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Painful swelling, lateral proptosis of right eye in a 9-year-old male
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A 9 ½-year-old male presented with painful swelling between the eyes, along with some lateral proptosis of the right eye and dysconjugate gaze. He had some headache, but no fever or other complaints. The history of this problem revealed that he had been previously diagnosed with allergic mycotic sinusitis at another facility with similar symptoms well more than a year earlier, which was treated with surgical debridement of the involved sinuses and a prolonged course of topical steroid nasal spray and oral itraconazole.
He was better for almost a year, but a couple of months ago (several months after stopping the above therapy), the swelling reoccurred. His new primary referred him to a sinus surgeon and our Pediatric Infectious Diseases Clinic for further evaluation.
James H. Brien
On exam his vital signs are normal and positive only for some edema and eye findings noted above (Figure 1 taken by his mother; no pictures were taken by physicians at this stage). This picture shows some preseptal swelling, and although it cannot be clearly seen, there is some restricted gaze on the right. A CT scan is shown (Figures 2 and 3) along with an intraoperative view by sinoscopy of his right maxillary sinus (Figure 4).
He is otherwise healthy with only some atopic symptoms of seasonal rhinitis and mild asthma. His immunizations are up-to-date. He has had no injury to the area.
On exam his vital signs are normal and positive only for some edema and eye findings noted above (Figure 1).
What’s Your Diagnosis?
A. Rhinocerebral mucormycosis
B. Recurrence of allergic mycotic sinusitis
C. Pott puffy tumor
D. Bacterial sinusitis
This is a recurrence of allergic mycotic sinusitis. Actually, that answer is probably not entirely true. It probably never resolved since the initial diagnosis. His original debridement and culture grew Aspergillus, and, when evaluated by a pediatric allergist, it was found that his skin test was positive for Aspergillus as well.
This picture shows some preseptal swelling, and although it cannot be clearly seen, there is some restricted gaze on the right. A CT scan is shown (Figures 2 and 3).
Source: Brien JH
This is a very uncommon and frustrating condition, seen in atopic patients who usually have elevated eosinophils, elevated IgE levels, asthma, nasal polyps and chronic allergic rhinitis. It might be compared to allergic bronchopulmonary Aspergillosis in both pathophysiology and approach to medical therapy. The surgical management includes periodic debridement of the involved sinuses, especially during the first year.
Figure 4 shows some brownish-gray material that represents some of the inflammatory debris that was removed. Meanwhile, topical steroid spray and oral antifungal therapy are used to help control the problem while immunotherapy in the form of desensitization shots to Aspergillus has time to work for a more permanent solution; at least theoretically.
Figure 4 shows intraoperative view by sinoscopy of the patient's right maxillary sinus.
The antifungal agent that seems to be preferred, probably because it can be given orally and is generally well, tolerated, is itraconazole; however, evidence for clear benefit is weak at best and remains controversial. In follow-up a couple of years later, this patient has been off itraconazole and topical steroids for about a year and is rarely requiring deep debridement procedures. He still receives desensitization therapy to Aspergillus.
My best advice when faced with a patient with this condition is to find an otolaryngologist who has a special interest in sinus disease and is familiar with this condition. Hopefully, he or she will have access to an allergist with similar experience. Although I am writing this column, the infectious disease specialist plays a minimal role at best. My role in this case was limited to giving some oral antifungal therapy and coordination with the ones who can really influence the outcome (the surgeon and allergist).
The problem begins as a frontal sinusitis that extends to involve the bone and classically results in a painful, subperiosteal abscess over the frontal bone (Figure 5), thus the name “puffy tumor.” It may also extend beyond the sinus into the cranium by direct extension or by venous spread (Figure 6 showing a frontal abscess).
Pott puffy tumor was first described by Sir Percivall Pott in 1760. Occasionally, there is a history of preceding trauma to the sinuses (including surgery) that may predispose the patient to this condition. It is usually caused by Staphylococcus aureus, but often mixed with anaerobes and other aerobes. The problem begins as a frontal sinusitis that extends to involve the bone and classically results in a painful, subperiosteal abscess over the frontal bone (Figure 5), thus the name “puffy tumor.” It may also extend beyond the sinus into the cranium by direct extension or by venous spread (Figure 6 showing a frontal abscess). Treatment is debridement of the sinus via sinoscopy, and if significant intracranial spread is found, then neurosurgical drainage and debridement will be needed, along with broad-spectrum antimicrobials directed against the most likely organisms noted above, pending culture results.
Rhinocerebral zygomycosis is another rare infectious complication of the sinuses in patients with underlying immune compromise, especially poorly controlled type 1 diabetes, as seen in Figures 7, 8 and 9.
Rhinocerebral zygomycosis is another rare infectious complication of the sinuses in patients with underlying immune compromise, especially poorly controlled type 1 diabetes, as seen in Figures 7, 8 and 9 (courtesy of
Case Discussion
At the risk of looking stupid, I’m going to point out a couple of mistakes commonly made. Note that I used the term “Pott puffy tumor,” not Pott’s puffy tumor. This is a grammatical mistake made in reference to many conditions (Down’s syndrome, Pott’s disease, Pott’s puffy tumor, Kawasaki’s disease, etc) seen throughout our profession, both spoken and written, to include very prestigious journal references and equally prestigious speakers. To place the “apostrophe s” on a word connotes ownership or possession. Sir Percival Pott described this condition in the British medical literature but did not, and does not, own or possess the condition.
Rhinocerebral zygomycosis is another rare infectious complication of the sinuses in patients with underlying immune compromise, especially poorly controlled type 1 diabetes.
Another equally common mistake is the use of the plural word “data” in the singular context; “this data showed that result.” It should be “this set of data, or these data showed that result.” The word “data” was derived as the plural form of the Latin word “datum” in the 17th century. However, as time goes by, these mistakes become so common that they are incorporated into the language as normal and academically acceptable. However, don’t despair. Since we don’t speak “Old English” anymore, I suppose this grammatical evolutionary process will always be true, and the English language that will be spoken a few thousand years from now will probably be equally unrecognizable. However, for now, my old high school English teacher from the early 1960s, the late Mrs. Brannon, still lives on, speaking to me in my subconscious, constantly compelling me to point out these things. I’m sure there is a DSM-5 code for the “Brannon scar” on my brain that makes me this way, but I’ve become too old for corrective therapy.
While on the subject of academic quality, I must sadly comment on the death of
Disclosure: Brien reports no relevant financial disclosures.