Picibanil®)•   lyophilized preparation of a low-virulence strain of S. pyogenes    (S. hemolyticus), inactivated...
CONTENTS• Recap• Causes in children• Differentiating features• Common causes discussion• Summary• Take home message• MCQs ...
Page 79   Proptosis in Children   Mar 25, 2013
PROPTOSISLid edema                              Little/no inflammationSinus disease                          Orbital lesio...
PROPTOSIS Child (5 yrs)                                          Child (7-8yrs) Upper nasal quadrant mass                 ...
CONTENTS• Recap• Causes in children• Differentiating features• Common causes discussion• Summary• Take home message• MCQs ...
TAKE HOME MESSAGE• Proptosis in children may be  – Benign as … Dermoid  – Malignant as … Rhabdomyosarcoma• Thorough Hx & c...
Page 84   Proptosis in Children   Mar 25, 2013
MCQs1. A 1-month old infant presents with a   purplish, spongy eyelid mass. Most likely   diagnosis:a.     Rhabdomyosarcom...
2. In the evaluation of child with unilateral     Proptosis, which assumption is true?a.     Cavernous hemangioma is the m...
3. The management of Rhabdomyosarcoma of    the orbit usually involvesa.     Lumbar puncture to rule out CNS Metsb.     Ex...
4. All of the following are true regarding optic    nerve tumors except:a.     ON gliomas in children are associated with ...
FOR MCQs 5-85. An 11-year old girl presents with acute,    unilateral, left-sided periocular pain,    Proptosis and double...
…Continued…6. 24 hrs later & without any treatment, the pain    has resolved. Periocular ecchymosis    develops, & the dou...
…Continued…7. If the patient was losing vision bcz of this     process, you would consider:a.     Intravenous antibioticsb...
…Continued8. The disease process is an example of :a.     The most common cause of proptosis in childrenb.     The most co...
9. Which one of the following is the most    common primary malignancy of the orbit in    children?a.     Neuroblastomab. ...
10. what is the most common cause of  unilateral childhood proptosis?A. capillary hemangiomaB. Thyroid related orbitopathy...
11. In orbital cellulitis, which of the following is  false:a. Common cause of proptosis in childrenb. Seen due to infecti...
12. Most common organism causing orbital    cellulitis in children < 5 yrs:a.     Pneumococcib.     Staph aureusc.     Str...
13. ON glioma includes following:a.     Usually seen in 1st decade of lifeb.     Can be seen either in the intraorbital or...
14. A young child presents with sudden loss of vision &    proptosis of right eye. On examination, the direct    pupillary...
NEXT                  Lecture• Dr. Gulzada  – Red eyes                Journal club• Dr. Shafqat                           ...
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Proptosis in children

  1. 1. PROPTOSIS children Dr. Yousaf Jamal FCPS Resident Ophthalmology UnitHayatabad Medical Complex 06-03-2010 Mar 25, 2013
  2. 2. CONTENTS• Recap• Causes in children• Differentiating features• Common causes discussion• Summary• Take home message• MCQs Page 2 Proptosis in Children Mar 25, 2013
  3. 3. Recap• Causes of Proptosis• Common causes in adults• Examination of proptosis Page 3 Proptosis in Children Mar 25, 2013
  4. 4. CONTENTS• Recap• Causes in children• Differentiating features• Common causes discussion• Summary• Take home message• MCQs Page 4 Proptosis in Children Mar 25, 2013
  5. 5. Causes in children• Orbital cellulitis • Retinoblastoma• Dermoid cyst • Leukemia• Capillary • Lymphangioma hemangioma • Metastasis• Optic nerve glioma – Metastatic• Rhabdomyosarcoma neuroblastoma – Ewings sarcoma Page 5 Proptosis in Children Mar 25, 2013
  6. 6. CONTENTS• Recap• Causes in children• Differentiating features• Common causes discussion• Summary• Take home message• MCQs Page 6 Proptosis in Children Mar 25, 2013
  7. 7. CAPILLARY ORBITAL LYMPHANGIOMA HEMANGIOMAPathology Vascular hemartoma Isolated vascular hemartomaDemographics Infant, commonest Early childhood benign orbital tumor in childhood, spontaneous involutionAssociated Visceral, - nasopharyngeal hemangioma, kasabach-merit syndromePresentation Nonaxial, +ve Valsalva, Nonaxial, -ve Valsalva, acute episodes of superficial/deep spontaneous hemorrhage, may be superficial/deepRadiology Intra/extraconal mass, Low density cyst-like mass, enlargement of poorly defined bony orbitTx Observation, surgery Guarded prognosis, surgery, drainage Page 7 Proptosis in Children Mar 25, 2013
  8. 8. ON GLIOMA ON MENINGIOMAPathology Fusiform enlargement of ON, origin - Tubular enlargement of ON, origin- glial tissues, meninges show reactive meninges, psammoma bodies hyperplasia, dura is normalDemographic Young girls (2-6yrs) Late middle aged womansAssociated NF-1 (20-40%) -Presentation Axial, VA decreases early, EOM Axial, gaze evoked amaurosis, normal, optociliary shunts - uncommon EOM impaired, optociliary shuntsRadiology Fusiform enlargement, isodense to Tubular enlargement, hyperdense, bone ON sheath enhancementTx Conservative, surgical, radiotherapy Conservative, radiotherapy, surgery Page 8 Proptosis in Children Mar 25, 2013
  9. 9. RHABDOMYOSARCOM ORBITAL CELLULITIS APathology Undifferentiated mesenchymal Infection of soft tissues behind the cells, most common primary orbital septum malignancy of childhoodDemographics Boys > girls (avg 7 yrs), Commonest cause of proptosis in children, adults also affectedAssociated Cervical L. nodes involved Usually after trauma, sinus relatedPresentation Nonaxial, painful, unilateral, rapid Painful, lids edema with reddish onset, swollen bluish lids, nose color, Ptosis, malaise, fever, life bleed, Ptosis, chemosis threatening, VA impaired, Ophthalmoplegia,Radiology Moderately well-defined CT-(orbit, sinuses, brain): diffuse homogenous mass, Bony orbital infiltrate, sinus opacity, destruction proptosis MRI for cavernous sinus thrombosis.Tx Radiotherapy, chemotherapy, Medical, surgical Exenteration for resistant cases Page 9 Proptosis in Children Mar 25, 2013
  10. 10. CONTENTS• Recap• Causes in children• Differentiating features• Common causes discussion• Summary• Take home message• MCQs Page 10 Proptosis in Children Mar 25, 2013
  11. 11. ORBITAL CELLULITIS• Bacterial orbital cellulitis – life-threatening• Infection of soft tissues behind septum• More common in children• Organisms – S. pneumoniae – S. aureus – S. pyogenes – H. influenzae Page 11 Proptosis in Children Mar 25, 2013
  12. 12. Etiology• Sinus-related • Post-trauma – Ethmoidal (90%) – Penetrating septum (48-• Extension of preseptal 72 hrs) cellulitis – Retained foreign body• Local spread • Post-surgical – Lacrimal – Dacryocystitis – Orbital – Facial cellulitis – Retinal – Dental infections• Systemic bacteremia Page 12 Proptosis in Children Mar 25, 2013
  13. 13. Presentation…• Malaise• Fever• Pain• Visual impairment• Tender, warm, red periorbital edema• Proptosis Page 13 Proptosis in Children Mar 25, 2013
  14. 14. … Presentation• Painful Ophthalmoplegia• Chemosis• Headache• Rhinorrhea• Elevated IOP Page 14 Proptosis in Children Mar 25, 2013
  15. 15. Courtesy: MedlinePage 15 Proptosis in Children Mar 25, 2013
  16. 16. Courtesy: MedlinePage 16 Proptosis in Children Mar 25, 2013
  17. 17. Work-up• Hx• Examination• Vital signs record• WBC/blood culture• Lumbar puncture• CT orbit• MRI – For cavernous sinus thrombosis Page 17 Proptosis in Children Mar 25, 2013
  18. 18. Complications• Ocular • Intracranial – Exposure – Meningitis (2%) keratopathy – Brain abscess – Raised IOP – Cavernous sinus – CRVO,CRAO thrombosis (1%) – Endopthalmitis • Orbital abscess – Optic neuropathy • Subperiosteal abscess Page 18 Proptosis in Children Mar 25, 2013
  19. 19. Treatment MEDICAL…• Hospital admission + ENT consultation• I.V. antibiotics – Ceftazidime, Cefotaxime, Ceftriaxone – Flucloxacillin – Vancomycin – Ampicillin-Sulbactam – Piperacillin-Tazobactam – Ciprofloxacin/Levofloxacin Page 19 Proptosis in Children Mar 25, 2013
  20. 20. • Metronidazole• Nasal decongestant – Phenylephrine – Oxymetazoline• Antibiotic eye ointment Page 20 Proptosis in Children Mar 25, 2013
  21. 21. SURGICAL…• Indications – Decreasing vision – Afferent pupillary defect – No response to antibiotics in 48-72 hrs – Orbital/Subperiosteal abscess – Atypical picture…..Needs biopsy Page 21 Proptosis in Children Mar 25, 2013
  22. 22. • Canthotomy/Cantholysis in emergency• Surgical drainage of fluid• Orbital surgery + sinusotomy• For fungal – Debridement – Exenteration Page 22 Proptosis in Children Mar 25, 2013
  23. 23. Doses• Ceftazidime (Fortum® ) – Adults=1-2g/8hr I.V. – Pediatric • Neonates=30mg/kg IV • 1 month-12 years=30-50mg/kg IV• Ceftriaxone (Rocephin® ) – Adults=2g/12hr IV – Pediatric • 20-50mg/kg/day IV Page 23 Proptosis in Children Mar 25, 2013
  24. 24. • Cefotaxime (Claforan® ) – Adults=1-2g/10ml/4hr IV in 3-5 min (max=12g/day) – Pediatric • 0-1 week=50mg/kg/12hr IV • 1-4weeks=50mg/kg/8hr IV • 1month-12years=50-80mg/kg/day IV in 4-6 doses Page 24 Proptosis in Children Mar 25, 2013
  25. 25. • Vancomycin (Vancocin® ) – Adults=1g/12hr IV (over 1 hour with rate < 10mg/min at concentration <5mg/ml) – Pediatric • Infants & neonates=15mg/kg loading• Ciprofloxacin – In > 17 years age=400mg/12hr IV• Levofloxacin – In > 17 years age=750mg/6hr IV Page 25 Proptosis in Children Mar 25, 2013
  26. 26. • Ampicillin-Sulbactam (Unasyn® ) – Adults=3g/6hr IV – Children=300mg/kg/day 4 divided doses• Piperacillin-Tazobactam (Zosyn® ) – Adults=4.5g/8hr IV – Children=240mg Piperacillin component/kg/day 3 divided doses• Metronidazole (Flagyl® ) – 500mg/6-8hr IV Page 26 Proptosis in Children Mar 25, 2013
  27. 27. Tx combinations• Metronidazole to all• Vancomycin + Ceftriaxone (3rd gen ceph) – Allergy to penicillin• Fluoroquinolone – Allergy to penicillin + cephalosporins• Vancomycin + Ampicillin-Sulbactam or Piperacillin-Tazobactam – For MRSA Page 27 Proptosis in Children Mar 25, 2013
  28. 28. Follow-up• Daily monitor – Symptoms – Temperature – Visual acuity – Ocular motility – Proptosis – Cornea – IOP – Retina and optic nerve Page 28 Proptosis in Children Mar 25, 2013
  29. 29. • Change to oral to complete 14 days course if – Afebrile for 48 hours• Oral Drugs – Amoxicillin-clavulanate (Augmentin® ) • Adults=500mg TDS • Children=20-40mg/kg/day 3 divided doses – Cefaclor (Ceclor® ) • Adults=500mg TDS • Children=20-40mg/kg/day 3 divided doses – Metronidazole Page 29 Proptosis in Children Mar 25, 2013
  30. 30. DERMOID CYST• A choristoma, found adjacent to suture lines• Connected to periorbita by fibrovascular tissue• Contain… – Keratinized stratified squamous epithelium – Blood vessels, fat, collagen, sebaceous glands, and hair follicles – a tan oily liquid to a white or yellow substance Page 30 Proptosis in Children Mar 25, 2013
  31. 31. Presentation…• Mostly in children• Mostly…Superotemporal aspect of orbit• A painless mass• Slowly growing• Usually < 1 cm in diameter, non-tender Page 31 Proptosis in Children Mar 25, 2013
  32. 32. … Presentation• Globe displacement• Inflammation if ruptures• May – Compress optic nerve – Diplopia Page 32 Proptosis in Children Mar 25, 2013
  33. 33. Courtesy: MedlinePage 33 Proptosis in Children Mar 25, 2013
  34. 34. Page 34 Proptosis in Children Mar 25, 2013
  35. 35. Courtesy: MedlinePage 35 Proptosis in Children Mar 25, 2013
  36. 36. Work-up CT/MRI – Indicated if posterior extent not palpated – Cystic appearance – Well circumscribed lesionPage 36 Proptosis in Children Mar 25, 2013
  37. 37. Courtesy: eyetext.orgPage 37 Proptosis in Children Mar 25, 2013
  38. 38. Courtesy: eyetext.orgPage 38 Proptosis in Children Mar 25, 2013
  39. 39. Treatment Surgical• Excision in Toto Page 39 Proptosis in Children Mar 25, 2013
  40. 40. CAPILLARY HEMANGIOMA• Most common orbital tumor of infancy• Benign endothelial cells neoplasms• Absent usually at birth• Rapid growth in infancy• Involution later on Page 40 Proptosis in Children Mar 25, 2013
  41. 41. Pathophysiology• Thought … of placental origin• 02 phases of growth – Proliferative • Rapid growth…8-18 months • Increase in no. of endothelial & mast cells (stimulus for vessel growth) – Involutional • 30-50% involute by 5 yrs • 75% by 7 yrs Kavanagh EC, Heran MK, Peleg A, et al. Imaging of the natural history of an orbital capillary hemangioma. Orbit. Mar 2006;25(1):69-72 Page 41 Proptosis in Children Mar 25, 2013
  42. 42. Mortality/Morbidity• Kasabach-Merritt syndrome – Coagulopathy, thrombocytopenia – large visceral / Nasopharyngeal hemangiomas – DIC may occur, high output CCF – Mortality…30-50%• Ophthalmic morbidity – Space occupying – Amblyopia Page 42 Proptosis in Children Mar 25, 2013
  43. 43. Presentation• F:M…3:1• 1-2% in neonates• All develop by 6 months• Typically…red spot growing in size in Periorbital area• Inability to open lids• Bulging of the eye Page 43 Proptosis in Children Mar 25, 2013
  44. 44. Classified as… 1. Cutaneous 2. Purely preseptal 3. Preseptal with extraconal element 4. Combination of preseptal, extraconal and intraconal Page 44 Proptosis in Children Mar 25, 2013
  45. 45. Signs• Usually…superonasal, brow or lid lesion• Typically blanches with pressure• Ptosis & proptosis if posterior extension• Visual loss• Preseptal…Dark blue or purple• Enlarge & change color while crying Page 45 Proptosis in Children Mar 25, 2013
  46. 46. Page 46 Proptosis in Children Mar 25, 2013
  47. 47. Work-up CT – poorly circumscribed mass with no bony erosion – Homogenous enhancing lesion MRI – hypointense to fat on T1-weighted & hyperintense on T2-weighted scans US & Doppler – For anatomic relations & extentPage 47 Proptosis in Children Mar 25, 2013
  48. 48. Treatment Medical• Observation• Steroids – Clobetasol propionate cream – Injectable steroid formulations – Systemic corticosteroids are used for amblyogenic lesions• Interferon alfa-2a – Resistant to steroid treatment Page 48 Proptosis in Children Mar 25, 2013
  49. 49. Surgery• Laser surgery – carbon dioxide laser – Argon laser – Nd:YAG laser• Primary excision Page 49 Proptosis in Children Mar 25, 2013
  50. 50. OPTIC NERVE GLIOMA• Most common primary tumor of ON – In children…mostly benign – In adults…Aggressive glioma• WHO classify it as – Grade I astrocytomas (pilocytic astrocytomas) bcz slow growing & tend not to metastasize• Generalized hyperplasia of glial cells in the nerve to complete disorganization Page 50 Proptosis in Children Mar 25, 2013
  51. 51. • 10-38% of pediatric pts with ON glioma have NF-1• Bilateral ON gliomas…almost pathognomonic for NF-1 Listernick R, Charrow J, Greenwald MJ, et al. Optic gliomas in children with neurofibromatosis type 1.  J Pediatr. May 1989;114(5):788-92.  Page 51 Proptosis in Children Mar 25, 2013
  52. 52. Presentation…• ON gliomas are…* – 4% of orbital tumors – 4% of intracranial gliomas – 2% of intracranial tumors – 2/3rd of all primary optic nerve tumors• 20% gliomas extend to the optic chiasm or beyond into the optic radiations & aggressive* Hollander MD, FitzPatrick M, OConnor SG, et al. Optic gliomas. Radiol Clin North Am. Jan 1999;37(1):59-71. Page 52 Proptosis in Children Mar 25, 2013
  53. 53. … Presentation…• F>M• In pediatrics – Median age…5 yrs – 80% pts present before age 15• In adults – 22-79 yrs with a mean age of 52 yrsHollander MD, FitzPatrick M, OConnor SG, et al. Optic gliomas.  Radiol Clin North Am. Jan 1999;37(1):59-71.Millar WS, Tartaglino LM, Sergott RC, et al. MR of malignant optic glioma of adulthood.  AJNR Am J Neuroradiol. Sep 1995;16(8):1673-6.  Page 53 Proptosis in Children Mar 25, 2013
  54. 54. … Presentation• Painless proptosis with inferior dystopia• Decreased vision• Nystagmus• Changes in appetite or sleep• Large lesion can obstruct 3rd ventricle & ICP• Optic atrophy• Opticociliary collaterals• CRVO Page 54 Proptosis in Children Mar 25, 2013
  55. 55. Page 55 Proptosis in Children Mar 25, 2013
  56. 56. Work-up CT• Diffuse Fusiform enlargement of the optic nerve MRI• T1-weighted images – Isointense to hypointense• T2-weighted images – Isointense to hyperintense Page 56 Proptosis in Children Mar 25, 2013
  57. 57. Page 57 Proptosis in Children Mar 25, 2013
  58. 58. Treatment• Not required if – Not growing – Good vision – No cosmetic blemish• Surgical excision – Large & growing lesions – Confined to orbit• Radiotherapy + chemotherapy – Intracranial extension Page 58 Proptosis in Children Mar 25, 2013
  59. 59. EMBRYONAL SARCOMA• Most common primary orbital malignancy of childhood• Origin…undifferentiated mesenchymal cells• Called Rhabdomyosarcoma if differentiate into striated muscle Page 59 Proptosis in Children Mar 25, 2013
  60. 60. Presentation…• Usually first decade (average…7yrs)• Boys > girls• Proptosis – Rapid (days to weeks) – Unilateral – Painful – Non-axial• Mimics orbital cellulitis Page 60 Proptosis in Children Mar 25, 2013
  61. 61. … Presentation…• Swollen lids but bluish appearance• Ptosis• Location – Superonasal – Retro bulbar – Superior – Inferior• Chemosis• Sinuses involvement & nose bleeds Page 61 Proptosis in Children Mar 25, 2013
  62. 62. … Presentation• Other findings – Disc edema – Choroidal folds – Decreased vision – Cervical lymph nodes involvement Page 62 Proptosis in Children Mar 25, 2013
  63. 63. Courtesy: medlinePage 63 Proptosis in Children Mar 25, 2013
  64. 64. Courtesy: medlinePage 64 Proptosis in Children Mar 25, 2013
  65. 65. Page 65 Proptosis in Children Mar 25, 2013
  66. 66. Work-up USG• Relatively well-circumscribed mass• Low-medium amplitude echoes CT & MRI• Moderately well-defined homogenous mass• Bony destruction Biopsy Systemic investigations Page 66 Proptosis in Children Mar 25, 2013
  67. 67. Treatment• Referred to pediatric oncologist• Radiotherapy• Chemotherapy• Surgery – Exenteration for recurrent & resistant cases Page 67 Proptosis in Children Mar 25, 2013
  68. 68. LYMPHANGIOMA• Introduction – Non-Neoplastic – Non-functional – Abortive – Vascular malformation• Hemodynamically isolated• Bleeding into lumen…chocolate cysts Page 68 Proptosis in Children Mar 25, 2013
  69. 69. Presentation…• Early childhood (average=5-6 yrs)• Multiple bluish soft masses• Upper nasal quadrant• Cystic conjunctival component• Proptosis – Due to spontaneous hemorrhage – Painful – Axial/non-axial Page 69 Proptosis in Children Mar 25, 2013
  70. 70. … Presentation• Worsened by RTI• Palatal involvement• Ptosis• Restricted eye movements• Optic nerve compression• Papilledema• Secondary glaucoma Page 70 Proptosis in Children Mar 25, 2013
  71. 71. Page 71 Proptosis in Children Mar 25, 2013
  72. 72. Page 72 Proptosis in Children Mar 25, 2013
  73. 73. Work-up CT• Multilobulated cystic mass within the orbit and/or intralesional calcifications MRI• Hemorrhagic cyst…Hyperintense• Fluid level Page 73 Proptosis in Children Mar 25, 2013
  74. 74. Page 74 Proptosis in Children Mar 25, 2013
  75. 75. Treatment• Conservative • Intralesional• Partial surgical injection of resection of the sclerosing agents major cyst – sodium tetradecyl sulfate (Sotradecol®)• Needle aspiration – OK-432 (Picibanil®)• CO2 laser • local radiotherapy• Cryotherapy Page 75 Proptosis in Children Mar 25, 2013
  76. 76. • Sodium tetradecyl sulfate (Sotradecol®)• An anionic surface-active agent used for its wetting properties in industry and used in medicine as an irritant and sclerosing agent for hemorrhoids and varicose veins• $259.00 Page 76 Proptosis in Children Mar 25, 2013
  77. 77. • OK-432 (Picibanil®)• lyophilized preparation of a low-virulence strain of S. pyogenes (S. hemolyticus), inactivated by heating with penicillin G. It has been proposed as a noncytotoxic antineoplastic agent because of its immune system-stimulating activity.Page 77 Proptosis in Children Mar 25, 2013
  78. 78. CONTENTS• Recap• Causes in children• Differentiating features• Common causes discussion• Summary• Take home message• MCQs Page 78 Proptosis in Children Mar 25, 2013
  79. 79. Page 79 Proptosis in Children Mar 25, 2013
  80. 80. PROPTOSISLid edema Little/no inflammationSinus disease Orbital lesionRestricted motility Young child/adultPain/Tender Slow growingLid trauma Located near boneFever suturesORBITAL CELLULITIS Dermoid cyst Page 80 Proptosis in Children Mar 25, 2013
  81. 81. PROPTOSIS Child (5 yrs) Child (7-8yrs) Upper nasal quadrant mass Hx of nose bleeds Painful Bluish color lids Few episodes of proptosis Unilateral Worsened by RTI Lymph node involved MRI shows fluid levels PainfulORBITAL LYMPHANGIOMA Rhabdomyosarcoma Page 81 Proptosis in Children Mar 25, 2013
  82. 82. CONTENTS• Recap• Causes in children• Differentiating features• Common causes discussion• Summary• Take home message• MCQs Page 82 Proptosis in Children Mar 25, 2013
  83. 83. TAKE HOME MESSAGE• Proptosis in children may be – Benign as … Dermoid – Malignant as … Rhabdomyosarcoma• Thorough Hx & clinical approach• Pediatric ophthalmologist & oncologist Page 83 Proptosis in Children Mar 25, 2013
  84. 84. Page 84 Proptosis in Children Mar 25, 2013
  85. 85. MCQs1. A 1-month old infant presents with a purplish, spongy eyelid mass. Most likely diagnosis:a. Rhabdomyosarcomab. Neurofibromac. Dermoid cystd. Capillary hemangiomae. Metastatic Ewing sarcomaAns. d Page 85 Proptosis in Children Mar 25, 2013
  86. 86. 2. In the evaluation of child with unilateral Proptosis, which assumption is true?a. Cavernous hemangioma is the most common cause of unilateral Proptosis in childrenb. Thyroid ophthalmopathy is the most common cause of unilateral Proptosis in childrenc. Neurofibroma is the malignant tumor that most commonly produce Proptosis in childrend. ON meningioma are more common than gliomas in childrene. None of the aboveAns: e Page 86 Proptosis in Children Mar 25, 2013
  87. 87. 3. The management of Rhabdomyosarcoma of the orbit usually involvesa. Lumbar puncture to rule out CNS Metsb. Exenterationc. Enucleation & orbital radiationd. Systemic chemotherapy & orbital radiatione. Radical neck dissection if cervical L. nodes are involvedAns: d Page 87 Proptosis in Children Mar 25, 2013
  88. 88. 4. All of the following are true regarding optic nerve tumors except:a. ON gliomas in children are associated with NF-1b. ON meningioma in children are associated with NF-2c. Radiation therapy is an accepted therapy for ON sheath meningiomad. ON gliomas of childhood can be malignantAns: d Page 88 Proptosis in Children Mar 25, 2013
  89. 89. FOR MCQs 5-85. An 11-year old girl presents with acute, unilateral, left-sided periocular pain, Proptosis and double vision. Which condition would NOT be included in D. Dx?a. Cavernous hemangiomab. Sinusitis with orbital abscessc. Traumatic retrobulbar hemorrhaged. Orbital LymphangiomaAns: a Page 89 Proptosis in Children Mar 25, 2013
  90. 90. …Continued…6. 24 hrs later & without any treatment, the pain has resolved. Periocular ecchymosis develops, & the double vision stabilized. MRI shows cystic mass & layered hemorrhage. Most likely Dx based on clinical Hx & MRI findings is:a. Rhabdomyosarcomab. Capillary hemangiomac. Orbital abscessd. LymphangiomaAns: d Page 90 Proptosis in Children Mar 25, 2013
  91. 91. …Continued…7. If the patient was losing vision bcz of this process, you would consider:a. Intravenous antibioticsb. Open surgery to excise the lesion in its entiretyc. CT-guided drainage of encysted bloodd. Biopsy of the lesion to establish DxAns: c Page 91 Proptosis in Children Mar 25, 2013
  92. 92. …Continued8. The disease process is an example of :a. The most common cause of proptosis in childrenb. The most common primary orbital malignancy in childrenc. A tumor that may enlarge with upper RTId. An orbital vascular lesion that will involute after intralesional corticosteroidsAns: c Page 92 Proptosis in Children Mar 25, 2013
  93. 93. 9. Which one of the following is the most common primary malignancy of the orbit in children?a. Neuroblastomab. Rhabdomyosarcomac. Ewing’s sarcomad. None of the aboveAns: b Page 93 Proptosis in Children Mar 25, 2013
  94. 94. 10. what is the most common cause of unilateral childhood proptosis?A. capillary hemangiomaB. Thyroid related orbitopathyC. Orbital hemorrhageD. Orbital cellulitisAns: D Page 94 Proptosis in Children Mar 25, 2013
  95. 95. 11. In orbital cellulitis, which of the following is false:a. Common cause of proptosis in childrenb. Seen due to infection spread from surrounding sinusitisc. Commonly bilaterald. Can cause cavernous sinus thrombosisAns: c Page 95 Proptosis in Children Mar 25, 2013
  96. 96. 12. Most common organism causing orbital cellulitis in children < 5 yrs:a. Pneumococcib. Staph aureusc. Strep pyogenesd. H. influenzaeAns: d Page 96 Proptosis in Children Mar 25, 2013
  97. 97. 13. ON glioma includes following:a. Usually seen in 1st decade of lifeb. Can be seen either in the intraorbital or intracranial part of optic nervec. Generally causes painless, bilateral proptosisd. Proptosis is axial, non-Pulsatile & irreducible1. a+b+c 2. a+b+c+d 3. a+b+d 4. a+c+dAns: 3 Page 97 Proptosis in Children Mar 25, 2013
  98. 98. 14. A young child presents with sudden loss of vision & proptosis of right eye. On examination, the direct pupillary reflex is absent but consensual pupillary reflex is present. He is having:a. ON gliomab. ON meningiomac. Retinoblastomad. ON angiomae. Capillary hemangiomaAns: a Page 98 Proptosis in Children Mar 25, 2013
  99. 99. NEXT Lecture• Dr. Gulzada – Red eyes Journal club• Dr. Shafqat Mar 25, 2013

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