This case was submitted by one of our readers:
I am a pediatrician in a very small town in Northeast Louisiana and have a case which has me at a loss. The pt is a 12 yr. old male with a history of RAD since having RSV at 5mos of age. He also has a history of partial seizures for which he is being followed by a pediatric neurologist. His last seizure was in 5/04. There are a few other CNS problems/symptoms in his history and will be glad to describe in more detail if anyone feels they may be relevant. To me, it is important to note that he has had no CNS symptoms reported for >1 year. He has been brought in to my office by his mother, a nurse, twice within the last year complaining of leg weakness, intermittent nausea, and occasional H/A. The mother brought him in fasting because the father had hypoglycemia so she wanted a FBS on the pt. Both times I complied and both times, they were found to be normal (91 and 100 respectively).
I ordered a CBC and the WBCs were found to be a little low (5-6,000) compared to his norm of 8,000 with slightly increased lymphocytes. He also had a slightly decrease platelet count of 113,000, but still within NL. I told the mother he presently appeared to have a viral infection, but with no fever, or other S&S I'd just leave him alone. I also told her the leg weakness, etc. may or may not be related to the presumed virus, so if it persisted, bring him back and I'd check an ESR and CRP. 4-6 months after his last visit, he returned to me because his brother (8 yrs) had recently been diagnosed with H-Pylori and since the mother mentioned that THIS patient had still had nausea, I suggested we test him as well. The mother reported that the leg weakness still occurred occasionally, it was a little less frequent. Upon auscultation of his chest, I found it sounded clear, however, he didn't sound like he was moving enough air as he should be. I ordered a CXR which showed consolidation in both lobes. I could not explain how he could have no fever or cough, but there it was, in black and white! Since he also had C-Diff a little over a year ago, we are still trying not to use oral antibiotics (for as long as we can, at least) so I ordered a Bicillin CR 1.2 mu-IM, Xopenex 0.63 q6h, and pulmocort 0.5 mg bid via neb for two weeks.
I noticed on the Xray, that there were small "nodules" in his hilar region, with a larger one in the Left lower lobe. I sent the Xray to a radiologist which reads film for the clinic I work for. The report said these were calcified granulomas. With the pts history of RAD and recurrent bronchitis, I figured it was just "old junk." When he returned for his two week follow up, I repeated the CXR and after the nebs, etc., and there still appeared to be some bronchitis in his films. Still no cough, no real symptoms.
I sent him the next day to a local Asthma, Allergy, and Immunology specialist. He felt the "pneumonia/bronchitis" I was seeing was probably atelectasis due to his asthma. He wasn't sure about the granulomas, but decided he would change him to Advair 250 bid and 10 days of Prednisone 40mg bid x 2wks, then repeat the CXR at the hospital in 1 month. This time, the hospital radiology report stated that there was one granuloma in the left lower lobe, and several hilar/mediastinal calcified lymph nodes. The asthma allergy specialist felt it was probably histoplasmosis, but I wasn't convinced. I didn't feel he met criteria for histo, and I've been very involved in the care of this patient for 8 years.
I chose to send him out of town to a pediatric pulmonologist. I've known this doctor for 10 years and feel he is very good with things, that may not be clear cut. The pulmonologist felt it was one of 5 things: histoplasmosis, sarcoidosis, coccidio-mycosis, LTBI, or lymphoma. He ordered a CT of the chest, Histo titers, ACE, Coccidio titers, and a TBST (PPD) with Candida control. The CT showed a left ghon complex, and ALL other tests were completely negative or normal... with the exception that both the TBST AND the control were BOTH also negative. The mother called the pulmonologist the day the skin tests were read stating that he appeared to be anergic and wanted him to have quantitative immune globulin studies with subclasses. They were ALL normal, so the pulmonologist felt everything had been ruled out except Sarcoid because it can't be definitively ruled out without a biopsy (but, he's seen an ophthalmologist and no lesions were present and a normal ACE would at least lead you away from sarcoid). The TBST was inconclusive due to anergy. That really only left LTBI and lymphoma according to the pulmonologist.
The pulmonologist then, sent the pt to a pediatric surgeon to look at the lymph nodes and attempt to get the granuloma as well. He wanted to perform a thorascopic procedure, collapse the left lung, resect a few of the lymph nodes and it would require a chest tube and 3-4 days in the hospital. The pt's mother heard the procedure described and felt with a ghon complex, there may be a good chance it was LTBI, and preferred to explore all avenues before putting the child through such an invasive procedure. I must admit, I agreed. I ordered a repeat TBST with Candida control just shy of three weeks after the first. Both were negative this time, as well. I spoke with a pediatric infectious disease specialist who said sometimes it takes a lot of PPD to get a reaction if the infection is old. She suggested the new Quantiferon TB-Gold test, but according to the CDC, no one in this state performs it. As of now, our plan is to repeat the CT three months from the first. The surgeon had a point when he agreed to the mom wanting to wait. He said "If it's lymphoma, it'll grow." I am to the point where I either need to tell the mother that the biopsy is now necessary, or do what I've been considering and refer him to Arkansas Children's Hospital. They have Surgery, pulmonology, tuberculosis, infectious disease, and oncology departments. I suppose I would start with infectious disease, but really don't know! I do NOT want to go in the wrong direction here, but cannot think of any other preliminary tests which I can do to help narrow things down further. We only want a biopsy, if we are certain that he doesn't have anything infectious. I would greatly appreciate any ideas/suggestions or insights into this case.
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Interesting case. We usually see more adult patients but I will be glad to add my two cents.
There are two components here: the RAD and the adenopathy/abnormal radiograph.
The 2 may be related as it would be the case with sarcoid, but given his Hx of RSV and RAD preceding all of his current troubles I think they are separate issues.
The RAD seems to be well treated at this point. I would try and keep him on the lowest Advair strength given his young age though.
His radiographic findings are complicated but I also believe there is a "double component" here: The pneumonia/resp tract infection seemed pretty real with the consolidations. However the calcified nodes and granuloma seem chronic and benign given their calcification. I would hold off any more invasive biopsies and maybe repeat a CT scan 2-3 months from the original one and monitor it.
Well, I think it is an important point that the patient is asymptomatic from a respiratory standpoint. He originally presente swith some nausea and weakness. Therefore, I would not aggressively treat based on radiographic findings. The prednisone would be especially worrisome without a definitive diagnosis, especially if this had turned out to be histoplasmosis.
I agree that the RADS seems to not be an active issue here.
Remeber, the diagnosis of whatever is causing the radiographic findings is important if treatment can modify the course of that disease. If it is infectious etiology it would have to be quite chronic given his somewhat modest symptomatology. Similary, a steroid-responsive disease would be associated with some respiratory symtpoms in this patient.
A lymphoma cannot be ruled out, but your description if the CT findings sound more like previous fungal exposure.
I would do:
1. following with periodic CT, maybe Q 6 months.
2. Biopsy if constitutional symptoms persist or progress, or if respiratory sx develop.
3. A spirometry would be helpful to determine the degree of restriction (if he is dyspneic)
4. Barring an obstructive defect on spiro, I would discontinue all inhalers, including the advair, and would not prescribe oral steroid therapy unless there is a definitive diagnosis established.
Thank you for sending in the case. The story is complex, and from what I can gather the salient features are:
-12 year old with a history of RADS
-initially presented with weakness, nausea, headache, and some low-normal blood counts that were attributed to a viral infection.
-A chest radiograph initially showed bilateral consolidations in the absence of other systemic signs/symptoms such as fever, cough, chest pain, wt. loss, etc.
-The CXR also showed some hilar nodules and a more dominant left lower lobe nodule/mass which, on follow-up CT scan showed dense calcification.
-Other testing, including fungal serologies and a PPD have been negative.
Before commenting further, I have several questions:
1) Did the bilateral consolidations resolve on the subsequent CT scan?
2)You mentioned the patient had C. diff at some point. Why was he on antibiotics previously, and does he have any history of recurrent infection.
3) What is the time frame from his initial presentation to the imaging showing the nodules.
My suspicion is that the patient does not have a lymphoma or an active infection at this point. Depending on the time frame, the initial presentation could have been histo, and the patient has not "seroconverted" although I would not expect calcifications to develop this quickly. So, I think it is likely that the patient has had a granulomatous process at some point in the past. It may have been TB, but without any history of exposure or reason to be considered high-risk, or signs/symptoms of infection now, I would not aggresively persue this diagnosis. For all of these reasons, I think follow-up imaging is more than reasonable, so long as he remains asymptomatic.
With regards to RADS, I have not heard anything about his pulmonary function. I would obtain spirometry and, if normal, start peeling off the steroids and inhalers. If he becomes symptomatic again, I would obtain spirometry to confirm obstruction and treat with step-wise escalation of therapy.
Thanks for the follow-up. I think that observation is the way to go here with regard to the nodules seen on CXR. For the record, ACE levels are very non-specific, and I would never exclude the possibility of Sarcoid based solely on an ACE level. His story has been evolving over a year, so, while noteworthy, I cannot attribute his symptoms to TB exposure associated with evacuees from a few months ago (not to say that this couldn't have been a sporadic exposure at some point in the past).
I think the histo question is interesting. Most of the time, acute histoplasmosis is a self-limited infection that is frequently attributed to "flu" or a "bacterial pneumonia" that resolves. The calcified granulomas are identified at some later point in time. But, I would expect some serologies to be consistent with histo exposure.
Finally, it sounds as if he has sinusitis now (with the post-nasal drip and headaches). If he has a history of recurrent sino-pulmonary infections, it may be worth checking quantitative immunoglobulins, although the yield is likely to be pretty low. Immunodeficiencies can sometimes present this way (hence my question regarding skin-infections...). Finally, although he is quite young and this would be very atypical, we need to keep things such as Wegeners, Churg-Strauss, and ABPA in the realm of possibility. My sense is still that he is a kid with asthma, and the rest of this is unrelated...
I agree with Jeff H. The histo issue seems fairly benign: the granulomata are calcified and whether he still has titers or not won't change management.
At age 12 the PPD testing can be fairly reliable (kids under 5 with clear exposure would be treated even with equivocal PPDs) and with the described symptoms I would not treat him for LTBI.
I know this is from many years ago, but am hoping for an update. The symptoms this child is showing are very familiar to the symptoms of my son, however he is only 6 years old. My son seems to be constantly sick, fever, cough, stuffy nose, and nausea. He just had a chest x ray and they found scattered calcified granuloma. He also had polyps opened in his turbinates. He is on his 2nd set of tubes and has had his tonsils and adenoids out due to reoccuring strep throat. After adenoidectomy he developed an infected mass where the adenids were that was benign and removed. He has pneumonia 7 times beginning at 5 months and rsv twice. He has been diagnosed with the flu seperately from these times atleast 3 times also. They have ran Cystic Fibrosis tests, immune blood work, allergy tests, and normal blood count tests. Everything has came back negative. He cannot keep up with the other children because he complains of leg pain and weekness. He does not sleep well, so in turn he sleeps long hours. 12-14 hours a day. He also sleep walks. He does not stay focused well either, as a smaller child he was very focused and his focus and temper seem to be getting worse. (like he is tired all the time) The teacher has even commented that he has almost fallen asleep in school. (kindergarten) I have noticed the lymphnodes behind his ears swell and go down quite often. The pedi says this is not a concern. I may have missed a few things, however these are the main. Any insight would be great. I am at a loss.
Thank you
Worried mother.
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