Hereditary hemorrhagic telangiectasia, known as Osler’s disease, is an autosomal-dominant mucocutaneous and visceral fibrovascular disorder characterized by the classic triad of telangiectasia, recurrent epistaxis, and a family history of the disorder. Due to the fragility of the blood vessels, mild trauma can instigate the onset of bleeding. Any trauma, however slight, may result in bleeding that can be profuse in the absence of normal vasoconstriction. Over the years a multitude of different treatment options have been described for control of the epistaxis in patients with Osler’s disease. Nasal dermoplasty is one of the most effective options with a time limitation due to grafted skin atrophy and vascularization. Hormone therapy is also effective for the control of recurrent epistaxis and is considered to be a maintenance therapy. The problems with hormone therapy include side effects such as weight gain, gynecomastia, loss of libido, thromboembolic diseases, and myocardial infarction. Considering these possible side effects under the systemic hormone treatment, topical application of 0.1% estriol as a nose ointment is an alternative treatment.
A 72-year-old man with Osler’s disease who suffered from severe epistaxis underwent nasal dermoplasty twice, but the transplanted skin failed to be engrafted at the perforation of the nasal septum, and the mucosa was partially exposed. Naked mucosa caused mild epistaxis and the patient was instructed to apply 0.1% estriol nose ointment twice a day. Within 3 months after topical 0.1% estriol application, epistaxis had disappeared and the surface of the nasal mucosa changed to epithelial metaplasia. No side effects from the use of topical 0.1% estroiol were observed.
We found that topical application of 0.1% estriol ointment served as a protective film on the nasal mucosa covered by a thick epithelial layer, making patients less vulnerable to local trauma. Furthermore, this treatment caused none of the systemic side effects associated with hormone therapy. Further observation is necessary, but short-term results are likely in patients with Osler’s disease by topical 0.1% estriol application.

View full abstract

Foreign bodies in the maxillary sinus are usually a result of iatrogenic effects or trauma. We present a 52-year-old man with a foreign body in the maxillary sinus that was discharged into the nasal cavity. The patient complained of left cheek pain after dental treatment. X-ray and computed tomography (CT) examinations showed a radiopaque linear shadow in the left maxillary sinus. We diagnosed this as a foreign body in the maxillary sinus. In this patient, the foreign body was discharged into the middle nasal meatus without surgery after sixteen months. Based on this, we conclude that a foreign body in the maxillary sinus can be discharged into the nasal cavity.

View full abstract

Patients diagnosed with obstructive sleep apnea syndrome (OSAS) between January 2009 and September 2011 in our hospital were examined for the presence of allergic rhinitis, and after excluding those with seasonal allergic rhinitis, 112 patients were enrolled in the study. Examinations showed that 59% of OSAS patients also had allergic rhinitis, and comparison to those without allergic rhinitis revealed that there was a significant difference in the degree of nasal airflow. We also conducted polysomnography to compare the sleep conditions of patients. Patients with allergic rhinitis had significantly longer sleep latency and a slightly higher. These results showed that allergic rhinitis is associated with OSAS and might be a factor contributing to sleep disruption, thus exacerbating sleep conditions and the severity of OSAS in these patients. The results also indicate that treatment and improvement of allergic rhinitis and nasal obstruction will likely improve sleep apnea and the quality of sleep.

View full abstract

Introduction: For the treatment of sinus mucoceles, simple intranasal drainage and marsupialization have been advocated. However, postoperative re-stenosis of the outflow tracts of mucoceles may occur. We have used a modified silicone sheet to maintain the patency of mucocele outflow tracts, and here report on the usefulness of this method.
Subjects and Methods: Nineteen cases (17 patients) of mucoceles, 11 (10 patients) involving the maxillary sinus, 4 (3 patients) the frontal sinus, and 4 (4 patients) the sphenoid sinus, were treated. After marsupialization of the sinus mucocele under endoscopic surgery, a silicone sheet, shaped to prevent dislodgement or migration into the sinus, was inserted. The sheet was removed in the office 12-24 weeks postoperatively, and outflow tract size was followed-up for at least 6 months.
Results: Two representative cases are shown: the first involved secondary sphenoid sinus mucoceles, and the other was a primary frontal sinus mucocele. In the first case, sphenoid sinus mucoceles were seen bilaterally. A silicone sheet was inserted only on the left side of the sphenoid sinus. After 10 months, the left-side outflow tract of the sphenoid sinus was well preserved, while the right-side tract was closed. In the second case, the nasofrontal passageway was well preserved, with an osteitic bone in the frontal recess. Overall, this method resulted in a high patency rate of mucocele outflow tracts. However, in 2 of 4 frontal sinus mucoceles and 1 of 11 maxillary sinus mucoceles, the silicone sheets dislodged spontaneously within 3 months postoperatively.
Conclusion: Silicone sheets may be better than firm tubes or catheters with respect to the patency rates of mucocele outflow tracts. However, in frontal sinus mucoceles, some modifications will be needed to prevent spontaneous dislodgement.

View full abstract

Sinus puncture and aspiration are often performed for maxillary sinusitis. We report a 76-year-old woman complaining of left cheek pain and purulent rhinorrhea, on whom we performed sinus puncture and aspiration using Sinoject^®. Acute maxillary sinusitis made hospitalization necessary, but chemotherapy did not improve cheek pain and laboratory findings worsened. We therefore performed sinus puncture using Sinoject^® via the inferior meatus with an indwelling tube. Pus in the maxillary sinus could not be irrigated because of cheek pain, despite successful aspiration. A second tube via the inferior meatus was indwelled and a large amount of pus was drained. 4 days of maxillary sinus irrigation through a tube led to improvement. The need for sinus puncture and aspiration has decreased, because of advancements in endoscopic sinus surgery, however, a procedure for sinus puncture in patients who cannot be hospitalized for surgery is needed. The Sinoject^® is a spring-activated instrument specifically for sinus puncture. It is used to insert a tube into the maxillary sinus. When narrowing or obstruction of the middle meatus prevents irrigation of the maxillary sinus, the placement of 2 tubes through the inferior meatus is useful.

View full abstract

The incidence of fungal paranasal sinusitis has increased in recent years because of societal aging and steroid administration. We report 36 patients with fungal paranasal sinusitis who underwent endoscopic sinus surgery (ESS) at Kurashiki Central Hospital between January 2006 and October 2011. The patients comprised 15 males and 21 females aged 27 to 93 years, with the mean age of 63. The primary fungal lesion was in the maxillary sinus in 27 patients, the sphenoid sinus in 5, the ethmoid sinus in 1, and multiple sinuses in 3.
Thirty-four cases were diagnosed by histopathological findings, while fungus was detected in 3 of 25 (12%) laboratory cultures. Aspergillus was detected in 35 cases, and no mycete was detected either histological findings or laboratory culture in one case. No recurrence has been seen to date, except one case of acute invasive fulminant type.
The ESS procedures were conducted with opening of the affected paranasal sinuses and removal of fungal masses. All patients with maxillary fungal sinusitis underwent mid-meatus antrostomy and inferior meatus antrostomy was added in two cases due to fungus balls at the bottom or anterior side of the maxillary sinus. Adequate opening of the infected sinuses and complete removal of fungal masses are considered essential in the treatment of fungal paranasal sinusitis.

View full abstract

Allergic fungal rhinosinusitis (AFRS) is a relatively new and incompletely understood clinical entity of chronic rhinosinusitis. It is intractable and recurrence is often seen after endoscopic sinus surgery. There have been few cases of AFRS in Japan, and awareness thus remains low.
We report a case of AFRS with local bone alternation and expansive encroachment into the skull base and orbits. A 29-year-old man presented with diplopia, right exophthalmos and bilateral nasal discharge and obstruction. Bone destruction of the right orbit medial wall and anterior skull base were suspected on CT scan evaluation. The pathological examination revealed marked eosinophilic infiltration in the sinus mucosa and accumulation of allergic mucin with fungus in the paranasal cavities. Evaluation of the patient using the Bent-Kuhn diagnostic criteria for AFRS allowed a positive diagnosis. Endoscopic sinus surgery was performed with a navigation system, and thick allergic mucin was removed from all of the sinuses.
Pathological examination of the paranasal sinus contents and fungal inspection are necessary for AFRS diagnosis. It is important to be aware of the possibility of AFRS in diagnosis and treatment of chronic sinusitis in routine clinical practice.

View full abstract

X-linked lymphoproliferative syndrome (XLP) is a congenital immunodeficiency which involves a defective specific immune response to Epstein-Barr virus (EBV).
We report a case of EBV-related lymphoproliferative disorder associated with an XLP primary lesion in the nasal sinus.
A 3-year, 9-month-old boy was being followed as an outpatient on suspicion of EBV infection, and onset of EBV encephalitis led to admission to the Paediatrics Department. He was started on multiagent chemotherapy, however this was not effective, leading to a congenital immunodeficiency being suspected. XLP was diagnosed using a genetic test 61 days after admission. On day 95, a brain MRI revealed a solid lesion spanning from the right nasal cavity to the maxillary sinus. Hemorrhage from the same lesion led to a referral to our department on day 125. A pale red hemorrhagic mass was observed in the right nasal cavity, and biopsy allowed a diagnosis of CD20+ EBV-related lymphoproliferative disorder. On day 143, rituximab was started, but on day 151, the patient died due to worsening systemic condition.
XLP is a disease with a poor prognosis. Over 70% of patients die by the age of 10 due to fatal infectious mononucleosis. Malignant lymphoma was associated in 30% of XLP cases, however, a literature search revealed no reports involving primary lesions of the nasal sinus.
Reports describe good results for hematopoietic stem cell transplantation, with this becoming the main focus for the treatment of XLP.

View full abstract