GRAND ROUNDS

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GRAND ROUNDS

Desiree Ong, M.D.

Vanderbilt Eye Institute

12/15/06

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.

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

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

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

Sympathetic Pathway

Differential diagnosis?

Acquired Pediatric Horner Syndrome

Differential Diagnosis

Acquired 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

• 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

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

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

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

Testing

• No standard evaluation protocol

• Previous studies suggested that a h/o birth trauma or urine studies alone is sufficient 3

• CBC, FTA-ABS, VDRL, PPD

• VMA and HVA urine tests

– positive in 90-95% with neuroblastomas

– localized tumors may be associated with normal urine studies 2

• MRI/MRA, extracranial Doppler, and/or chest x-ray

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

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.

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

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

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

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

Cervical Lymphadenopathy

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.

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

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

References

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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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