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Congenital Nasal Pyriform Aperture Stenosis CNPAS

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Transfered to the nursery although BB had occasional nasal flaring, ... McGovern nipple. Oral airway. Orotracheal intubation. Conservative management. For whom? ... – PowerPoint PPT presentation

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Title: Congenital Nasal Pyriform Aperture Stenosis CNPAS


1
Congenital Nasal Pyriform Aperture Stenosis CNPAS
  • SAUD ALROMAIH

2
Contents
  • Case presentation
  • Introduction
  • Anatomy and embryology
  • Pathology
  • Clinical presentation
  • Investigation
  • Management
  • Summary

3
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

4
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

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

6
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

7
Case presentation
  • DOL 14 Patient transfered to MCH
  • Seen by ENT
  • CT scan

8
Case presentation
  • DOL 14 Patient transfered to MCH
  • Seen by ENT
  • CT scan
  • Bilateral CNPAS
  • SMMCI
  • Hypotelorism
  • Hypoblastic optic nerves

9
Case presentation
  • MRI
  • TFT
  • Echo
  • Fundoscopy

10
Case presentation
  • MRI
  • Normal brain
  • Hypoblastic mid and posterior parts of optic
    nerves
  • TFT Normal
  • Echo Normal
  • Fundoscopy Normal

11
Case presentation
  • DOL 21 OR Sublabial aproach, resection of
    maxillary over growth, insertion of bilateral
    stents, ETT size 3
  • Post operative period smooth

12
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

13
Introduction
  • Ey et al Brown et al
  • other midline anomalies

14
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.

15
Introduction
  • ? prevalence
  • emergency airway obstruction
  • ??severe asphyxia.

16
Anatomy
17
Anatomy
  • The pyriform aperture is a pear-shape inlet which
    is the narrowest, most anterior bony inlet of the
    nasal cavity

18
Anatomy
  • Bounderies

19
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.

20
Embryology
21
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

22
Pathology
  • pathogenesis
  • midline anomalies
  • Chromosomal anomalies

23
Pathology
  • The exact pathogenesis of CNPAS is still unknown.

24
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.

25
Pathology
  • Association between CNPAS and chromosomal
    abnormalities such as 18p, 13p, ring 18, trisomy
    13 or 18 have been reported

26
Clinical Presentation
  • Neonates nasal breathing
  • Apnea
  • Cyanosis
  • ? Crying
  • ? Feeding

27
Clinical Presentation
  • Neonates are preferably nasal breathers
  • Apneic episodes and cyclic cyanosis
  • Relieved by crying
  • More during feeding

28
Clinical Presentation
  • Stridor
  • Respiratory distress
  • tracheobronchial airway and lungs
  • NG tube

29
Clinical Presentation
  • Noisy nasal breathing
  • Inspiratory stridor
  • Other signs of respiratory distress
  • Normal tracheobronchial airway and lungs
  • Introduction of NG tube is difficult

30
Differential diagnosis
  • Most likely
  • Exclude
  • Not to forget

31
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

32
Inestigations
  • Fibro-optic scope
  • CT scan nasal cavity
  • Others
  • MRI
  • Hormonal studies
  • Chromosomal

33
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

34
Investigation CT
  • Width less than 11 mm in a term infant is
    considered to be diagnostic.

35
Investigation CT
  • Presence of bony ridge along the inferior aspect
    of the palate and dental anomalies involving the
    incisors strongly suggest the diagnosis

36
3D CT
  • Pre and post operative

37
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

38
Investigation Others
  • MRI brain, chromosomal studies, pituitary
    hormones studies should be done to look for
    associated anomalies

39
Management
  • Based on severity
  • Immediate airway management as needed
  • Consrevative
  • Gradual dilatation
  • Surgical resection
  • Role of stents

40
Airway management
  • McGovern nipple
  • Oral airway
  • Orotracheal intubation

41
Conservative management
  • For whom?
  • Includes what?
  • Why?

42
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

43
Management dilatation
  • Mentioned only in a case report
  • Kawamura et al, a case of congenital nasal
    pyriform aperture stenosis, Pediatrics
    international, 1999

44
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

45
Surgical resection and stenting
  • Definitive treatment
  • Premaxillary approach through sublabial incision
  • Resection of the inferior margin of the bone in
    anterior nasal aperture

46
Surgery, Indications
  • Severe symptoms
  • ? 5F catheter
  • ? conservative management

47
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

48
Surgery, Complications
  • Tooth bud
  • nasolacrimal ducts
  • Mid-facial and maxillary hypoplasia

49
Surgery, Complications
  • Tooth bud damage
  • Injuries to nasolacrimal ducts
  • Mid-facial and maxillary hypoplasia

50
Stenting
  • Consept
  • Duration
  • Complications

51
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.

52
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

53
Summary
  • Clinical presentation resembles choanal atresia
  • CT scan is the modality of choice
  • Management depends mainly on severity of symptoms

54
Questions?
55
Merci!
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