Endoscopic view of cystic fibrosis with nasal polyposis.

A 25-year-old man with cystic fibrosis (CF) presented with a complaint of increasing nasal congestion. He denied headaches, rhinorrhea, and epistaxis, and he had no history of sinus problems. Of note was the fact that his two siblings had cystic fibrosis, and both had required sinus surgery in the past.

Endoscopic examination revealed the presence of nasal polyposis (figure, A). Computed tomography (CT) of the sinuses demonstrated opacification of the right maxillary and ethmoid sinuses; an expanding, roundish soft-tissue mass in the left ostiomeatal complex; and an almost-clear maxillary sinus on the left side (figure, B).

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The patient elected to undergo endoscopic surgery. Multiple polyps were found in the middle meatus bilaterally. A large polypoid mass originated in the ethmoid area and expanded inferolaterally on the left side. A microdebrider was used to remove the polyps (figure, C). Middle meatal antrostomies were carried out. On suctioning the sinus through the middle meatal window, no inspissated mucus was found (figure, D). The patient's postoperative course was uneventful, and he remained free of polyps 3 years following his surgery.

CF is the most common life-shortening inherited genetic disease in children and young adults of European origin. Most patients exhibit the triad of chronic obstructive pulmonary disease, pancreatic exocrine deficiency, and abnormally high sweat electrolyte concentrations. (1)

Chronic sinusitis--with and without nasal polyposis--is also common in children with CF. (1-4) Sinus CT in these patients will demonstrate several characteristic features (5,6):

* The maxillary sinuses are almost always opacified.

* The ethmoid sinuses may or may not show disease.

* Medial bulging of the medial wall of the maxillary sinus often occurs.

* The uncinate process may be displaced anteromedially by polyps.

* The frontal or sphenoid sinuses are usually hypoplastic.

CF is usually associated with thick mucus in the upper respiratory tract and lungs. (1,2,4) The maxillary sinus is usually filled with hyperplastic mucosa and tenacious purulent secretions. A finding of extensive polyposis without associated sinusitis and inspissated mucus is not common in patients with CF. In the case described here, maxillary and ethmoid sinusitis with nasal polyposis was present on one side, while almost-clear sinuses with nasal polyposis were present on the other side. The presence of polyps can be significant and result in bony remodeling and destruction of the sinus cavities.

Most children with CF and sinusitis can be managed medically. (4) Those with obstructive symptoms from polyposis benefit from surgery. (4-8) Sinus surgery does not improve pulmonary function in CF. Endoscopic surgery with a microdebrider may provide safe management of chronic sinusitis in CF.

References

(1.) Rosenstein BJ. Cystic fibrosis. In: McMillan JA, Feigin RD, DeAngelis CD, Jones MD Jr. Oski's Pediatrics: Principles & Practice. Ch. 236. Philadelphia: Lippincott Williams & Wilkins; 2006.

(2.) Manning SC. Pediatric sinusitis. Otolaryngol Clin North Am 1993;26(4):623-38.

(3.) Cunningham MJ. Pediatric otolaryngology. In: Bailey BJ, Johnson JT, Kohut RI, et al, eds. Head and Neck Surgery--Otolaryngology. Vol 1. Chapter 19. Philadelphia: Lippincott; 1993:233.

(4.) Isaacson G, Yanagisawa E. Cystic fibrosis and sinusitis. Ear Nose Throat J 1998;77(11):886-8.

(5.) Nishioka GJ, Cook PR, McKinsey JP, Rodriguez FJ. Paranasal sinus computed tomography scan findings in patients with cystic fibrosis. Otolaryngol Head Neck Surg 1996;114(3):394-9.

(6.) Gentile VG, Isaacson G. Patterns of sinusitis in cystic fibrosis. Laryngoscope 1996;106(8):1005-9.

(7.) Crockett DM, McGill TJ, Healy GB, et al. Nasal and paranasal sinus surgery in children with cystic fibrosis. Ann Otol Rhinol Laryngol 1987;96(4):367-72.

(8.) Duplechain JK, White JA, Miller RH. Pediatric sinusitis. The role of endoscopic sinus surgery in cystic fibrosis and other forms of sinonasal disease. Arch Otolaryngol Head Neck Surg 1991;117(4):422-6.

Dewey A. Christmas Jr., MD; Joseph P. Mirante, MD, FACS; Eiji Yanagisawa, MD, FACS

From the Department of Otolaryngology, University of South Florida College of Medicine, Tampa, and the Halifax Medical Center, Daytona Beach, Fla. (Dr. Christmas and Dr. Mirante); and the Southern New England Ear, Nose, Throat, and Facial Plastic Surgery Group; the Section of Otolaryngology, Hospital of St. Raphael; and the Section of Otolaryngology, Yale University School of Medicine, New Haven, Conn. (Dr. Yanagisawa).