Q:

7/23/2013
I saw a 15 y.o. boy who presented to the ED with gradually progressive facial swelling over a few days. He sensed difficulty swallowing. He had an itchy rash on his face and chest attributed to surgical tape (has history of rash with adhesives). Tryptase was 13 ng/ml. Treated with epinephrine and oral steroids. Symptoms resolved and have not recurred for past year.

Repeat tryptases several months apart w/o symptoms was 13 and 14. Mature tryptase 1.9 ng/ml. Complicated past medical history of remote craniopharyngioma, s/p resection and radiation. Has panhypopituitarism and VP shunt for shunted hydrocephalus. From his records, he has chronic headaches that date back about 4 months prior to his facial swelling episode. Has had multiple VP shunt revisions for daily headaches but headaches continue despite functional shunt. Had one shunt revision about 1-2 wks before facial swelling. CSF has repeatedly elevated eosinphils documented since 1 wk before facial swelling, ranging 5% - 33%. Most recent CSF eos is 5%. CSF cultures negative. Neurosurgeon wondering if headaches and high CSF eos due to allergic reaction to shunt. Tried skin testing with shunt material and negative. Trying to see if tryptase, CSF eos and persistent headaches tie together. Otherwise, any other follow-up for slightly elevated trypase? Thank you.

A:

Dear Dr. Gimenez:

Thank you for your inquiry.

The most salient feature of this boy’s problem of course is his cerebrospinal fluid eosinophilia. I have copied below a link that will give you access to an excellent review of cerebrospinal fluid eosinophilia. In the absence of parasitic infection, by far, the most likely cause of the eosinophilia is his shunt. Chronic cerebrospinal fluid eosinophilia is a well known complication of ventricular shunts. Copied below are abstracts of articles which discuss this in detail. Unfortunately, the pathophysiology underlying the eosinophilia has not been established. However, the treatment would remain in the hands of his neurosurgeon.

Other points of his history were the modestly elevated serum tryptase and his single episode of facial swelling. I could not tell you what caused his single episode of facial swelling, but since it has not recurred during the last year, I do not think any further workup at this time would be helpful. It does not sound (at least as described as facial swelling) like it was actually angioedema - which is usually asymmetric and does not involve the entire face. However, I do not think there is enough information available for us to establish the diagnosis or the cause at this time. If he does have another episode, and you are unable to see him during the event, I would suggest that a photograph be taken so that you could at least retrospectively see what the swelling looked like, and then go from there.

I feel the same way regarding his modestly elevated tryptase levels. I do not think there is enough information in his history to warrant any further investigation in this regard at this time. Cerebrospinal fluid eosinophilia is not, to my knowledge, a feature for systemic mastocytosis, and there is nothing else in his history that would suggest the need of a bone marrow, which would be the next step in the workup of an elevated serum tryptase.

In summary, I believe that the eosinophils in his spinal fluid are related to his ventricular shunt as described in the abstracts copied below. In view of the fact that he has only had one episode of facial swelling, and this was one year ago, I do not think a workup at this time for the swelling is indicated. And I also do not feel that you need to pursue a workup for his elevated serum tryptase at this time.

Finally, I know of no condition which would explain all four manifestations (cerebrospinal fluid eosinophilia, modestly elevated tryptase, facial swelling, and headaches). But I do feel, as noted, the shunt clearly explains the CSF eosinophilia and probably the headaches as well..

Thank you again for your inquiry and we hope this response is helpful to you. Should further episodes of facial swelling or other manifestations occur that you feel would alter our perspective, we would of course be happy to hear from you again at any time.

doi: 10.1128/CMR.00044-08 Clin. Microbiol. Rev. April 2009
vol. 22 no. 2 322-348
Update on Eosinophilic Meningoencephalitis and Its Clinical Relevance

J Neurosurg Pediatr. 2008 Apr;1(4):288-95. doi: 10.3171/PED/2008/1/4/288.
Cerebrospinal fluid eosinophilia in children with ventricular shunts.
Fulkerson DH, Boaz JC.
Source
Department of Neurosurgery, Division of Pediatric Neurosurgery, James Whitcomb Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, Indiana 46202, USA.
Abstract
Object: Eosinophils have been reported in children with cerebrospinal fluid (CSF) shunts. The goal of this study was to describe the risk factors, relationship to infection, and clinical significance of CSF eosinophilia in a large group of shunt-treated patients.
Methods: The authors performed a retrospective review of data obtained in all patients who underwent ventricular shunt placement or revision at the James Whitcomb Riley Hospital for Children between 2000 and 2004.
Results: Eosinophils were identified during a follow-up shunt evaluation in 93 (31%) of 300 patients after initial shunt placement. Eosinophilia was statistically related to CSF extravasation (p < 0.0001), shunt infection (p = 0.031), blood in CSF (p < 0.0001), younger age at shunt insertion (p = 0.030), and the diagnosis of posthemorrhagic hydrocephalus (p < 0.0001). Patients with CSF eosinophilia had a higher risk of subsequent shunt failure (p < 0.0001). Analysis was performed using data obtained in a cohort of patients with a total of 130 shunt infections. Cerebrospinal fluid eosinophils were identified in 118 infections (90.8%). The leukocytic and eosinophilic reactions were dependent on the infecting organism. Propionibacterium acnes had a statistically lower CSF leukocyte count but higher differential percentage of eosinophils than the other common pathogens.
Conclusions: Cerebrospinal fluid eosinophilia is a relatively common finding in children with shunts. Patients with CSF eosinophilia had an increased risk of shunt malfunction in the present series. Eosinophilia is associated with infection, CSF extravasation, and blood in the CSF. Patients with P. acnes-induced shunt infections have higher eosinophil percentages than are found in infections associated with other common organisms. Therefore, in patients with eosinophilia, extended anaerobic culture studies should be performed with particular attention paid to searching for this pathogen.

J Neurosurg. 1991 Oct;75(4):541-4.
Ventricular cerebrospinal fluid eosinophilia in children with ventriculoperitoneal shunts.
Tung H, Raffel C, McComb JG.
Source
Division of Neurosurgery, Childrens Hospital, Los Angeles, California.
Abstract
To determine the significance of cerebrospinal fluid (CSF) eosinophilia, the charts of 106 patients treated with shunt-related procedures during the calendar year 1985 were reviewed. Sixty-nine patients presented for a shunt revision; their charts were retrospectively reviewed from the time of shunt insertion until January, 1988. The remaining 37 patients had a ventriculoperitoneal shunt inserted during the study period and were subsequently followed to January, 1988. A total of 558 shunt-related procedures were performed on these patients during the study period, with a mean follow-up period of 6.9 years. The infection rate was 3.8%. Eosinophilia was diagnosed when eosinophils accounted for 8% or more of the total CSF white blood cell count. Ventricular CSF eosinophilia occurred in 36 patients sometime during their clinical course. These 36 patients required a mean of 8.5 shunt revisions, while the remaining patients required a mean of 2.5 revisions (p less than 0.001). Shunt infections were also more frequent in patients with eosinophilia (p less than 0.01). In no case was peripheral eosinophilia or a parasitic infection present. This study demonstrates that CSF eosinophilia is common in children with shunts. Children with this laboratory finding will experience more shunt failures. In addition, the new appearance of eosinophilia in the CSF of a patient with a shunt in place suggests the possibility of a shunt infection.

Sincerely,
Phil Lieberman, M.D.

AAAAI - American Academy of Allergy Asthma & Immunology