MINENHLE SIKHAKHANE

2320822

MBBCh V

WITS UNIVERSITY

CONGENITAL NASAL PYRIFORM APERTURE STENOSIS

- A CASE REPORT

INTRODUCTION
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This case report is about congenital pyriform aperture stenosis. The discussion part focuses on
management.

Congenital nasal pyriform aperture stenosis (CNPAS) is the narrowing of the nasal cavity at the
level of the pyriform aperture due to either overgrowth or medial positioning of the maxillary
bone processes (Wine, Dedhia and Chi, 2014). Literature review shows that there might be a
significant role of nasal dilation in the management of congenital nasal pyriform aperture
stenosis.

CASE PRESENTATION

Patient details

Name: SHADI MASUKU

Hospital number: 8930066

Age: 3 MONTHS

Gender: FEMALE

Race: BLACK

HISTORY
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1. MAIN COMPLAINT

A 3-month-old female child was referred from Edenvale Hospital with a confirmed diagnosis of
Congenital Nasal Pyriform Aperture Stenosis. At the time of the presentation, the child also
had noisy breathing.

- the child was on oxygen on arrival.

- The child had noisy breathing.

- there were no signs of respiratory distress.

History of the main complaint:

At the time of birth, the neonate had low APGAR scores and was then put on oxygen
supplementation. She then stayed on oxygen supplementation until she was brought to Charlotte
Maxeke Academic Hospital for management.

3. PERINATAL AND BIRTH HISTORY

- the child was born via normal vaginal delivery at term.

- no irregularities were noted antenatally.

4. MEDICAL HISTORY AND IMMUNISATIONS

- the child has no other clinical conditions.

- they are not on any medications.

- they are HIV unexposed.

- the child received all necessary vaccines.

5. FEEDING HISTORY

- the child is formula-fed.

6. GROWTH AND DEVELOPMENTAL HISTORY

- the child has normal growth parameters.

7. FAMILY HISTORY AND GENETIC CONDITIONS

- there is no history of pyriform aperture stenosis or other disabilities in the family.

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8. SYSTEMS REVIEW

- All systems were normal.

EXAMINATION

GENERAL

Inspection

- the child looked normal with no signs of respiratory distress.

- no signs of malnutrition.

Vitals were all normal

Pulse Rate 143 bpm

Respiration Rate 48 br.pm
Temperature 36.8 *C
Blood Pressure 91/72
Oxygen Saturation 98 %

General signs

- No Jaundice, Pallor, Cyanosis, Oedema, or Dehydration.

ENT EXAMINATION

EAR
- nothing abnormal was detected.

NOSE
- Noisy breathing was heard from the nostrils.

- There was no choanal atresia.

- There was no nasal discharge.

THROAT
- nothing abnormal was detected.

OTHER SYSTEMS
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- RESP

- Vesicular breath sounds bilaterally, with no wheezes or crackles.

- CVS – normal S1 and S2 with no added sounds.

INVESTIGATIONS

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A CT scan was done to confirm the diagnosis. The scan showed a nasal diammeter of less than
11mm with the presence of a central incisor tooth.

This is the CT-scan image that shows the narrowed pyriform aperture.

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This is the CT-scan image that shows the central incisor tooth (ignore the white arrow).

MANAGEMENT

- The infant was taken off oxygen with the plan to re-oxygenate should saturation go below
90%.

- Normal saline drops were given three times daily

- Surgery was planned for the reconstruction of the nasal airway via the sublabial approach plus
nasal stents.

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DISCUSSION

A CLOSER LOOK AT THE ROLE OF NASAL DILATION IN MANAGEMENT OF

CONGENITAL PYRIFORM APERTURE STENOSIS IS NEEDED.

The general management of congenital pyriform aperture stenosis starts with medical support
and then surgery if there is no improvement. Another uncommon management that is uprising is
nasal dilation.

Medical management is conservative and is the first line, it includes nasal irrigation with saline,
using intranasal steroids, and nasal suction. Failure of conservative intervention which may be
evident due to cyanosis, poor growth, failure to thrive, apnoeic episodes or inability to wean
away from airway assistance is an indication for surgery (Wood and Van Der Meer, 2018).

Surgery involves drilling out the maxillary bone to widen the pyriform aperture. Two surgical
approaches can be employed. The sublabial approach and the trans-nasal approach. The
sublabial approach is superior to the trans-nasal and is the mainstay in neonates. The advantages
of the sublabial over trans-nasal are good field exposure, less risk of damage to nasolabial soft
tissues and no visible scarring (Sesenna et al., 2012). The trans-nasal approach is not advised
according to Sesenna et al because of reduced dimensions of anatomical structures in neonates
which are associated with a risk of soft-tissue trauma. Despite its superiority, the sublabial
approach also has its associated complications. These are nasolacrimal duct damage, septal
perforation, granulation tissue and maxillofacial growth implications (O’Neil et al., 2023).
Surgery is often followed by nasal stents post-operatively.

Nasal stents are used to reduce the reappearance of the original stenosis and scar-related
stenosis (Sesenna et al., 2012). Stenting also has its own associated complications. These are
soft palate perforation, alar pressure necrosis, granulation tissue, vestibule stenosis, and
haemorrhage (O’Neil et al., 2023).

Nasal dilation involves widening the pyriform aperture via the use of balloon dilation or Hagar
dilators. It is less invasive and has less usage and need of nasal stents. It has been showed to
have fewer failure rates in the literature. A recent literature review of papers that have been
published on nasal dilation was done by O’Neil et al in 2023. It showed that there are fewer
incidences of re-dilation or need for bone drill-out following nasal dilation. The role of stenting
also seems to be less significant as there were cases that did successfully without the use of
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stents. Of the 11 cases mentioned in this paper, a total of 7 did not need further intervention,
only 3 used stents, and only 2 needed re-dilation and eventually proceeded to drill out (O’Neil
et al., 2023). Interestingly, a study conducted by Wine et, al. in four patients using Hegar
dilators had a 100% success rate without any use of stents (Wine, Dedhia and Chi, 2014).

It is therefore apparent that the role of nasal dilation is not to be ignored in the management of
congenital pyriform aperture stenosis. A management algorithm that has been proposed by
O’Neil et al. is that of medical management, followed by nasal dilation, and lastly sublabial
drill-out – this is a modified Moreddu et al. algorithm which starts with medical management
followed by sublabial drill-out (O’Neil et al., 2023).

The limitations of the current evidence are that fewer studies have reported on the prevalence of
the complications associated with both nasal surgery and the use of stents, which makes it
uncertain whether there is a need to consider other options. Hence, more studies are needed on
this. Likewise, fewer studies have been conducted on the role of nasal dilation in managing
congenital pyriform aperture stenosis. Hence, a closer look is needed.

CONCLUSION

The case presented in this report was a typical case of CNPAS as it has been shown that the
infant had both a narrow pyriform aperture as well as a central incisor tooth. Literature review
shows that there might be a significant role of nasal dilation in the management of congenital
nasal pyriform aperture stenosis, however, more studies are needed to support this.

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REFERENCES

3. O’Neil, L.M., Pollaers, K., Sale, P., Shyan Vijayasekaran and Herbert, H. (2023). The utility
of balloon dilation for piriform aperture stenosis – A first line surgical treatment. International
journal of pediatric otorhinolaryngology, 171, pp.111654–111654.

1. Sesenna, E., Leporati, M., Brevi, B., Oretti, G. and Ferri, A. (2012). Congenital nasal
pyriform aperture stenosis: diagnosis and management. Italian Journal of Pediatrics, 38(1),
p.28.

4. Wine, T.M., Dedhia, K. and Chi, D.H. (2014). Congenital Nasal Pyriform Aperture Stenosis.
JAMA Otolaryngology–Head & Neck Surgery, 140(4), p.352.

2. Wood, J. and Van Der Meer, G. (2018). Surgery for congenital nasal pyriform aperture
stenosis (CNPAS).

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