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Congenital Laryngeal / Glottic Web

last modified on: Tue, 09/19/2023 - 10:48

see also: Anterior Glottic Web

GENERAL INFORMATION

  • Uncommon congenital anomalies spanning the glottic aperture. Congenital webs are almost entirely anterior (Izadi 2010).

  • Presenting symptoms include abnormal/weak/absent cry, dysphonia, inspiratory/biphasic stridor and failure to thrive. There are often comorbid airway or heart conditions.

    • Despite significant congenital airway compromise and severe webbing children may not show symptoms until the first few months of life (Kuo 2020).

  • Failure of laryngeal lumen recannulation after epithelial obliteration. The process is actually a spectrum of laryngeal atresia as opposed to acquired webs (Zaw-tun 1988, Milczuk 2000)

  • There appears to be a female preponderance [2.4:1] (Lawlor 2020)

EVALUATION

  • If stable, awake flexible fiberoptic laryngoscopy can exclude comorbid pathology.

  • Rigid bronchoscopy is necessary, generally under spontaneous ventilation. This can be aided by flexible bronchoscopy or 70 degree telescopes.

  • There is a strong association of anterior glottic webs with 22q11.2 deletion (Miyamoto 2004, Lawlor 2020). Additionally, one study showed 21% of children with 22q11.2 deletion who underwent direct laryngoscopy had a glottic web (Sacca 2017). All congenital glottic webs should be tested by FISH.

  • CT Neck with thin slices should be considered before open airway intervention to assess craniocaudal extension.

COHEN Classification System adapted from descriptions in (Cohen 1985)

  
 

Glottic Percentage

Subglottic stenosis

Thickness

Vocal Folds

Voice

Airway obstruction

Type 1

<35%

Little to none

Uniform

Identifiable

Hoarse

Minimal

Type 2

35-50%

Minimal SGS at anterior portion of the web

Thin or Thick

Identifiable

Hoarse/Weak

With infection/trauma

Type 3

50-75%

Anterior subglottic stenosis

Thick anterior and thinner posterior

Mostly visible, possibly obscured

Weak

Moderately severe,

Frequent Tracheostomy

Type 4

75-90%

Severely narrowed

Uniformly thick

Not identifiable

Aphonic

Severe, Tracheostomy shortly after birth

  • Success of endoscopic lysis and keel placement is correlated to web thickness but not to Cohen grading (Chen 2017)

  • Classification does not appear to be predictive of surgical success, evaluated by either symptomatic improvement or risk of recurrence. (Lawlor 2020)

TREATMENT

  • A wide variety of surgical techniques have been described. Surgical decision making depends on surgeon experience and comfort. In general endoscopic procedures have been more successful on thinner flaps as noted above (Kuo 2020).

  • For less severe webs where voice is the primary symptom late repair right before entering school is appropriate.

  • For more severe webs where airway is the primary concern early intervention is necessary.

  • Some authors cite DiGeorge being associated with significant post operative edema suggesting utility of a double-stage procedure (Kuo 2020)

  • Recurrence rate is high >70% (Lawlor 2020)

INTERVENTIONS

  • Tracheostomy

    • Especially in smaller infants, early tracheostomy allows for catch up growth, buying time for a future reconstructive procedure.

    • One study showed 45% decannulation rate of those requiring tracheotomy (Lawlor 2020)

  • Balloon dilation laryngoplasty (Described in Yoo 2015)

  • Endoscopic laser anterior commissurotomy (Described in Su 2010)

  • Endoscopic 'Butterfly Mucosal Flap' (Described in Xiao 2014, Yilmaz 2019)

  • Endoscopic lateralization with silastic (Described in Hseuh 2000)

  • Endoscopic silastic keel placement (Described in Chiu 1996, Benmansour 2012, Paniello 2013, Kuo 2020)

  • Open Butterfly Perichondrial/Cartilage draft (Described in Izadi 2010)

  • Laryngofissure +/- LTR/Silastic keel either single or double-staged (Variations described in Biavati 1995, Wyatt 2005, Kuo 2020)

REFERENCES

Cohen SR. Congenital glottic webs in children. A retrospective review of 51 patients. Ann Otol Rhinol Laryngol Suppl. 1985 Nov-Dec;121:2-16. PMID: 3935032.

Chen J, Shi F, Chen M, Yang Y, Cheng L, Wu H. Web thickness determines the therapeutic effect of endoscopic keel placement on anterior glottic web. Eur Arch Otorhinolaryngol. 2017 Oct;274(10):3697-3702. doi: 10.1007/s00405-017-4689-2. Epub 2017 Aug 2. PMID: 28770347.

Benmansour N, Remacle M, Matar N, Lawson G, Bachy V, Van Der Vorst S. Endoscopic treatment of anterior glottic webs according to Lichtenberger technique and results on 18 patients. Eur Arch Otorhinolaryngol 2012; 269: 2075– 2080.

Biavati MJ, Wood WE, Kearns DB, Smith RJ. One-stage repair of congenital laryngeal webs. Otolaryngol Head Neck Surg. 1995 Mar;112(3):447-52. doi: 10.1016/s0194-5998(95)70282-2. PMID: 7870448.

Chiu LD, Ragson BM, Cruz RM. Laryngoscopic placement of laryngeal keels with percutaneous fixation. Laryngoscope 1996; 106: 788– 790.

Hsueh JY, Tsai CS, Hsu HT. Intralaryngeal approach to laryngeal web using lateralization with silastic. Laryngoscope 2000; 110: 1780– 1782.

Izadi F, Delarestaghi MM, Memari F, Mohseni R, Pousti B, Mir P. The butterfly procedure: a new technique and review of the literature for treating anterior laryngeal webs. J Voice. 2010 Nov;24(6):742-9. doi: 10.1016/j.jvoice.2009.03.005. Epub 2009 Oct 21. PMID: 19850447.

Kuo IC, Rutter M. Surgical Management of Anterior Glottic Webs. Front Pediatr. 2020 Oct 19;8:555040. doi: 10.3389/fped.2020.555040. PMID: 33194889; PMCID: PMC7604345.

Lawlor CM, Dombrowski ND, Nuss RC, Rahbar R, Choi SS. Laryngeal Web in the Pediatric Population: Evaluation and Management. Otolaryngol Head Neck Surg. 2020 Feb;162(2):234-240. doi: 10.1177/0194599819893985. Epub 2019 Dec 17. PMID: 31842676.

Milczuk HA, Smith JD, Everts EC. Congenital laryngeal webs: surgical management and clinical embryology. Int J Pediatr Otorhinolaryngol. 2000 Jan 30;52(1):1-9. doi: 10.1016/s0165-5876(99)00284-0. PMID: 10699233.

Miyamoto RC, Cotton RT, Rope AF, Hopkin RJ, Cohen AP, Shott SR, Rutter MJ. Association of anterior glottic webs with velocardiofacial syndrome (chromosome 22q11.2 deletion). Otolaryngol Head Neck Surg. 2004 Apr;130(4):415-7. doi: 10.1016/j.otohns.2003.12.014. PMID: 15100636.

Paniello RC, Desai SC, Allen CT, Khosla SM. Endoscopic keel placement to treat and prevent anterior glottic webs. Ann Otol Rhinol Laryngol. 2013 Nov;122(11):672-8. doi: 10.1177/000348941312201102. PMID: 24358626.

Sacca R., Zur K.B., Crowley T.B., et. al.: Association of airway abnormalities with 22q11.2 deletion syndrome. Int J Pediatr Otorhinolaryngol 2017; 96: pp. 11-14.

Su CY, Alswiahb JN, Hwang CF, Hsu CM, Wu PY, Huang HH. Endoscopic laser anterior commissurotomy for anterior glottic web: one-stage procedure. Ann Otol Rhinol Laryngol. 2010 May;119(5):297-303. doi: 10.1177/000348941011900505. PMID: 20524574.

Wyatt ME, Hartley BE. Laryngotracheal reconstruction in congenital laryngeal webs and atresias. Otolaryngol Head Neck Surg. 2005 Feb;132(2):232-8. doi: 10.1016/j.otohns.2004.09.032. PMID: 15692532.

Yılmaz T. Surgical treatment of glottic web using butterfly mucosal flap technique: Experience on 12 patients. Laryngoscope. 2019 Jun;129(6):1423-1427. doi: 10.1002/lary.27531. Epub 2018 Dec 26. PMID: 30585630.

Yoo MJ, Roy S, Smith LP. Endoscopic management of congenital anterior glottic stenosis. Int J Pediatr Otorhinolaryngol. 2015 Dec;79(12):2056-8. doi: 10.1016/j.ijporl.2015.09.013. Epub 2015 Sep 25. PMID: 26412460.

Xiao Y, Wang J, Han D, Ma L, Ye J, Xu W. Vocal cord mucosal flap for the treatment of acquired anterior laryngeal web. Chin Med J (Engl). 2014;127(7):1294-7. PMID: 24709183.

Zaw-Tun HI. Development of congenital laryngeal atresias and clefts. Ann Otol Rhinol Laryngol. 1988 Jul-Aug;97(4 Pt 1):353-8. doi: 10.1177/000348948809700405. PMID: 3408110.