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Basics ⬇

Basics

Overview

  • Congenital (autosomal recessive) disorder of ciliary dysfunction in the respiratory tract, auditory tubes, ventricles of the brain, spinal canal, oviducts, efferent ducts of the testes, and sperm flagellum.
  • In normal animals ciliary beating is coordinated-characteristically dyskinetic or absent in affected dogs.
  • Hydrocephalus and/or situs inversus (Kartagener syndrome) can be concurrent.
  • Dogs with chronic respiratory disease and situs inversus likely have primary ciliary dyskinesia and do not warrant extensive workup.

Signalment

  • Generally young dogs (<8 weeks) but some are asymptomatic until older (1/2–10 years).
  • Reported predominately in purebred dogs, higher incidence in Bichon frise, Old English sheepdog, but mixed-breed dogs and cats can be affected.

Signs

Historical Findings

  • Chronic sneezing, nasal discharge, coughing, exercise intolerance, and respiratory distress. Dramatic initial response to antibiotics, but relapse after treatment is stopped.
  • Family history-large litters can have >1 affected dog.
  • Infertility in males.

Physical Examination Findings

  • Bilateral mucopurulent nasal discharge.
  • Productive cough.
  • Tachypnea, dyspnea, and cyanosis during exacerbation of infection.
  • Diffuse increase in lung sounds of variable intensity.
  • Heart sounds can be inaudible with severe bronchopneumonia, can be loudest on the right side with situs inversus.

Causes & Risk Factors

Genetics-recessive mutation identified in the Old English sheepdog, inbreeding.

Diagnosis ⬆ ⬇

Diagnosis

Differential Diagnosis

  • Congenital (neutrophil dysfunction, immunoglobulin deficiency) or acquired disease (canine distemper) producing chronic rhinosinusitis and bronchopneumonia.
  • Recurrent aspiration pneumonia.
  • Chronic bacterial pneumonia.
  • Bronchoesophageal fistula.
  • Exposure to cigarette smoke-can delay mucociliary clearance.
  • Mycoplasma spp. or Bordetella infection-can cause acquired ciliary defects.

CBC/Biochemistry/Urinalysis

  • Mature neutrophilic leukocytosis-left-shift and toxic neutrophils with severe bronchopneumonia.
  • Hyperglobulinemia-older dogs.
  • Polycythemia-with chronic hypoxemia.

Other Laboratory Tests

  • Blood gas analysis can reveal hypoxemia with normocapnia or hypocapnia.
  • Bronchoalveolar lavage typically yields mucoid to mucopurulent material characterized cytologically as a purulent exudate with one or more bacterial species isolated on culture. Mycoplasma culture should be requested.
  • Normal sperm motility in an intact male rules out ciliary dyskinesia.

Imaging

Radiography

  • Changes consistent with chronic bronchopneumonia.
  • Situs inversus.
  • Bronchiectasis.
  • Thickened or sclerotic tympanic bullae.

Tracheal Scintigraphy (Mucociliary Clearance)

  • First exclude infections or chronic exposure to cigarette smoke.
  • Affected animals show no movement of radiopharmaceutical from the carina.

Diagnostic Procedures

Electron Microscopy

  • Ultrastructural lesions in the cilia of nasal or bronchial mucosa identified in most patients, including central microtubular disarrangement and loss of dynein arms.
  • Specific lesions must be found in >20% of cilia.
  • Acquired ultrastructural lesions involving <20% of cilia common with chronic respiratory tract infection.
  • Dogs with primary ciliary dyskinesia but no ultrastructural ciliary lesions have been described and require in vitro analysis of ciliary beat frequency and synchrony for diagnosis.

Pathologic Findings

Upper Respiratory Tract

Chronic bacterial rhinitis with mucoid to mucopurulent exudate, and mucosal inflammation, mucous gland hyperplasia, and occasionally hypoplastic nasal turbinates, atresia of the frontal sinuses, frontal sinusitis, and rhinoliths.

Lower Respiratory Tract

Mucoid to mucopurulent material within airways with bronchitis, bronchiectasis, atelectasis, and subpleural emphysema.

Miscellaneous

  • Hydrocephalus.
  • Situs inversus of thoracic viscera or abdominal viscera or both (situs inversus totalis).
  • Impaction of one or both middle ears with a sterile gelatinous material.

Treatment ⬆ ⬇

Treatment

Medications ⬆ ⬇

Medications

Drug(s)

  • Antibiotics based on bacterial culture and sensitivity testing; duration varies with severity of infection.
  • Continuous antibiotic therapy not advised due to colonization with resistant bacteria.

Contraindications/Possible Interactions

Cough suppressants trap secretions and exacerbate airway inflammation.

Anesthesia

  • Patients have impaired gas exchange and increased risk of complications.
  • Minimize respiratory depression and recovery time.

Follow-Up ⬆ ⬇

Follow-Up

Possible Complications

  • Pneumothorax-subpleural cysts, bronchiectatic cysts, interstitial cysts, and emphysematous bullae can develop from prolonged infection or inflammation causing air entrapment and rupture.
  • Pulmonary arterial hypertension, cor pulmonale, and right-sided heart failure can result from chronic hypoxemia.
  • Systemic reactive amyloidosis secondary to persistent bacterial bronchopneumonia.

Expected Course and Prognosis

  • Clinical course of disease and longevity of patients highly variable.
  • Some dogs will become subclinical with age.
  • Appropriate antibiotic treatment and pulmonary physical therapy can result in prolonged survival.

Miscellaneous ⬆

Miscellaneous

See Also

Pneumonia, Bacterial

Author Ned F. Kuehn

Consulting Editor Lynelle R. Johnson

Suggested Reading

Edwards DF, Patton CS, Kennedy JR. Primary ciliary dyskinesia in the dog. Probl Vet Med 1992, 4:291–319.

Merveille AC, Battaille G, Billen F, et al. Clinical findings and prevalence of the mutation associated with primary ciliary dyskinesia in Old English Sheepdogs. J Vet Intern Med 2014, 28(3):771–778.