1 / 30

GRAND ROUNDS

GRAND ROUNDS. Desiree Ong, M.D. Vanderbilt Eye Institute 12/15/06. Our Patient. CC: “Droopy eyelid”

betty_james
Download Presentation

GRAND ROUNDS

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript


  1. GRAND ROUNDS Desiree Ong, M.D. Vanderbilt Eye Institute 12/15/06

  2. Our Patient • CC: “Droopy eyelid” • HPI: 7 mo old male with unequal pupil size x 3 days noted by mother. Pt was evaluated at an outside ER, head CT was negative. Over the next 24 hrs, mother noticed that left eyelid began to droop.

  3. History • POHx: none • PMHx: full-term, normal birthweight, no complications • FHx: no eye disease, healthy 6 yo sibling • Social hx: non-contributory • Allergies: NKDA • Meds: none

  4. Exam • VA: Fixes and follows • Pupils: OD bright 4-->3mm dim 5.5-->4.5mm OS bright 2.5-->2mm dim 2-->1.5mm • Motility: grossly full OU • Tp: soft to palpation OU

  5. Exam continued • PLE • External: palpebral fissure OD 9mm, OS 6mm, small movable cervical nodes left>right • Lids/lashes: quiet OU, mild ptosis OS • S/C: quiet OU • K: quiet OU • A/C: formed OU • Iris: intact OU • Lens: clear OU • DFE • WNL OU, C/D 0.2 OU

  6. Sympathetic Pathway

  7. Differential diagnosis?Acquired Pediatric Horner Syndrome

  8. Differential DiagnosisAcquired Pediatric Horner Syndrome • First-order lesions (hypothalamus to C8-T2) • Meningitis • Basal skull tumors, pituitary tumor • Brain stem vascular malformation • Cerebral vascular accident • Demyelinating disease • Intrapontine hemorrhage • Neck trauma/cervical disk disease • Second-order (preganglionic) lesions (T1 to SCG) • Neuroblastoma • Lymphadenopathy (reactive or malignant) • Apical lung tumors • Metastases • Mandibular tooth abscess • Lesions of the middle ear (eg, acute otitis media) • Thyroid adenoma • Thoracic aorta, subclavian or common carotid artery aneurysm • Trauma/surgical injury/chest tube/central venous catheter

  9. Third-order lesions (SCG to post-ganglionic neurons) • Internal carotid dissection/aneurysm/vasopasm • Extension of cavernous sinus tumor, nasopharyngeal tumor • Carotid cavernous fistula • Cluster/migraine headaches • Herpes zoster • Otitis media • Neck trauma/tumor (i.e. rhabdomyosarcoma)/inflammation • Drugs • Bupivacaine • Chlorprocaine • Chlorpromazine • Deserpidine • Diacetylmorphine • Diethazine • Fluphenazine • Guanethidine • Influenza virus vaccine • Levodopa • Lidocaine • Mepivacaine • Mesoridazine • Oral contraceptives • Procaine • Prochlorperazine • Promethazine • Propoxycaine • Reserpine • Thioridazine

  10. Lab Results • Pt was admitted for observation • CXR: negative • MRI/CT brain/chest/abdomen: negative • CBC, CMP, HVA/VMA urine tests were WNL • LDH was elevated at 552

  11. Horner Syndrome • Ptosis - denervation of Müller muscle • “Reverse ptosis” - lower lid elevation • Miosis - greater in dim light (dilation lag) • Anhidrosis – impaired flushing and sweating • First-order: ipsilateral body • Second-order: ipsilateral face • Post-ganglionic (third-order): absent or limited • Iris heterochromia – affected iris is lighter - Congenital or children < 2 yrs - Long-standing lesions

  12. Other associations • First-order lesions: • Hemisensory loss, weakness, dysarthria, dysphagia, ataxia, vertigo, and nystagmus • Second-order lesions: • H/o trauma/surgery, facial/neck, axillary/shoulder or arm pain/swelling, cough, hemoptysis • Third-order lesions: • Diplopia (CN VI palsy), numbness/pain in V1 and V2 • Cluster headaches • may cause temporary or permanent Horner syndrome

  13. Testing • No standard evaluation protocol • Previous studies suggested that a h/o birth trauma or urine studies alone is sufficient3 • CBC, FTA-ABS, VDRL, PPD • VMA and HVA urine tests • positive in 90-95% with neuroblastomas • localized tumors may be associated with normal urine studies2 • MRI/MRA, extracranial Doppler, and/or chest x-ray

  14. Testing • Cocaine (4% or 10%) • Inhibits the re-uptake of norepinephrine • Denervation  poor dilation regardless of level • Anisocoria greater than 0.8 mm = positive • Apraclonidine (0.5% or 1%) • Alpha-receptor agonist • Denervation supersensitivity of the iris dilator • Reversal of anisocoria = positive

  15. Chen et al. (2006) • Small randomized crossover study (10 pts) • Testing with 0.5% apraclonidine and 4% cocaine • Mean differences in pupil diameter -before/after 4% cocaine = -2.08/-2.97 mm (p=0.0047) -before/after 0.5% apraclonidine = -2.04/+1.08 mm (p=0.005) • Conjunctival hyperemia in two patients • Conclusion: 0.5% apraclonidine is safe and effective for diagnosis of Horner syndrome in children Chen PL, Chen JT, Lu DW, Chen YC, Hsiao CH. Comparing efficacies of 0.5% apraclonidine with 4% cocaine in the diagnosis of Horner syndrome in pediatric patients. J Ocul Pharmacol Ther. 2006 Jun;22(3):182-7.

  16. Testing • Hydroxyamphetamine 1% (Paredrine) • Stimulates presynaptic norepinephrine release • Distinguishes presynaptic from postganglionic lesions • 40-97% sensitive; inaccurate within 24-48 hours of cocaine test • Failure of affected pupil to dilate equally or greater to normal pupil = third-order lesion

  17. Mahoney et al. (2006) • Retrospective review (56 children) • 28 (50%) had no previously identified cause - 18 with complete imaging and urine studies - Mass lesions found in 6/18 (33%); 4 had neuroblastoma; all negative urine studies • Of all patients, 13/56 had a neoplasm (23%) • Conclusions: - Urine testing alone is inadequate - Recommend physical exam with palpation - MRI brain/neck/chest, VMA and HVA by spot Mahoney NR, Liu GT, Menacker SJ, Wilson MC, Hogarty MD, Maris JM. Pediatric Horner syndrome: etiologies and roles of imaging and urine studies to detect neuroblastoma and other responsible mass lesions. Am J Ophthalmol. 2006 Oct;142(4):651-9

  18. Cervical Lymphadenopathy in Children • Acute bilateral - adenovirus, influenza, RSV; EBV and CMV • Acute unilateral - strep or staph (40-80%) • Indications for biopsy: - Persistent enlargement - Solid fixed or supraclavicular mass - Constitutional signs and symptoms • Increased risk for malignancy:8 - Generalized LAD - LAD > 3 cm - Hepatosplenomegaly - High LDH levels

  19. Cervical Lymphadenopathy in Children • Subacute/chronic LAD • Cat scratch, mycobacteria, and toxoplasmosis • EBV, CMV, histoplasmosis, HIV • Leukemia, lymphoma, neuroblastoma, rhabdomyosarcoma, and nasopharyngeal carcinoma • Laboratory tests are not necessary in majority • Most cases are self-limited and require no treatment

  20. Cervical Lymphadenopathy

  21. Oguz et al. (2006) - Retrospective review • 457 children aged 2 mo -19 yrs • 76% benign, 24% malignant • 61% of the benign group had an unknown etiology • Most common benign etiologies: EBV and acute lymphadenitis • Most common malignant: Hodgkin’s and NHL • None in the infant group had a malignant process Oguz A and Karadeniz C. Evaluation of Peripheral Lymphadenopathy in Children. Pediatric Hematology and Oncology. 23:549-561, 2006.

  22. Our Patient • Started on Unasyn • Improvement was noted over the next few days • Repeat CT showed decreased necrosis • Persistent Horner syndrome, monitored closely • Recently started on 10 days of Omnicef for a persistent otitis media/URI

  23. Take Home Points • Horner syndrome may be the first symptom of a potentially serious condition • Children with Horner syndrome should undergo a thorough physical exam with urine testing and MRI • Acute cervical lymphadenopathy in children usually has an infectious cause but should be monitored closely

  24. References • Chen PL, Chen JT, Lu DW, Chen YC, Hsiao CH. Comparing efficacies of 0.5% apraclonidine with 4% cocaine in the diagnosis of Horner syndrome in pediatric patients. J Ocul Pharmacol Ther. 2006 Jun;22(3):182-7. • Fritsch P, Kerbl R, Lackner H, et al. “Wait and see” strategy in localized neuroblastoma in infants: an option not only for cases detected by mass screening. Pediatr Blood Cancer 2004;43:679-682. • George ND, Gonzalez G, Hoyt CS. Does Horner's syndrome in infancy require investigation? J Ophthalmol. 1998 Jan;82(1):51-4 • Leung AK, Robson WL. Childhood cervical lymphadenopathy. J Pediatr Health Care. 2004 Jan-Feb;18(1):3-7 • Mahoney NR, Liu GT, Menacker SJ, Wilson MC, Hogarty MD, Maris JM. Pediatric horner syndrome: etiologies and roles of imaging and urine studies to detect neuroblastoma and other responsible mass lesions. Am J Ophthalmol. 2006 Oct;142(4):651-9 • Oguz A and Karadeniz C. Evaluation of Peripheral Lymphadenopathy in Children. Pediatric Hematology and Oncology. 23:549-561, 2006. • Sauer C, Levingohn MW. Horner's syndrome in childhood. Neurology. 1976 Mar;26(3):216-20 • Twist CJ. Assessment of lymphadenopathy in children. Pediatr Clin North Am 2002;49(5):1009-1025. • Yaris N et al. Analysis of Children with Peripheral lymphadenopathy. Clinical Pediatrics. 2006;45:544-549.

More Related