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Fluid-refractory orthostatic hypotension in preteen female

Fluid-refractory orthostatic hypotension in preteen female. Christine Hrach, MD Pediatric Hospitalist. I have no disclosures. Case Presentation. AF is a 12 y.o. female presenting with 5 days of malaise, emesis, weakness, intermittent diaphoresis

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Fluid-refractory orthostatic hypotension in preteen female

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  1. Fluid-refractory orthostatic hypotension in preteen female Christine Hrach, MD Pediatric Hospitalist

  2. I have no disclosures.

  3. Case Presentation • AF is a 12 y.o. female presenting with 5 days of malaise, emesis, weakness, intermittent diaphoresis • She endorsed rigors, neck pain, and a burning, pruritic rash to neck and arms • 11 pound weight loss in the previous 3 weeks • Tmax of 100-101 • Initially evaluated in the ER, found to be monospot positive, received 20 ml/kg NS Bolus and admitted for dehydration

  4. Admission exam • Initial exam: • Vital signs: Wt 44kg (50%), T 37.2, P 76, R 24, BP 131/74 • Neck stiffness • Report of diffuse weakness, but 5/5 strength on exam • Symmetric mild hyperreflexia, 3+ brachial and patellar • Excoriations above L clavicle, and macular erythematous rash in distribution of t-shirt collar

  5. Hospital course • Admitted for four days • Received iv fluids and Zofran • Neck stiffness resolved with NSAIDs • Lab findings: wbc 9.2, hgb 16.5, plt 411, 78% N/11% L • Ua: SG 1.030, 2+ ketones • Normal CMP, mg, and phos (BUN = 22) • Normal TSH and free T4 • AF’s emesis and oral intake gradually improved • PT consult done – exercise regimen established for home while she was recovering from mono • Discharged to home

  6. Return to ER • AF presented again to the ER 4 days after discharge with decreased po intake and persistent NBNB emesis • Now a 15 pound weight loss • New symptoms of dizziness, blurry/ tunnel vision, “seeing black” when sitting, feeling faint upon standing, and unable to ambulate without assistance • Difficulty initiating micturition, urinating only 2-3 times a day

  7. ER course • Lab analysis • Prior EBV titers reviewed: • IgM negative • IgG reactive • CMV non-reactive • Na – 131, BUN – 20 • Wbc 6.8, hgb 14.9, plts 404, 58% N/ 27%L • EKG = normal sinus rhythm • Received 20 ml/kg NS bolus, admitted to the floor

  8. Additional history • Visual changes with sitting or standing, black spots • Pruritus and rash to upper chest • Uncomfortable sensations of burning or itching to bilateral upper extremities involving thumbs and radial aspects of hands and forearm • Constipation – last BM 11 days prior to admission • Hiccups – up to one hour at times, 2-3 times a day

  9. Vital signs

  10. Admission exam • Significant orthostatic hypotension • Ill-appearing, thin, pale • CV – RRR without murmur • Neuro exam: • 4/5 strength in upper and lower extremities • 3-4+ DTR’s throughout • Required assistance with ambulation

  11. Differential Diagnosis • Summary – 12 year old female with dysaesthesias, weakness, hyperreflexia, severe orthostatic hypotension, fifteen pound weight loss, and urinary retention • Infectious • Malignancy • Neurologic • Eating disorder

  12. Hospital course • Neurology consultation – concern for a cervical spine lesion • MRI/MRA ordered of brain and MRI with and without contrast of cervical, thoracic, and lumbar spine • Echo obtained: normal segmental anatomy and normal cardiac structure

  13. MRI Results • Bright T2 signal within the R optic nerve • Diffuse swelling of the cord with hyperintense T2 signal extending from C1 to T3-4 level • Cord swelling is most significant at the cervical and upper thoracic level • Most likely represents neuromyelitis optica • Less likely considerations include transverse myelitis, viral/postviral myelitis

  14. Hospital course • LP done: cell count 84, elevated protein 72.9, glucose 47 • Transferred to PICU for emergent plasmapheresis • Laboratory data: positive serum and CSF NMO antibodies • Completed 7 cycles of plasmapheresis and a 5-day steroid burst followed by a one-month steroid wean • Ophthalmology consulted – normal eye exam • Rituximab infusion given for immunosuppression, plans to continue infusions as an outpatient

  15. NMO • Neuromyelitis optica • A relapsing demyelinating disorder, distinct from multiple sclerosis • Predilection for the optic nerves and spinal cord • NMO-IgG is an antibody that seems to be present in about 70% of those with NMO (not found in MS) • Usual presenting symptoms include visual impairment, weakness and gait disturbance

  16. NMO • Treatment guidelines are based on consensus opinion and retrospective studies • Treatment usually consists of a combination of intravenous corticosteroids and plasma exchange • Rituximab is a second-line agent • 3 aspects to treatment • Relapse treatment • Relapse prevention • Symptom management and rehabilitation

  17. Conclusion • Orthostasis is commonly attributed to dehydration in children • Orthostasis out of proportion to history of fluid loss or the apparent degree of dehydration on exam should prompt consideration for other causes • Spinal cord pathology should be in the differential diagnosis as it may present with autonomic dysfunction

  18. Thank you • Co-authors on this case: • Kim Hamlin, MD • Liza Harrison, MD

  19. Questions??

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