Genetic Welfare Problems of Companion Animals

Shar Pei

Brachycephalic Airway Obstruction Syndrome (BAOS)

Related terms: Brachycephalic Onstructive Airway Syndrome (BOAS), Brachycephalic Airway Syndrome (BAS), Brachycephalic Airway Disease (BAD), Brachycephalic disease

Outline: The short-nosed shape of Shar pei causes breathing difficulties and ‘knock-on’ effects which cause chronic discomfort and prevent normal exercise.

Summary of Information

(for more information click on the links below)

1. Brief description

Brachycephalic Airway Obstruction Syndrome (BAOS) occurs in all dog breeds with brachycephaly. The Shar Pei is affected by this to some extent but less often and less severely than dogs of breeds with more extreme brachycephaly. Brachycephaly is characterised by greatly shortened upper jaws and noses, but the soft tissues of the nose and throat remain unchanged, thus these tissues are squeezed into a substantially smaller space. This leads to narrowing and increased resistance in the respiratory system. BAOS describes the clinical signs seen due to this. The main congenital abnormalities (those present from birth) are stenotic nares (abnormally narrowed nostrils), excessively long soft palate in relation to head shape and tracheal hypoplasia (abnormally narrowed windpipe). The permanent narrowing and obstruction of the airways makes breathing harder. The constantly increased respiratory effort leads to secondary changes which further narrow the air passages, including collapse of the larynx (the voice box, the opening to the windpipe). BAOS leads to snoring, respiratory noise, mouth breathing, respiratory distress with rapid breathing and struggling for breath, and can lead to collapse and death. Dogs with BAOS are unable to take even moderate amounts of exercise, are very prone to heat stroke and have constantly disrupted sleep.

2. Intensity of welfare impact

BAOS can cause major welfare problems with bouts of severe respiratory distress in dogs that are severely affected. Even mildly affected dogs are likely to suffer disrupted sleep and be unable to run and exercise normally due to their compromised breathing.

3. Duration of welfare impact

BAOS is a progressive, life-long condition. BAOS can affect immature dogs under 6 months of age.

4. Number of animals affected

We are unaware of data on the proportion of Shar peis affected by the complications of BAOS but a brachycephalic head shape is part of the breed standard and many may be affected to some degree.

5. Diagnosis

BAOS may be suspected in any Shar pei showing the typical signs associated with it, as the breed is predisposed to the condition. However, specific diagnosis of most of the abnormalities, that together form the syndrome, requires examination under anaesthetic, plus radiographs (x-rays) and possibly endoscopy (examination of the airways with a fibre optic tube). These procedures have to be performed under anaesthesia, which creates a dilemma as dogs with BAOS have a substantially increased risk of dying under anaesthesia because of their respiratory abnormalities.

6. Genetics

BAOS is caused by brachycephaly. This inherited defect is a consequence of selecting for the particular abnormalities of the head which is part of the standard for the breed.

7. How do you know if an animal is a carrier or likely to become affected?

Many Shar pei are affected with BAOS to some degree. Examination of any puppy prior to purchase is essential, along with its dam and sire. To avoid the risk of perpetuating the welfare problems associated with this condition, dogs showing signs of BAOS, or whose parents have any signs or have had surgical procedures to alleviate the condition should not be purchased.

8. Methods and prospects for elimination of the problem

As BAOS is due to the brachycephalic head shape, it seems unlikely that it will be possible to eliminate the condition without changing this conformation (and the breed standard). This may involve out-crossing with dogs of non-brachycephalic breeds. Opinions differ as to whether it is ethically acceptable to breed animals whose welfare is likely to be compromised.

For further details about this condition, please click on the following:
(these link to items down this page)

• Clinical and pathological effects
• Intensity of welfare impact
• Duration of welfare impact
• Number of animals affected
• Diagnosis
• Genetics
• How do you know if an animal is a carrier or likely to become affected?
• Methods and prospects for elimination of the problem
• Acknowledgements
• References

1. Clinical and pathological effects

The term “brachycephaly” comes from the Greek words meaning short and head. The term is applied to all breeds of dog (and cat) with this abnormality. Some brachycephalic breeds include Pugs, English bulldogs Pekingese, French bulldogs, Lhasa apsos, Shih tzus, Boston terriers and, in cats, Persians.

The brachycephalic head shape is due to an inherited defect in the development of the bones of the skull (Stockard 1941). The head is of a normal width but the length is markedly reduced. However, the soft tissues of the head are not correspondingly reduced in size and have to be squeezed into a smaller, shortened space. This affects the passage of air into the lower airways and lungs (Harvey 1989).

Brachycephalic Airway Obstruction Syndrome (BAOS) is a result of breeding for a shortened facial appearance. Most brachycephalic dogs are affected by upper airway obstruction to some degree (Brown and Gregory 2005). The airways are narrowed in several places due to the shortened facial features and the resultant increased airflow resistance, so affected dogs have to use excessive effort to breathe (Shell 2008). BAOS is the term given to this collection of anatomical effects, the secondary anatomical changes they cause and the clinical signs that result.

There are a number of congenital anatomical abnormalities that result in BAOS and which contribute to the breathing problems:

1. Stenotic Nares (abnormally narrow nostrils) – This usually affects both nostrils, and when present, adds to occlusion of the airways, increasing the effort needed to breathe in. This extra effort has a further impact of pulling the soft palate deeper into the larynx (the opening to the airway) and blocking the airway opening further (Shell 2008). Distorted nasal passages are also thought to cause some occlusion of the larynx (voice box) (Hendricks 1995).
2. Elongated soft palate – It is believed that the genetic defect responsible for shortening the nose does not correspondingly affect the soft tissues of the head, so that the soft palate (and tongue) at the back of the mouth appear disproportionately large (Venker-van Haagen 1995); in a dog of the same size with a normal head shape, they would be the correct length. In the brachycephalic dog, however, the overlong soft palate extends backwards, partly obstructing the larynx (Hendricks 1995). The elongated soft palate also increases airflow turbulence and rubs against other tissues, such as the larynx, leading to inflammation and swelling of airway tissues (Torrez & Hunt 2006). As a result of this inflammation, the soft palate itself, in time, becomes swollen and thickened, further blocking the airways (Shell 2008).
3. Tracheal hypoplasia (Abnormally narrow windpipe relative to the size of dog) –Tracheal hypoplasia is often seen in conjunction with the abnormalities described above. (It can also occur in association with other abnormalities not considered to be part of BAOS, including megaoesophagus - abnormally enlarged oesophagus (Coyne and Fingland 1992)). Opinions differ about the extent to which tracheal hypoplasia plays a part in BAOS. It has been suggested that tracheal hypoplasia worsens the prognosis for dogs with BAOS (Orsher 1993, Monnet 2003) and causes chronic or recurrent lower airway and lung infections (Harvey 1989).
4. Laryngeal hypoplasia (abnormally small, underdeveloped larynx) - In this condition the cartilages that form the larynx are soft and underdeveloped in shape, and the abductor muscles that open the larynx do not function properly (Venker-van Haagen 1995). This is a common cause of laryngeal collapse in brachycephalic dogs according to Venker-van Haagen (1995), though other authors do not to discuss this condition as part of BAOS. Burbidge et al (1988) recorded a case of BAOS with laryngeal malformation and suggested that this may be seen in other cases of BAOS.

Dogs with BAOS usually have some combination of the above defects (Brown and Gregory 2005) and most have more than one defect (Fasanella et al 2010). These anatomical defects result in increased respiratory effort leading, in time, to a series of secondary problems. These include:

• Collapse of the larynx - The reduced pressure in the pharyngolaryngeal (throat) region, as a result of the obstructions described above, ultimately results in distortion and collapse of the larynx (Wykes 1991). Three stages of laryngeal collapse have been described:
  • In stage I laryngeal collapse, the laryngeal saccules (small sac-like structures in the larynx) balloon and turn inside out. Their eversion adds to the airway obstruction.
  • In stage II there is loss of rigidity and medial displacement of the cuneiform processes of the arytenoid cartilage (part of the cartilage that form the support structure of the larynx) which collapse inwards.
• In stage III, there is collapse of the corniculate processes of the arytenoid cartilages - this involves further collapse of the laryngeal structures blocking the airway (Pink et al 2006).
• Bronchial collapse. Laryngeal collapse has been found to be significantly associated with bronchial collapse - collapse of the ranching airways in the lungs (De Lorenzi et al 2009).
• Eversion and hypertrophy of the tonsils – Inflammation of the soft tissues in the throat area leads to the tonsils enlarging, adding to the amount of tissue protruding into the back of the throat) (Fasanella et al 2010).
• Hypertrophy of the pharyngeal muscles – The muscles in the throat become larger and thicker as they work harder to keep the pharynx, at the back of the throat, open to enable the passage of air into the lungs. However, this hypertrophy of the muscles further narrows the pharyngeal cavity.
• Gastrointestinal problems: dysphagia, regurgitation, vomiting and acid reflux – Dysphagia is defined by Poncet et al (2005) as difficult or painful swallowing that can be associated with regurgitation  of the contents of the oesophagus (often saliva in BAOS) and vomiting . Dysphagia in brachycephalic dogs has been reported by many authors (eg Ducarouge 2002, Dupre and Freiche 2002, Koch and others 2003). Poncet et al (2005) also noted many reports of anatomical defects in the gastrointestinal tract of brachycephalic dogs. For example, hiatus hernias (in which part of the stomach can pass up into the chest through a larger than normal hole in the diaphragm, and pyloric stenosis (in which the stomach exit is narrowed preventing food leaving the stomach normally). They found a correlation between the severity of BAOS and of the gastrointestinal disease and postulated that the severity of respiratory signs affected the severity of gastrointestinal signs and vice versa.
• Heart Failure – This occurs secondarily to inadequate oxygenation of the blood in the lungs, due to the constant obstructions of the airways. In response to inadequate blood oxygen concentration, there is constriction of the small blood vessels in parts of the lung that are poorly ventilated. Chronic (long term) vasoconstriction and increased blood pressure in the lungs results in increased blood pressure in the right side of the heart and this can eventually lead to right-sided heart failure (Monnet 2008).
• Breathing problems affect some brachycephalic bitches when giving birth (Harvey 1989) and are one of the reasons for caesarean sections in these dogs.

These secondary BAOS changes are progressive so the signs shown at any one time depend on which anatomical defects are present, the severity of these and how long the secondary conditions have been progressing. The primary congenital abnormalities produce signs in puppyhood. The mean age at which brachycephalic dogs are presented to vets for treatment of BAOS is 3 to 4 years of age (Monnet 2008). Laryngeal collapse is usually seen in older animals (Harvey 1982a).

Clinical signs in the mildest cases consist of snoring and respiratory noise on inspiration (breathing in), also called stridor (Brown and Gregory 2005, Fasanella et al 2010). Signs in more severe cases include exercise intolerance (inability to cope with exercise), mouth breathing, gagging, restlessness, rapid breathing, cyanosis (blue coloured membranes of the mouth – due to lack of oxygen in the blood), dysphagia, abnormal posture, and intermittent collapse due to respiratory compromise (Shell 2008, Fasanella et al 2010). Vomiting and regurgitation are also frequently seen (Dupre 2008). Flatulence is common due to aerophagia (swallowing air) (Harvey 1989).

Respiratory crises occur commonly in individuals that are moderately to severely affected. In these cases, the animals rapidly develop respiratory distress – struggling to breathe - and may collapse and die. Severely affected individuals with laboured breathing, stand with their elbows held away from their chests in an attempt to ease breathing (Brown and Gregory 2005). With laboured breathing, over inflation of the chest is seen (Dupre 2008).

Dogs with more severe BAOS live a precarious existence and minor aggravations can lead to severe respiratory distress and crisis (Hendricks 1995). Stress, exercise, and excitement can all act as aggravators (Hendricks 1995, Dupre 2008). These dogs are very prone to heat stroke (Hendricks 1995).

Hendricks (1995) examined sleep disorders in 20 English bulldogs. All those over 2 weeks of age exhibited  breathing disorders during sleep including pauses in breathing and blood oxygen levels dropping below normal many times per hour. It is likely that this also happens in Shar peis with BAOS.

BAOS is a progressive disease. Some of the primary problems, such as stenotic nares and elongated soft palate can be helped by surgery. Tracheal hypoplasia is untreatable (Brown and Gregory 2005). Surgical treatment is a major intervention but without treatment moderate to severely affected dogs will suffer progressive respiratory distress, incapacity and ultimately death.

Some of the secondary problems can also be alleviated by surgery. However, the outlook for dogs with BAOS depends on which primary and secondary conditions they have at the time of treatment and how advanced they are. Overall prognosis for dogs that have had surgical interventions varies. Reicks et al (2007), in a study of 62 cases in various brachycephalic breeds, reported favourable outcomes in all dogs needing surgery. Poncet et al (2006) found good or excellent improvement in 88% of the 51 brachycephalic cases they studied. 

The complexity of the surgery needed and the outcome vary depending on the characteristics of the disease in each dog.

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2. Intensity of welfare impact

BAOS can have a major welfare impact in severely affected dogs due to their distress (which may include apprehension or fear) when struggling to breathe. Without major surgical treatment these crises can reoccur and can be life-threatening. Disrupted sleep patterns because of brachycephalic respiratory compromise are probably common. Even individuals with mild to moderate BAOS are unable to exhibit, what for non-brachycephalic breeds of dogs, is considered normal behaviour such as running and resting comfortably.

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3. Duration of welfare impact

This is a life-long disorder, starting in puppies, progressing throughout life and ultimately often shortening it (Dupre 2008). The average age of presentation of brachycephalic dogs (of all breeds) to vets for treatment is 3-4 years of age (Monnet 2008). Even with surgical treatment, life-expectancy can be compromised and some authors report death, or euthanasia due to BAOS, in a relatively high percentage of cases.

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4. Number of animals affected

Brown and Gregory (2005) suggested that most brachycephalic dogs are affected by upper airway obstruction to some degree. The severity of the problem depends on the number and magnitude of the anatomical abnormalities present.

• Stenotic nares are present in between 46 and 50% of dogs with BAOS (Lorinson et al 1997, Harvey 1982a, Fasanella et al 2010).
• Elongated soft palate is present in 96 to 100% of brachycephalic dogs have an overlong soft palate (Harvey 1982b, Dupre 2008, Fasanella et al 2010).
• Everted tonsils were seen in 56% of brachycephalic dogs with BAOS (Fasanella et al 2010).
• Nasopharyngeal turbinates are found in some brachycephalics.
• Laryngeal collapse.  Fasanella et al (2010) found that 66% of brachycephalic dogs with BAOS syndrome had everted laryngeal saccules.
• Tracheal hypoplasia is common and often severe in some brachycephalic breeds.

Secondary problems are also common.  The prevalence of laryngeal problems in brachycephalic dogs was found to vary from 30% to 64% (Harvey 1982c, Harvey 1982d, Dupre 2008). In one study of 73 cases of BAOS, on presentation, 97% were found to have oesophageal, gastric or duodenal anomalies, and 74% to have moderate or severe gastrointestinal problems (Poncet et al 2005).

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5. Diagnosis

The syndrome should be suspected in any brachycephalic dog showing the characteristic signs (see ‘Clinical and pathological effects’ above). Diagnosis of stenotic nares can be made on clinical examination, but definitive diagnoses of elongated soft palate, tonsillar hypertrophy and laryngeal collapse depend upon examination under anaesthetic. Diagnoses of tracheal hypoplasia, right sided heart failure and confirmation of elongated palate are made with radiography (x-rays) under anaesthesia and gastrointestinal complications can be investigated and confirmed using endoscopy and biopsies, under anaesthetic (Monnet 2008). Anaesthesia carries substantially increased risks for BAOS sufferers and most authors only recommend anaesthesia for diagnosis when corrective surgical treatment can also be carried out at the same time, under the same anaesthetic, so that the risks during recovery are reduced.

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6. Genetics

Brachycephalic head shape has long been thought to be the result of an inherited defect (Stockard 1941). Recently the region of the canine genome associated with brachycephaly has been identified and 2 particular genes have been implicated. The genes involved and the nature of the genetic defects are currently unknown (Bannasch et al 2010).

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7. How do you know if an animal is a carrier or likely to become affected?

Most brachycephalic animals have some degree of BAOS (Brown and Gregory 2005), so it is extremely difficult to identify a puppy that will be free of this condition. Anyone wishing to obtain a Shar pei should ensure that it and its sire and dam are free of signs of BAOS and that they have not had corrective surgery for any of the primary or secondary conditions.

Currently, identifying individuals that have had surgery is difficult because scars on the nostrils may not be easy to see and the other surgical scars are hidden inside the throat. Because of this problem, Harvey (1989) pursued a policy of simultaneously neutering all animals that had corrective surgery. The extent to which others have shared this policy is not known but it is certainly not universal. Puppies with signs of BAOS should not be purchased, as this is likely to help perpetuate the condition. If signs appear after purchase, the puppy should be returned to its breeder.

Dogs which have shown any signs of BAOS or have had corrective surgery for BAOS should not be used for breeding.

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8. Methods and prospects for elimination of the problem

The clear identification and neutering of all dogs which have had surgical interventions to treat BAOS seems vital in tackling this problem. If dogs with BAOS were not used for breeding, the remaining gene pool of animals of this breed might become unsustainably small, so it may be that outcrossing with dogs of non-brachycephalic breeds will be necessary. However, opinions differ as to whether it is ethically acceptable to breed animals whose welfare is likely to be compromised.

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9. Acknowledgements

UFAW is grateful to Rosie Godfrey BVetMed MRCVS and David Godfrey BVetMed FRCVS for their work in compiling this section.

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10. References

Bannasch D, Young A, Myers J, Truvé K, Dickinson P et al (2010) Localization of Canine Brachycephaly Using an Across Breed Mapping Approach. PLoS ONE 5(3): e9632. doi:10.1371/journal.pone.0009632

Brown D and Gregory S (2005) Brachycephalic Airway Disease. In Brockman D & Holt D Eds BSAVA Manual of Canine and Feline Head, Neck and Thoracic Surgery. pp 84. BSAVA: Cheltenham

Burbidge HM, Goulden BE and Dickson LR (1988) Surgical relief of severe laryngeal malformation in an English Bulldog. New Zealand Veterinary Journal 36: 29-31

Coyne B and Fingland R (1992) Hypoplasia of the tracheal in dogs: 103 cases (1974–1990). Journal of the American Veterinary Medical Association 201: 768–772

Ducarouge B (2002) Le syndrome obstructif des voies respiratoires supérieures chez les chiens brachycéphales. Etudes clinique à propos de 27 cas. Doctoral thesis, Lyon

De Lorenzi D, Bertoncello D and Drigo M (2009) Bronchial abnormalities found in a consecutive series of 40 brachycephalic dogs. Journal of American Veterinary Medical Association 235: 835-40

Dupre G (2008) Brachycephalic Syndrome: New Knowledge, New Treatments. Presentation at WSAVA Congress, Dublin, Ireland, 20-24th August 2008 (On-line). Available at http://www.vin.com/proceedings/Proceedings.plx?CID=WSAVA2010&Category=&PID=56236&O=Generic. Accessed 20.7.10

Dupre G and Freiche V (2002) Ronflements et vomissements chez les bouledogues: traitement médical ou chirurgical? Proceedings of the AFVAC Annual Congress. Paris, France, November 10, 2002. pp 235-236

Fasanella FJ, ShivleyJM, Wardlaw JL and Givaruangsawat S (2010) Brachycephalic airway obstructive syndrome in dogs: 90 cases (1991–2008). Journal of the American Veterinary Medical Association 237: 1048-1051

Ginn JA, Kumar MSA, McKiernan BC and Powers BE (2008) Nasopharyngeal Turbinates in Brachycephalic Dogs and Cats. Journal of the American Animal Hospital Association 44: 243-249

Harvey C (1982a) Upper airway obstruction surgery 1: Stenotic nares surgery in brachycephalic dogs. Journal of American Hospital Association 18: 535-537

Harvey C (1982b) Upper airway obstruction surgery 2: Soft palate resection in brachycephalic dogs. Journal of the American Animal Hospital Association 18: 538-544

Harvey C (1982c) Upper airway obstruction surgery 3: Everted laryngeal saccule surgery in brachycephalic dogs. Journal of the American Animal Hospital Association 18: 545-547

Harvey C (1982d) Upper airway obstruction surgery 4: Partial laryngectomy in brachycephalic dogs. Journal of the American Animal Hospital Association 18: 548-550

Harvey C (1989) Inherited and congenital airway conditions. Journal of Small Animal Practice 30: 184-187

Hendricks J (1995) Recognition and treatment of congenital respiratory tract defects in brachycephalics. In Bonagura J (Ed) Kirk’s Current Veterinary Therapy XII small animal practice. Philadelphia: WB Saunders

Koch D, Arnold S, Hubler M and Montavon P (2003) Brachycephalic syndrome in dogs. Compendium on Continuing Education for the Practicing Veterinarian 25: 48-55

Lorinson D, Bright R and White R (1997) Brachycephalic airway obstruction syndrome a review of 118 cases. Canine Practice 22: 18-21

Monnet E (2003) Brachycephalic airway syndrome. In Slatter D (Eds) Textbook of Small Animal Surgery. Philadelphia: WB Saunders

Monnet E (2008) Brachycephalic airway syndrome (CVC Proceedings). (On-line). Available at http://veterinarycalendar.dvm360.com/avhc/Medicine/Brachycephalic-airway-syndrome-Proceedings/ArticleStandard/Article/detail/587147. Accessed 19th July 2010

Orsher R J (1993) Brachycephalic airway disease. In Bojrab M (Ed) Disease Mechanisms in Small Animal Surgery. Philadelphia: Lea and Febiger

Pink J, Doyle R, Hughes J, Tobin E and Bellenger C (2006) Laryngeal collapse in seven brachycephalic puppies. Journal of Small Animal Practice 47: 131–135

Poncet C, Dupre G, Freiche G, Estrada M, Poubannet Y and Bouvy B (2005) Prevalence of gastrointestinal tract lesions in 73 brachycephalic dogs with upper respiratory syndrome. Journal of Small Animal Practice 46: 273–279

Reicks TW, Birchard SJ and Stephens JA (2007) Surgical correction of brachycephalic syndrome in dogs: 62 cases (1991–2004). Journal of the American Veterinary Medical Association 230: 1324-8

Shell L (2008) Brachycephalic Airway Syndrome. (On-line). Available at http://www.vin.com/Members/Associate/Associate.plx?DiseaseId=564. Accessed 16.7.10

Stockard (1941) Wistar Institute Monograph. The genetic and endocrine basis for differences in form and behaviour as elucidated by studies of contrasted pure line dog breeds and their hybrids. Animal Anatomical Memoirs No 19, The Wistar Institute

Torrez C and Hunt G (2006) Results of surgical correction of abnormalities associated with brachycephalic airway obstruction syndrome in dogs in Australia. Journal of Small Animal Practice 47: 150–154

Venker-van Haagen A (1995) Diseases of the throat. In Ettinger S and Feldman E (Eds) Textbook of Veterinary Internal Medicine. Philadelphia: WB Saunders

Wykes P (1991) Brachycephalic airway obstructive syndrome. Problems in Veterinary Medicine 3(2): 188-97

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