International Journal of Pediatric Otorhinolaryngology Extra
Volume 3, Issue 3 , Pages 132-135, September 2008

Isolated acute sphenoiditis with visual loss: A rare disorder in pediatric patients

  • Marco Berlucchi

      Affiliations

    • Department of Pediatric Otorhinolaryngology, Spedali Civili, Piazza Spedali Civili 1, 25123 Brescia, Italy
    • Corresponding Author InformationCorresponding author. Tel.: +39 0303996226.
    • ,
  • Manuela Rossini

      Affiliations

    • Department of Pediatric Otorhinolaryngology, Spedali Civili, Piazza Spedali Civili 1, 25123 Brescia, Italy
    • ,
  • Maria P. Bondioni

      Affiliations

    • Department of Pediatric Radiology, University of Brescia, Brescia, Italy
    • ,
  • Piero Nicolai

      Affiliations

    • Department of Otorhinolaryngology, University of Brescia, Brescia, Italy

Received 31 December 2007; accepted 18 January 2008. published online 07 March 2008.

Article Outline

Summary 

Although inflammatory disease of the sinuses is a common pathology in pediatric patients, isolated acute sphenoiditis is a rare disorder and its diagnosis is difficult due to the non-specific clinical picture. Since the sphenoid sinus has anatomical relationships with several vital structures, any delay of correct diagnosis, and therefore of prompt and adequate treatment, can result in severe and life-threatening complications. Broad-spectrum antibiotic therapy is usually sufficient to resolve sphenoiditis, but when symptoms persist or complications occur, surgical drainage of the sinus is mandatory. To date, several surgical approaches are available to drain the sphenoid sinus. We report the history of an 11-year-old girl with sphenoiditis complicated with unilateral visual loss treated with transnasal endoscopic surgery, which led to complete resolution of symptoms. Furthermore, the importance of correct diagnosis and treatment of this rare disease is also highlighted.

Keywords: Sphenoiditis, Visual loss, Pediatric sinusitis, Complications, Endoscopic sinus surgery

 

Back to Article Outline

1. Introduction 

Rhinosinusitis is a common inflammatory disorder in a pediatric age. This disease, which frequently occurs in association with upper respiratory tract infection, generally involves the anterior ethmoid or maxillary sinus in 78.3% and 56.1% of cases, respectively [1]. Isolated acute sphenoiditis rarely occurs; however it is challenging to diagnose because of the non-specific symptoms and paucity of clinical findings. For this reason, this uncommon pathological entity is often misdiagnosed [2]. Any delay in its diagnosis and treatment can result in severe and life-threatening complications such as meningitis, pituitary abscess, peri-orbital cellulitis, orbital cellulitis, orbital abscess, optic neuritis, carotid artery thrombosis, and cavernous sinus thrombosis [2], [3], [4], [5], [6]. Herein, we report the case of a pediatric patient with isolated acute sphenoiditis complicated with right visual loss successfully treated with transnasal endoscopic surgery.

Back to Article Outline

2. Case report 

In December 2000, an 11-year-old girl was admitted to the Department of Pediatric Neuropsychiatry, Spedali Civili, Brescia, Italy for right visual loss and mild frontal headache. Ophthalmologic and neuropsychiatric evaluation as well as a fluoroangiography were unremarkable. No immunologic deficiencies were found. MR revealed a large left sphenoid sinus that had anatomic relationship with both optic nerves. This sinus, on spin-echo T1-weighted sequences after contrast media administration, appeared completely occupied by homogeneously hypointense tissue associated with hypertense septa (Fig. 1). No lesions of the central nervous system were observed. For this reason, the child was transferred to the Department of Pediatric Otorhinolaryngology.

At admission, left nasal endoscopy showed a white-gray secretion in the superior meatus probably coming from the sphenoid sinus. The remaining otorhinolaryngologic evaluation was normal. CT-scan of sinuses showed complete opacification of the left sphenoid sinus without erosion of its bony wall or invasion of adjacent structures (Fig. 2). These radiological findings were suspicious for acute sphenoid sinusitis. Paraseptal transnasal endoscopic sphenoidotomy was performed associated with IV antibiotic therapy (ampicillin/sulbactam). Bacterial studies performed on sphenoid muco-purulent secretions were negative. The postoperative course was uneventful. She was discharged 3 days after surgery with complete resolution of visual loss and given amoxicillin with clavulanate (25mg/kg ×2/day) and daily fluticasone nasal spray for 10 days. At the time of writing, the sphenoidotomy remains patent and she is symptom-free 7 years after endoscopic surgical treatment.

  • View full-size image.
  • Fig. 2. 

    Coronal CT-scan shows the left sphenoid sinus completely filled with homogeneous hypodense tissue (asterisk). No erosions of the sinus walls are evident. Both optic nerves are in anatomical relationship with this sinus.

Back to Article Outline

3. Discussion 

Sphenoiditis usually occurs in association with inflammation of the maxillary and ethmoidal sinuses; isolated acute sphenoid sinus infections are rare disorders in pediatric patients. In a study of 1087 patients affected by sinusitis, the incidence of isolated sphenoiditis was 2.7% [3] and, in two different studies over a 10-year period, Hanatuk et al. [7] and Lahat et al. [2] observed only 3 and 10 cases, respectively.

Several disorders such as maxillo-facial fractures, diabetes, immunodeficiency, cocaine abuse, paranasal tumors, previous radiotherapy, and anatomical variations altering the drainage of the sphenoid sinus such as intersinus septa variation, abnormally placed or small ostium, and hypertrophic superior turbinate are considered predisposing factors for sphenoid infection. Moreover, diving and/or swimming accompanied by forceful water entry into the sinus can also favor isolated sphenoiditis [8], [9], [10]. None of these predisposing factors was identified in our case.

The most frequently isolated pathological organisms in these illnesses are gram-positive microorganisms such as Staphylococcus aureus, various Streptococcus sp. (Streptococcus pneumoniae is the predominant agent), and Hemophilus influenzae, whereas fungi, in particular Aspergillus, are mainly observed in immunocompromised patients [3], [8], [11]. Finally, a viral origin, as was likely in our patient, has been advocated when surgical sphenoid sinus aspirates are negative for bacterial pathogens (about 20% of cases) [12].

The clinical picture of this disease is not specific and includes mainly headache. This symptom that usually is refractory to analgesics such as acetylsalicylic acid and codeine can be either dull and constant or sharp and intermittent with a variable localization (i.e., frontal, temporal, retro-orbital, occipital, or vertex region). Moreover, it can interfere with sleep and may be associated with fever, purulent rhinorrhea, and nuchal rigidity [2], [3], [11], [13], [14]. When complications (i.e., meningitis, pituitary abscess, peri-orbital cellulitis, orbital cellulitis, orbital abscess, optic neuritis, carotid artery thrombosis, and cavernous sinus thrombosis) occur, patients also complain of facial pain, paresthesia at the level of V1, V2, or V3 area, sixth nerve palsy, ocular signs and symptoms (i.e., blurred vision, photophobia, diplopia, eye tearing, proptosis, visual loss, and ptosis), and mental status changes [2], [3], [4], [5], [6], [9], [12], [13], [15], [16]. These complications are due to the anatomical relationship of the sphenoid sinus with close vital structures such as the middle cranial fossa, hypophysis, superior orbital fissure, optic canal, and the cavernous sinus which contain the internal carotid artery, the third, fourth, fifth, and sixth cranial nerves. Thus, when infection of sinus spreads to these structures, it may mimic other neurological disorders, thus delaying correct diagnosis and appropriate treatment.

The optic nerve is commonly located along the superior-lateral wall of sphenoid sinus and its dehiscence into this sinus has been observed in 4% of cadaverous studies. Moreover, a bony wall between the sinus and the optic nerve less than 0.5mm has been identified in 78% of cadavers [17], [18]. All these anatomical variants might favor the spread of sphenoid infection to this important nerve putting a patient at risk for significant ocular disorder. In 2004, Lee et al. [19] theorized that visual loss in patients affected by sphenoiditis could result by an optic nerve ischemia due to infection, vasculitis, or thrombophlebitis. In the present case, no previously cited paranasal anatomical variants, which might block sphenoid sinus drainage supporting optic neuritis, were observed either at preoperative imaging or during surgery; for this reason, we believe Lee's hypothesis might help to explain the pathogenesis of visual loss.

Diagnostic work-up of sphenoiditis first includes a careful analysis of the clinical picture and nasal endoscopy using 0° and 45° Hopkins telescopes. Performed after local anesthesia associated with nasal decongestion, endoscopic procedures might reveal purulent secretions in the superior meatus and/or nasal anatomical variants blocking sphenoid ostium. CT-scan of sinuses represents the next diagnostic step and it is considered the gold standard instrumental procedure to assess inflammatory disease of sinuses. This diagnostic study, which is performed in axial sections with coronal and sagittal reconstructions, may display total or partial opacification of sphenoid sinus associated with air-fluid levels. Moreover, it provides important information about possible erosion of sphenoid bony wall, and paranasal anatomic anomalies favoring optic neuritis [1], [9], [10]. MR imaging with contrast medium is recommended when complications manifest and/or other soft tissue lesions are suspected [9], [10], [20]. By MR, sphenoid inflammatory lesions have usually a high, homogeneous signal intensity and low signal intensity on T2- and T1-weighted images, respectively. Furthermore, it is superior to CT for estimation of intraorbital and/or intracranial inflammatory spread. Our patient first underwent MR of the brain that revealed a large left sphenoid sinus filling by pathologic tissue and a relationship with both optic nerves at another institute. When she was admitted at our department an urgent CT of sinuses was performed to evaluate bony wall status of the aforementioned paranasal sinus as this imaging is the ideal technique to assess bony erosion.

Antibiotic treatment should be started immediately in uncomplicated acute sphenoiditis. Such prompt therapy is necessary to avoid potential severe morbidity and mortality [3], [11], [15]. In 1983, Lew et al. [3] reported a patient cohort affected by acute sphenoid sinusitis who developed serious complications when their management was delayed. Four patients in this series died and another four patients experienced irreversible injury of the second, third, and fifth cranial nerves.

An adequate antibiotic coverage, which is administrated parenterally for at least 2 weeks, usually includes high dose of anti-staphylococcal agents such as clindamycin and a second or third generation cephalosporin (i.e., cefotaxime) sometimes associated with metronidazole [2], [3], [10], [11], [21]. Furthermore, topical nasal decongestants and saline irrigation may be helpful to promote sphenoidal drainage [9], [10].

When the clinical picture persists or worsens, or when signs and symptoms of dangerous complications manifest, immediate surgical drainage of the sphenoid sinus is mandatory [2], [9], [11]. In 1995, Postma et al. [9] described a 17-year-old male affected by acute sphenoiditis associated with bilateral visual loss and complete abducens nerve paralysis. He underwent sublabial transseptal sphenoidotomy plus intravenous ceftizoxime, tobramycin, and nafcillin. All cultures studies were negative and pathological examination revealed only acute inflammation. After surgery the patient's vision returned to normal, but bilateral sixth cranial nerve palsy persisted. In the present case, we carried out immediate drainage of the sphenoid sinus. Among several surgical procedures to drain the sphenoid sinus such as washout through the natural ostium, puncture, and washout through the anterior wall of the sinus, intranasal sphenoidostomy, trans-septal sphenoidectomy, trans-ethmoidal sphenoidectomy, and trans-antral sphenoidectomy [4], an intranasal endoscopic approach associated with IV antibiotic therapy was performed. In the last years, the advent of high-resolution rigid endoscopes and the development of endoscopic instrumentation allow to consider endoscopic sinus surgery the standard surgical technique to drain sphenoid sinus. In the present case, endoscopic treatment was safe, less invasive, and allowed complete resolution of visual loss within 24h of intervention.

Back to Article Outline

4. Conclusion 

To our knowledge, the present case is the second pediatric patient with acute sphenoid sinusitis complicated by visual loss reported to date. Early diagnosis of sphenoiditis and its prompt, broad-spectrum antibiotic treatment are the key to avoid involvement of close vital structures. When symptoms persist or complications occur, surgical drainage is mandatory. Endoscopic surgery is currently considered the ideal approach for sphenoid sinus drainage. The present case is the first sphenoiditis associated with visual loss successful treated by transnasal endoscopic surgery in a pediatric patient.

Back to Article Outline

References 

  1. Turgut S, Ozcan KM, Celikkanat S, Ozdem C. Isolated sphenoid sinusitis. Rhinology. 1997;35:132–135
  2. Lahat E, Danieli J, Cohen H, Paret G, Barzilai A. Acute isolated sphenoid sinusitis in children. Pediatr. Infect. Dis. J. 1997;16:1180–1182
  3. Lew D, Southwick FS, Montgomery WW, Weber AL, Baker AS. Sphenoid sinusitis. A review of 30 cases. N. Engl. J. Med. 1983;309:1149–1154
  4. Dale BAB, Mackenzie IJ. The complications of sphenoid sinusitis. J. Laryngol. Otol. 1983;97:661–670
  5. Holt GR, Standefer JA, Brown WE, Gates GA. Infectious diseases of the sphenoid sinus. Laryngoscope. 1984;94:330–334
  6. Deans JAJ, Welch AR. Acute isolated sphenoid sinusitis: a disease with complications. J. Laryngol. Otol. 1991;105:1072–1074
  7. Hanatuk LAP, Macdonald RE, Papsin BC. Isolated sphenoid sinusitis: the Toronto Hospital for sick Children experience and review of the literature. J. Otolaryngol. 1994;23:36–41
  8. Abramovich S, Smelt GJC. Acute sphenoiditis alone and in concert. J. Laryngol. Otol. 1982;96:751–757
  9. Postma GN, Chole RA, Nemzek WR. Reversible blindness secondary to acute sphenoid sinusitis. Otolaryngol. Head Neck Surg. 1995;112:742–746
  10. Tan HKK, Ong YK. Acute isolated sphenoid sinusitis. Ann. Acad. Med. Singapore. 2004;33:656–659
  11. Cohen YH, Amir J, Zeharia A, Danziger Y, Ziv N, Mimouni M. Isolated sphenoidal sinusitis in children. Eur. J. Pediatr. 1999;158:298–304
  12. Goldman GE, Fontanarosa PB, Anderson JM. Isolated sphenoid sinusitis. Am. J. Emerg. Med. 1993;11:235–238
  13. Kibblewhite DJ, Cleland J, Mintz DR. Acute sphenoid sinusitis: management strategies. J. Otolaryngol. 1998;17:159–163
  14. Marseglia GL, Pagella F, Licari A, Scaramazza C, Marseglia A, Leone M, et al. Acute isolated sphenoid sinusitis in children. Int. J. Pediatr. Otorhinolaryngol. 2006;70:2027–2031
  15. Urquhart AC, Fung G, McIntosh WA. Isolated sphenoiditis: a diagnostic problem. J. Laryngol. Otol. 1989;103:526–527
  16. Ada M, Kaytaz A, Tuskan K, Guvenc MG, Selcuk H. Isolated sphenoid sinusitis presenting with unilateral VIth nerve palsy. Int. J. Pediatr. Otorhinolaryngol. 2004;68:507–510
  17. Renn WH, Rhoton AL. Microsurgical anatomy of the sellar region. J. Neurosurg. 1975;43:288–298
  18. Fujii K, Chambers SM, Rhoton AF. Neurovascular relationships of the sphenoid sinus: a microsurgical study. J. Neurosurg. 1979;50:31–39
  19. Lee LA, Huang CC, Lee TJ. Prolonged visual disturbance secondary to isolated sphenoid sinus disease. Laryngoscope. 2004;114:986–990
  20. Nemzek W, Postma G, Poirier V, Hecht S. MR features of pachymeningitis with six-nerve palsy secondary to sphenoid sinusitis. Am. J. Neuroradiol. 1995;16:960–963
  21. Brook I. Bacteriology of acute and chronic sphenoid sinusitis. Ann. Otol. Rhinol. Laryngol. 2002;111:1002–1004

PII: S1871-4048(08)00007-5

doi:10.1016/j.pedex.2008.01.005

International Journal of Pediatric Otorhinolaryngology Extra
Volume 3, Issue 3 , Pages 132-135, September 2008

Article Tools

* Image Usage & Resolution