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GRAND ROUNDS

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

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GRAND ROUNDS

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  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.

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