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YangW, etal. BMJ Case Rep 2018. doi:10.1136/bcr-2016-218906
SUMMARY
Spontaneous perforation of the pharynx is an unusual
condition. Due to its non-specific presentation and
general lack of awareness, diagnosis and intervention
may be delayed resulting in potential complications.
This case reports a rare spontaneous perforation of the
pyriform sinus after a forceful sneeze, leading to cervical
subcutaneous emphysema and pneumomediastinum.
BACKGROUND
Pharyngeal perforation is a condition which often
derives from trauma, surgery or infection. It can
result in complications without early recognition
and treatment. Spontaneous pharyngeal perforation
with no identified cause is rare. Here, we report a
case of spontaneous pharyngeal rupture after a
forceful sneeze, leading to cervical subcutaneous
emphysema and pneumomediastinum.
CASE PRESENTATION
A previously fit and well 34-year-old man presented
to the emergency department with an acute onset
of odynophagia and change of voice after a forceful
sneeze. He described a popping sensation in his
neck and some bilateral neck swelling after he tried
to halt a sneeze by pinching the nose and holding
his mouth closed. There was no proceeding history
of recent iatrogenic procedure or cervical trauma
and he denied having eaten anything sharp.
Patient’s vital signs were stable on admission
with no fever. There was no stridor or other signs
of respiratory distress. On physical examination,
there was swelling and tenderness especially on the
right side of his cervical region. Crepitus was noted
in both sides of the anterior neck extending down
to the sternum. Fibre-optic nasal pharyngoscopy
showed a normal laryngeal appearance with bilat-
eral functioning vocal cords.
INVESTIGATIONS
Lateral soft tissue neck radiograph showed streaks
of air in the retropharyngeal region and exten-
sive surgical emphysema in the neck anterior to
the trachea (figure 1). Urgent CT of the neck and
thorax with contrast confirmed the presence of
extensive soft tissue emphysema predominantly
centred within the neck as well as pneumome-
diastinum extending from skull base down to T9
vertebra (figure 2A,B). There was no pneumo-
thorax or evidence of bronchial injury. Lung paren-
chyma and oesophagus both appeared normal. The
presence of a subtle relative prominence of the right
pyriform sinus suggested a possible fistula with the
cervical subcutaneous tissue. The air collections
were judged as a result from a pharyngeal tear
possibly from the right pyriform sinus.
DIFFERENTIAL DIAGNOSIS
Boerhaave’s syndrome.
TREATMENT
The patient was admitted due to risk of progres-
sion to deep neck infection or even mediastinitis.
He was treated conservatively with enteral feeding
via a nasogastric tube and prophylactic intrave-
nous antibiotics. His symptoms and subcutaneous
emphysema gradually resolved during the course of
admission.
OUTCOME AND FOLLOW-UP
A repeat CT neck and thorax with water-soluble
contrast swallow 7 days later showed markedly
subsided soft tissue emphysema and no evidence
of contrast leak. The nasogastric tube was removed
and a soft diet was introduced without any prob-
lems. The patient was subsequently discharged
with advice to avoid obstructing both nostrils while
sneezing. On 2 months follow-up, patient did not
present any further recurrence or complications.
DISCUSSION
Perforation of the pharynx most commonly result
from iatrogenic procedures or blunt neck trauma.1
Spontaneous pharyngeal perforation can also occur
but is relatively rare. The mechanism is thought
to involve a sudden rise in intraluminal pressure
against closed vocal folds often following coughing,
straining, forceful retching or vomiting.2 Sneezing,
especially with both nostrils and mouth obstructed,
is a rare predisposing clinical situation.
In the present case, the rupture site is likely from
the right pyriform sinus which suggest the patho-
physiology is different from that of Boerhaave’s
syndrome where the tear tends to involve the
distal part of the thoracic oesophagus.3 The pyri-
form sinus is at risk of perforation during a sudden
increase in the pharyngeal pressure due to its lack
of a reinforcing longitudinal muscle layer.2 In Boer-
haave’s syndrome, the spontaneous transmural
perforation occurs as a consequence of neuromus-
cular incoordination which leads to failure of the
cricopharyngeus muscle to relax.2 Although both
conditions may present in similar ways with cervical
CASE REPORT
Snap, crackle and pop: when sneezing leads to
crackling in theneck
Wanding Yang, Raguwinder S Sahota, Sudip Das
Rare disease
To cite: YangW, SahotaRS,
DasS. BMJ Case Rep
Published Online First:
[please include Day Month
Year]. doi:10.1136/bcr-2016-
218906
ENT, Head and Neck Surgery,
University Hospitals of Leicester
NHS Trust, Leicester, UK
Correspondence to
Miss Wanding Yang,
dingyang@ doctors. org. uk
Accepted 20 December 2017
2YangW, etal. BMJ Case Rep 2018. doi:10.1136/bcr-2016-218906
Rare disease
subcutaneous emphysema and spontaneous pneumomedias-
tinum, Boerhaave’s syndrome carries a much higher morbidity
and mortality and requires prompt and aggressive surgical treat-
ment.3 As the oesophagus ruptures in Boerhaave’s syndrome,
the gastric contents transgress into the mediastinum resulting
in overwhelming mediastinitis, respiratory failure, shock, sepsis
and potentially death.3 In contrast, pharynx or pyriform sinus
perforation can usually be managed non-surgically and cured
without significant morbidity due to its low rate of respiratory
or intrathoracic complication.4 Patients with well-contained leak
and no significant complications can be treated conservatively
with enteral/parenteral feeding and broad spectrum antibiotics.
However, if patients show signs of sepsis or have large perfora-
tion, surgical drainage of the paracervical space with or without
primary repair is strongly recommended to control the infection
and to prevent its expansion into the thorax.4
Early diagnosis is often difficult in pharyngeal perforation.
Pain is the most common symptoms in perforation of larynx or
oesophagus and is usually localised to the site of the perforation.2
In our case, the patient presented with an unusual ‘popping’
sensation over the pyriform sinus immediately after sneezing. It
is followed by odynophagia and dysphonia a few hours later. He
did not complain of any chest pain which is the most common
symptom associated with spontaneous pneumomediastinum.
Cervical emphysema is frequent after cervical perforation and
can be easily detected with plain X-ray or CT scan. It often
descends to cause pneumomediastinum which sometimes can
produce Hamman’s sign (crackling sound synchronous with
the heart beat) when heart beats against air-filled tissue. Nasal
endoscopy can occasionally demonstrate the fistula in pharyn-
geal perforation but it is often difficult as shown in our case. CT
scan of the neck and thorax with water-soluble contrast swallow
should be used as the gold standard investigation which can
confirm the diagnosis and defines the exact pathological site.5
In addition, the normal CT appearance of the lung parenchyma
and oesophagus helps to exclude more serious causes of pneu-
momediastinum such as tracheobronchial rupture and Boer-
haave’s syndrome.
Halting sneeze via blocking nostrils and mouth is a dangerous
manoeuvre and should be avoided, as it may lead to numerous
complications such as pneumomediastinum, perforation of
tympanic membrane and even rupture of cerebral aneurysm.
In conclusion, spontaneous pharyngeal perforation can rarely
occur after a forceful sneeze especially against a closed glottis.
We should maintain a high degree of suspicion and initiate inves-
tigation and treatment early to avoid complication.
Learning points
►Spontaneous perforation of the pharynx is extremely rare and
has a non-specific presentation.
►The pyriform sinus is at risk of perforation during a sudden
increase in the pharyngeal pressure due to its lack of a
reinforcing longitudinal muscle layer.
►Patients with well-contained leak and no significant
complications can be treated conservatively.
►CT scan of the neck and thorax with water-soluble contrast
swallow should be used as the gold standard investigation.
►Simultaneously obstructing both nostrils and mouth during
sneezing should be avoided.
Contributors WY and RSS: literature research and cowriter of the manuscript. SD:
identified the case and cowriter of the manuscript.
Competing interests None declared.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
Open Access This is an Open Access article distributed in accordance with the
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permits others to distribute, remix, adapt, build upon this work non-commercially,
and license their derivative works on different terms, provided the original work
is properly cited and the use is non-commercial. See: http:// creativecommons. org/
licenses/ by- nc/ 4. 0/
© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article)
2018. All rights reserved. No commercial use is permitted unless otherwise expressly
granted.
Figure 1 Lateral soft tissue neck radiograph with narrow window
setting shows streaks of air in the retropharyngeal region (black arrow)
and extensive surgical emphysema in the neck anterior to the trachea
(white arrow).
Figure 2 (A and B) Coronal and sagittal CT scan of the neck
and thorax showing surgical emphysema within the neck and
pneumomediastinum extending from skull base up to T9 vertebra (black
arrow).
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YangW, etal. BMJ Case Rep 2018. doi:10.1136/bcr-2016-218906
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Rare disease
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