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Leukemic infiltration of the Iris and Cranial Nerves in recurrent ALL

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Title: Leukemic infiltration of the Iris and Cranial Nerves in recurrent ALL
Author: Heidi Dahl MS4
Photographer: if available
Date: 06/26/19
Keywords/Main Subjects: Acute Lymphoblastic Leukemia
Diagnosis: Pre-B-Cell Acute Lymphoblastic Leukemia

Description of Case: A 7-year-old female with a past medical history of high risk pre-B-cell Acute Lymphoblastic Leukemia (ALL) was seen in the pediatric ophthalmology clinic with a six-day history of left-sided exotropia, anisocoria and blurred vision. The patient’s parents had noticed a change in the color of her left Iris for a few months but had been told that it was due to her chemotherapy treatments. Also of note, the patient had been diagnosed with a left-sided Bell’s Palsy two months prior to this presentation. A Computed Tomography (CT) scan of the head done at that time showed no changes. She was treated with steroids and antibiotics and the Bells Palsy resolved except during times of illness.

Basic Exam at Presentation:

Right eye Left eye
Visual Acuity 20/40 20/50
Pupils 5mm in dark, 3.5mm in light, round with brisk reaction. 6mm in dark, 5mm in light, round with slow to minimal reactions. No Relative Afferent Pupillary Defect
VF Full Full
EOM XT’ of 45 PD

XT of 50 PD Diopters

-2 Adduction Deficits in both eyes

IOP Unable to Obtain Unable to Obtain

 

Slit Lamp Exam:

Right eye Left eye
Lids/Lashes Normal Normal
Conjunctiva/sclera Normal Nasal Hyperemia
Cornea Clear Clear
Anterior chamber Quiet and Deep, No Cell Quiet and Deep, No Cell
Iris Iris Blue Yellow-Green discoloration with thickening diffusely and mild ectropion uveae
Lens Clear Clear
Vitreous Normal Normal

 

Dilated Fundus Exam:

Supero/Nasal blurring of disc margin, no heme Normal
Disc Normal Normal
Macula Normal Normal
Vessels Normal Normal

 

Images 1 – 2:

Left Iris showing diffuse thickening and yellowish infiltrate.

 

Right Iris unaffected.

 

Oncology was consulted and the patient was admitted to the hospital that day for and LP, bone marrow biopsy and MRI of brain and orbits.

Lumbar puncture showed numerous malignant cells morphologically compatible with blasts. Bone marrow biopsy showed Bone marrow aspirate showed recurrent B-Lymphoblastic Leukemia with 5% blasts by morphology and 9% precursor B-Cells by flow cytometry.

Images:

Fiesta scan showing symmetric involvement of extra ocular eye muscles, indicative of an increase in cellularity. This is consistent with a Leukemic relapse.

 

Diffusion weighted scan showing diffusion restriction in EOM symmetrically and bilaterally. Indicative of an increase in cellularity.

 

T2 MRI with contrast. Right optic nerve sheath showing enhancement.

 

Fiesta MRI showing enhancement of cranial nerves V (right arrows), VI (left blue) , and VII (left Orange). There was enhancement of almost all cranial nerves throughout their course, including CN III.

 

Summary of the Case: 7-year-old patient with a history of high risk pre-B-cell ALL, in remission for eight months, presents with new onset exotropia, anisocoria and blurred vision. On exam she is found to have bilateral partial Cranial Nerve III palsy, right sided optic nerve involvement as well as heterochromia. Upon further workup she was found to have a relapse of ALL in both her CNS and bone marrow.

Acquired heterochromia has an extensive differential including malignant and benign tumors of the iris, bleeding in the eye, iritis, uveitis, Fuchs’ heterochromic cyclitis, acquired Horner’s syndrome, medication side effects, pigment dispersion syndrome, ocular melanosis, Posner-Schlossman syndrome and iris ectropion syndrome.6 Acquired heterochromia in children is always concerning, especially in a patient with a history of leukemia.

Anterior chamber involvement in leukemic relapses is extremely rare, occurring in only 0.5-2.5% of patients.Leukemic infiltration of the anterior chamber has been known to present with many different signs including conjunctivitis, scleral hyperemiacorneal edema, corneal opacification, iris heterochromia, thickened iris, rubeosis iridis, plastic iridocyclitisanisocoriamydriasis, glaucoma or raised intraocular pressurehyphema, blood-streaked hypopyonuveitis, and panuveitis. Symptoms included epiphora, painful injected eyes, photophobia, visible iris hemorrhage, tearing, decreased vision, and pseudostrabismus.1 Ocular involvement of ALL relapse portends a poor prognosis and these patients need to be started on chemotherapy as soon as possible. Therefore, a high index of suspicion is indicated when examining a patient with new onset of ocular symptoms and a history of leukemia.

CNS involvement occurs in 5-10% of leukemic relapses with the most common presenting signs being meningismus and cranial nerve palsies. The most commonly involved cranial nerve is CN VII.2

References:

  1. Yu, A. M., et al. (2019). “Anterior segment infiltration of acute lymphoblastic leukemia: case report and systematic review.” Can J Ophthalmol 54(1): 20-26.
  2. Sung, S. H. and I. S. Jang (2014). “Isolated central nervous system relapse of acute lymphoblastic leukemia.” Brain Tumor Res Treat 2(2): 114-118.
  3. Harada, Y., et al. (1994). “[Anterior segment of the eye and cranial nervous (II, III, VI, VII) infiltration in relapsing acute lymphoblastic leukemia (L1)].” Rinsho Ketsueki 35(12): 1371-1377.
  4. Sen, S., et al. (2016). “Bilateral Facial Nerve Palsy in Acute B Cell Lymphoblastic Leukemia: A Case Report and Review of the Literature.” Indian J Hematol Blood Transfus 32(Suppl 1): 15-19.
  5. Reddy, S. C. and B. S. Menon (1998). “A prospective study of ocular manifestations in childhood acute leukaemia.” Acta Ophthalmol Scand 76(6): 700-703.
  6. David Turbet. Heterochromia. American Academy of Ophthalmology. Available at https://www.aao.org/eye-health/diseases/what-is-heterochromia. Accessed on 07/23/2019.

Faculty Approval by: Dr. Griffin Jardine
Identifier: Moran_CORE_26959
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