Radiological Case of the Month

Figure 1. Swallowing study using fluoroscopy shows reflux of contrast from the oropharynx into the nasopharynx and out of the nostrils.

Figure 2. Swallowing study using fluoroscopy showing part of the contrast passed down the esophagus to the stomach and a silent aspiration episode into the trachea.

Definitive diagnosis of velopharyngeal insufficiency (VPI) was made by fiberoptic and nasopharyngoscopy. Initially, this infant had poor sucking, swallowing, and breathing coordination, and needed to rest between sucks. He was treated with cisapride to enhance esophageal motility, and after treatment for a week with parenteral fluids, he began oral feeding using a "cleft palate" nipple. Gradual improvement in feeding tolerance and reduction in episodes of nasopharyngeal reflux occurred over the next week. He still had occasional regurgitation episodes and was diagnosed with failure to thrive by age 3 months. Follow-up fiberoptic nasopharyngoscopy revealed persistent VPI.

Velopharyngeal insufficiency represents inability to separate the content of the oropharynx from the nasopharynx during feeding and speech by the muscles of the palate and the posterior and lateral pharyngeal wall. It is a relatively uncommon diagnosis in children and has not been reported in the neonate.¹ Velopharyngeal insufficiency may be organic or functional, produced by congenital or acquired causes or due to paresis or local disorders. It most frequently occurs as a result of cleft palate, submucous cleft palate (any combination of bifid uvula, muscular diastasis of soft palate, or bony defect of the hard palate) or following adenoidectomy.¹ Other entities associated with VPI are DiGeorge syndrome, velocardiofacial syndrome, neurofibromatosis, hemifacial microsomia (facial-auriculovertebral malformation complex),² Chiari malformation,³ myasthenia gravis, histiocytosis (eosinophilic granuloma), Pierre Robin syndrome, Dubowitz syndrome,⁴ Kabuki make-up syndrome,⁵ encephalopathy, peritonsillar abscess, Möbius syndrome, and myotonic dystrophy.⁶ Familial cases of VPI with autosomal dominant inheritance have been reported.⁷ Velopharyngeal insufficiency may be complicated by speech hypernasality, snoring,⁸ Eustachian tube dysfunction, and chronic otitis media with conductive hearing loss and paranasal sinus infections.⁹

Velopharyngeal insufficiency may be diagnosed by multiview videofluoroscopy¹⁰ but the gold standard for diagnosis is flexible fiberoptic nasal endoscopy.¹¹ Rapid magnetic resonance imaging is a costly but sensitive method to evaluate VPI.¹² Treatment modalities include speech therapy for minimal VPI and dental prosthesis for cases in which surgery is contraindicated. The mainstay of treatment for posterior pharyngeal wall augmentation includes using materials such as paraffin, fascia, autologous cartilage, and Teflon (DuPont, Wilmington, Del) paste.¹³ If these measures fail, a push-back palatoplasty can be attempted. Other surgical procedures used are sphincter pharyngoplasty and palatopharyngoplasty (velopharyngeal flap).¹⁴ Obstructive sleep apnea has been reported as a complication of pharyngeal flap surgery.

Accepted for publication February 18, 1999.

Reprints: Oussama Itani, MD, Director of Neonatology, Borgess Medical Center, 1521 Gull Rd, Kalamazoo, MI 49048.