Congenital Nasal Pyriform Aperture Stenosis CNPAS

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Congenital Nasal Pyriform Aperture StenosisCNPAS

• SAUD ALROMAIH

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Contents

• Case presentation
• Introduction
• Anatomy and embryology
• Pathology
• Clinical presentation
• Investigation
• Management
• Summary

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Case presentation

• BB T. Male BB
• DOB 07-11-08 at RVH
• GA 37 5/7
• Prenatal Hx GDM on diet
• Natal Hx C-section b/c Macrosomia
• Birth wt 3.866 kg
• Apgar score 9 9

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Case presentation

• Transfered to the nursery although BB had
  occasional nasal flaring, retractions and
  grunting, thick nasal secretions.
• Over the night respiratory distress increased,
  with stridor and occasional desaturations
• Trasfered to NICU

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Case presentation

• DOL 2 NICU on 22-24 O2
• DOL 3 4 RA /- O2 PRN
• DOL 5 RA
• DOL 6 started on bottle feeding

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Case presentation

• Then, he started again to have occasional
  desatting, espicially with feeding
• DOL 13 again respiratory distress, relieved by
  prone positioning
• Evaluated by ENT at RVH Mostly CNPAS

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Case presentation

• DOL 14 Patient transfered to MCH
• Seen by ENT
• CT scan

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Case presentation

• DOL 14 Patient transfered to MCH
• Seen by ENT
• CT scan
• Bilateral CNPAS
• SMMCI
• Hypotelorism
• Hypoblastic optic nerves

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Case presentation

• MRI
• TFT
• Echo
• Fundoscopy

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Case presentation

• MRI
• Normal brain
• Hypoblastic mid and posterior parts of optic
  nerves
• TFT Normal
• Echo Normal
• Fundoscopy Normal

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Case presentation

• DOL 21 OR Sublabial aproach, resection of
  maxillary over growth, insertion of bilateral
  stents, ETT size 3
• Post operative period smooth

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Case presentation

• POD 3 started on feeding
• regurgitation of food through stents
• X-ray stents below the soft palate
• OR readjustment
• Post readjustment
• feeding well, no deasaturations

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Introduction

• Ey et al Brown et al
• other midline anomalies

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Introduction

• First described by Ey et al and was defined by
  Brown et al as a bony overgrowth of the nasal
  process of the maxilla.
• May be isolated or associated with other midline
  anomalies.

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Introduction

• ? prevalence
• emergency airway obstruction
• ??severe asphyxia.

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Anatomy
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Anatomy

• The pyriform aperture is a pear-shape inlet which
  is the narrowest, most anterior bony inlet of the
  nasal cavity

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Anatomy

• Bounderies

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Anatomy

• Bounded superiorly by the nasal bones, laterally
  by the nasal process of the maxilla, and
  inferiorly by the premaxilla and the anterior
  nasal spine.

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Embryology
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Embryology

• Stenosis is thought to occur around the fourth
  month of fetal development because of an
  overgrowth of the ossification at the nasal
  process of the maxilla

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Pathology

• pathogenesis
• midline anomalies
• Chromosomal anomalies

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Pathology

• The exact pathogenesis of CNPAS is still unknown.

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Pathology

• It may be associated with midline anomalies like
  a solitary median maxillary central incisor
  (SMMCI) only as part of holoprosencephaly.
• Also with central endocrine insufficiency.

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Pathology

• Association between CNPAS and chromosomal
  abnormalities such as 18p, 13p, ring 18, trisomy
  13 or 18 have been reported

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Clinical Presentation

• Neonates nasal breathing
• Apnea
• Cyanosis
• ? Crying
• ? Feeding

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Clinical Presentation

• Neonates are preferably nasal breathers
• Apneic episodes and cyclic cyanosis
• Relieved by crying
• More during feeding

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Clinical Presentation

• Stridor
• Respiratory distress
• tracheobronchial airway and lungs
• NG tube

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Clinical Presentation

• Noisy nasal breathing
• Inspiratory stridor
• Other signs of respiratory distress
• Normal tracheobronchial airway and lungs
• Introduction of NG tube is difficult

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Differential diagnosis

• Most likely
• Exclude
• Not to forget

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Differential diagnosis

• Choanal atresia
• Nasopharyngeal encephalocele
• Cysts dacrocystocele, demoid or epidermoid cysts
• Skull base defects meningoencephalocele,
  encephalocele.

• Tumors gliomas, hemangiomas, lymphangiomas,
  teratoma and rhabdomyosarcoma
• Traumatic luxated septum, sepatal hematoma

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Inestigations

• Fibro-optic scope
• CT scan nasal cavity
• Others
• MRI
• Hormonal studies
• Chromosomal

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Investigation CT

• CT measurement of the pyriform aperture between
  the medial aspects of the maxilla at the level of
  the inferior meatus is a simple and accurate
  method for diagnosis

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Investigation CT

• Width less than 11 mm in a term infant is
  considered to be diagnostic.

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Investigation CT

• Presence of bony ridge along the inferior aspect
  of the palate and dental anomalies involving the
  incisors strongly suggest the diagnosis

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3D CT

• Pre and post operative

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3D CT

• Entire prospect of pyriform aperture can be
  evaluated
• Quantity of bone which is to be resected is
  clearly defined
• Can be useful for post operative evaluation

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Investigation Others

• MRI brain, chromosomal studies, pituitary
  hormones studies should be done to look for
  associated anomalies

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Management

• Based on severity
• Immediate airway management as needed
• Consrevative
• Gradual dilatation
• Surgical resection
• Role of stents

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Airway management

• McGovern nipple
• Oral airway
• Orotracheal intubation

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Conservative management

• For whom?
• Includes what?
• Why?

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Conservative management

• For mild symptoms
• Includes saline nasal solutions, nasal
  decongestants, humidified oxygen
• As nasal and maxillary bones continue to grow it
  leads to the enlargement of the nasal cavities in
  the 1st 6 months of life

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Management dilatation

• Mentioned only in a case report
• Kawamura et al, a case of congenital nasal
  pyriform aperture stenosis, Pediatrics
  international, 1999

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Management dilatation

• Mentioned in a case report
• Should be tried before surgical resection
• Step-by-step dilatation using NGT
• Bones are not rigid in the neonatal life

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Surgical resection and stenting

• Definitive treatment
• Premaxillary approach through sublabial incision
• Resection of the inferior margin of the bone in
  anterior nasal aperture

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Surgery, Indications

• Severe symptoms
• ? 5F catheter
• ? conservative management

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Surgery, Indications

• Patients with sleep apnea, repeated intubations,
  failed extubations, feeding difficulties with
  cyanosis or unresponsiveness to CPAP.
• Failure to pass size 5F catheter
• Failure of conservative management after 10 to 15
  days

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Surgery, Complications

• Tooth bud
• nasolacrimal ducts
• Mid-facial and maxillary hypoplasia

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Surgery, Complications

• Tooth bud damage
• Injuries to nasolacrimal ducts
• Mid-facial and maxillary hypoplasia

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Stenting

• Consept
• Duration
• Complications

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Stenting

• To allow the nasal mucosa to become attached to
  the new bony margins and to avoid recurrent
  obstruction
• Duration varies between 5 days to 4 weeks
• Complications Pressure necrosis, food
  reguritation and granulation tissue formation
  around the posterior end.

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Summary

• Airway obstruction in the newborn or infant can
  be a life threatening emergency.
• Immediate recognition and appropriate therapy are
  required to prevent severe asphyxia.
• May be isolated or associated with other midline
  anomalies

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Summary

• Clinical presentation resembles choanal atresia
• CT scan is the modality of choice
• Management depends mainly on severity of symptoms

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Questions?
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Merci!