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Laryngeal stenosis

MedGen UID:
7274
Concept ID:
C0023075
Finding; Pathologic Function
Synonyms: Laryngeal Stenoses; Laryngeal Stenosis; Laryngostenoses; Laryngostenosis; Stenoses, Laryngeal; Stenosis, Laryngeal
SNOMED CT: Stenosis of larynx (75547007)
 
HPO: HP:0001602
Monarch Initiative: MONDO:0001305

Definition

Stricture or narrowing of the larynx that may be associated with symptoms of respiratory difficulty depending on the degree of laryngeal narrowing. [from HPO]

Conditions with this feature

Atelosteogenesis type I
MedGen UID:
82701
Concept ID:
C0265283
Congenital Abnormality
The FLNB disorders include a spectrum of phenotypes ranging from mild to severe. At the mild end are spondylocarpotarsal synostosis (SCT) syndrome and Larsen syndrome; at the severe end are the phenotypic continuum of atelosteogenesis types I (AOI) and III (AOIII) and Piepkorn osteochondrodysplasia (POCD). SCT syndrome is characterized by postnatal disproportionate short stature, scoliosis and lordosis, clubfeet, hearing loss, dental enamel hypoplasia, carpal and tarsal synostosis, and vertebral fusions. Larsen syndrome is characterized by congenital dislocations of the hip, knee, and elbow; clubfeet (equinovarus or equinovalgus foot deformities); scoliosis and cervical kyphosis, which can be associated with a cervical myelopathy; short, broad, spatulate distal phalanges; distinctive craniofacies (prominent forehead, depressed nasal bridge, malar flattening, and widely spaced eyes); vertebral anomalies; and supernumerary carpal and tarsal bone ossification centers. Individuals with SCT syndrome and Larsen syndrome can have midline cleft palate and hearing loss. AOI and AOIII are characterized by severe short-limbed dwarfism; dislocated hips, knees, and elbows; and clubfeet. AOI is lethal in the perinatal period. In individuals with AOIII, survival beyond the neonatal period is possible with intensive and invasive respiratory support. Piepkorn osteochondrodysplasia (POCD) is a perinatal-lethal micromelic dwarfism characterized by flipper-like limbs (polysyndactyly with complete syndactyly of all fingers and toes, hypoplastic or absent first digits, and duplicated intermediate and distal phalanges), macrobrachycephaly, prominant forehead, hypertelorism, and exophthalmos. Occasional features include cleft palate, omphalocele, and cardiac and genitourinary anomalies. The radiographic features at mid-gestation are characteristic.
Leri pleonosteosis
MedGen UID:
331978
Concept ID:
C1835450
Disease or Syndrome
Leri pleonosteosis is an autosomal dominant skeletal disorder characterized by flexion contractures of the interphalangeal joints, limited movement of multiple joints, and short, broad metacarpals, metatarsals, and phalanges. Additional features may include chronic joint pain, short stature, bony overgrowths, spinal cord compression, scleroderma-like skin changes, and blepharophimosis. The clinical features overlap with several other musculoskeletal conditions, including Myhre syndrome (MYHRS; 139210) and geleophysic dysplasia (GPHYSD1; 231050) (summary by Banka et al., 2015).
Thoracolaryngopelvic dysplasia
MedGen UID:
349978
Concept ID:
C1861197
Disease or Syndrome
A short-rib dysplasia with characteristics of thoracic dystrophy, laryngeal stenosis and a small pelvis. Less than 10 cases have been reported in the literature so far. Patients present with severe respiratory distress (requiring intubation) during the neonatal period. The rib shortening is less severe than in Jeune syndrome and the thorax is characteristically small, narrow and bell-shaped. The pelvis is reduced in all dimensions and the combination of the thorax anomalies and the small pelvis give the appearance of a protruding abdomen. Subglottic stenosis has also been described but it remains unclear whether this is a congenital anomaly or is secondary to long-term intubation. Transmission is autosomal dominant.
VATER association
MedGen UID:
902479
Concept ID:
C4225671
Disease or Syndrome
VATER is a mnemonically useful acronym for the nonrandom association of vertebral defects (V), anal atresia (A), tracheoesophageal fistula with esophageal atresia (TE), and radial or renal dysplasia (R). This combination of associated defects was pointed out by Quan and Smith (1972). Nearly all cases have been sporadic. VACTERL is an acronym for an expanded definition of the association that includes cardiac malformations (C) and limb anomalies (L). The VACTERL association is a spectrum of various combinations of its 6 components, which can be a manifestation of several recognized disorders rather than a distinct anatomic or etiologic entity (Khoury et al., 1983). Also see VATER/VACTERL association with hydrocephalus (VACTERL-H; 276950) and VACTERL with or without hydrocephalus (VACTERLX; 314390).
Fraser syndrome 1
MedGen UID:
1639061
Concept ID:
C4551480
Disease or Syndrome
Fraser syndrome is an autosomal recessive malformation disorder characterized by cryptophthalmos, syndactyly, and abnormalities of the respiratory and urogenital tract (summary by van Haelst et al., 2008). Genetic Heterogeneity of Fraser Syndrome Fraser syndrome-2 (FRASRS2) is caused by mutation in the FREM2 gene (608945) on chromosome 13q13, and Fraser syndrome-3 (FRASRS3; 617667) is caused by mutation in the GRIP1 gene (604597) on chromosome 12q14. See Bowen syndrome (211200) for a comparable but probably distinct syndrome of multiple congenital malformations.

Professional guidelines

PubMed

Schweiger C, Manica D
Semin Pediatr Surg 2021 Jun;30(3):151052. Epub 2021 May 21 doi: 10.1016/j.sempedsurg.2021.151052. PMID: 34172219
Incandela F, Missale F, Mora F, Marchi F, Fiz I, Piazza C, Peretti G
Eur Arch Otorhinolaryngol 2021 Jan;278(1):149-158. Epub 2020 Jul 23 doi: 10.1007/s00405-020-06210-5. PMID: 32705360Free PMC Article
Redondo-Sedano J, Antón-Pacheco JL, Valverde RM, Díaz ML, Paredes CL, Guardia LM, Alelu RM, Huerta IJ, Gordo MIB, Fraile AG
J Pediatr Surg 2019 Sep;54(9):1933-1937. Epub 2018 Nov 6 doi: 10.1016/j.jpedsurg.2018.09.027. PMID: 30503025

Recent clinical studies

Etiology

Jiam NT, Dwyer CD, Rosen CA
Laryngoscope 2021 May;131(5):E1633-E1637. Epub 2020 Dec 2 doi: 10.1002/lary.29295. PMID: 33264421
Prince ADP, Cloyd BH, Hogikyan ND, Schechtman SA, Kupfer RA
Otolaryngol Head Neck Surg 2020 Jul;163(1):78-80. Epub 2020 May 12 doi: 10.1177/0194599820927002. PMID: 32393105
Colliard A, Ishii A, De Sandre C, Gorostidi F, Sandu K
Eur Arch Otorhinolaryngol 2020 Jan;277(1):293-300. Epub 2019 Oct 10 doi: 10.1007/s00405-019-05677-1. PMID: 31602523
Alataş F, Ozkan R, Metintaş M, Moral H, Erginel S, Uçgun I
Respirology 2003 Mar;8(1):99-103. doi: 10.1046/j.1440-1843.2003.00428.x. PMID: 12856750
Stell PM, Maran AG, Stanley RE, Murray JA
Ann Otol Rhinol Laryngol 1985 Mar-Apr;94(2 Pt 1):108-13. doi: 10.1177/000348948509400202. PMID: 3994225

Diagnosis

Kimura Y, Hirabayashi E, Yano M, Fujitani S, Shioiri S
Auris Nasus Larynx 2023 Aug;50(4):637-640. Epub 2022 Aug 23 doi: 10.1016/j.anl.2022.08.007. PMID: 36114072Free PMC Article
Colliard A, Ishii A, De Sandre C, Gorostidi F, Sandu K
Eur Arch Otorhinolaryngol 2020 Jan;277(1):293-300. Epub 2019 Oct 10 doi: 10.1007/s00405-019-05677-1. PMID: 31602523
Jefferson ND, Cohen AP, Rutter MJ
Semin Pediatr Surg 2016 Jun;25(3):138-43. Epub 2016 Feb 20 doi: 10.1053/j.sempedsurg.2016.02.006. PMID: 27301599
Schroeder JW Jr, Holinger LD
Otolaryngol Clin North Am 2008 Oct;41(5):865-75, viii. doi: 10.1016/j.otc.2008.04.015. PMID: 18775338
Alataş F, Ozkan R, Metintaş M, Moral H, Erginel S, Uçgun I
Respirology 2003 Mar;8(1):99-103. doi: 10.1046/j.1440-1843.2003.00428.x. PMID: 12856750

Therapy

Jiam NT, Dwyer CD, Rosen CA
Laryngoscope 2021 May;131(5):E1633-E1637. Epub 2020 Dec 2 doi: 10.1002/lary.29295. PMID: 33264421
Incandela F, Missale F, Mora F, Marchi F, Fiz I, Piazza C, Peretti G
Eur Arch Otorhinolaryngol 2021 Jan;278(1):149-158. Epub 2020 Jul 23 doi: 10.1007/s00405-020-06210-5. PMID: 32705360Free PMC Article
Xu H, Fan GK
Laryngoscope 2021 Jan;131(1):139-145. Epub 2020 Apr 15 doi: 10.1002/lary.28507. PMID: 32293731
Alataş F, Ozkan R, Metintaş M, Moral H, Erginel S, Uçgun I
Respirology 2003 Mar;8(1):99-103. doi: 10.1046/j.1440-1843.2003.00428.x. PMID: 12856750
Stell PM, Maran AG, Stanley RE, Murray JA
Ann Otol Rhinol Laryngol 1985 Mar-Apr;94(2 Pt 1):108-13. doi: 10.1177/000348948509400202. PMID: 3994225

Prognosis

Meenan K, Bhatnagar K, Guardiani E
Ann Otol Rhinol Laryngol 2021 Sep;130(9):1078-1084. Epub 2021 Feb 15 doi: 10.1177/0003489421995285. PMID: 33583187
Yang MM, Higano NS, Gunatilaka CC, Hysinger EB, Amin RS, Woods JC, Bates AJ
Laryngoscope 2021 Apr;131(4):E1220-E1226. Epub 2020 Oct 14 doi: 10.1002/lary.29169. PMID: 33280109Free PMC Article
Tang Y, Li P, Cua D, Lai J
In Vivo 2020 Jan-Feb;34(1):255-260. doi: 10.21873/invivo.11768. PMID: 31882486Free PMC Article
Lawlor CM, Dombrowski ND, Nuss RC, Rahbar R, Choi SS
Otolaryngol Head Neck Surg 2020 Feb;162(2):234-240. Epub 2019 Dec 17 doi: 10.1177/0194599819893985. PMID: 31842676
Alataş F, Ozkan R, Metintaş M, Moral H, Erginel S, Uçgun I
Respirology 2003 Mar;8(1):99-103. doi: 10.1046/j.1440-1843.2003.00428.x. PMID: 12856750

Clinical prediction guides

Azwal N, Lokanathan Y, Azman M, Ng MH, Mohamed AS, Baki MM
Acta Otorhinolaryngol Ital 2022 Jun;42(3):250-256. doi: 10.14639/0392-100X-N1981. PMID: 35880366Free PMC Article
Schweiger C, Manica D
Semin Pediatr Surg 2021 Jun;30(3):151052. Epub 2021 May 21 doi: 10.1016/j.sempedsurg.2021.151052. PMID: 34172219
Yang MM, Higano NS, Gunatilaka CC, Hysinger EB, Amin RS, Woods JC, Bates AJ
Laryngoscope 2021 Apr;131(4):E1220-E1226. Epub 2020 Oct 14 doi: 10.1002/lary.29169. PMID: 33280109Free PMC Article
Incandela F, Missale F, Mora F, Marchi F, Fiz I, Piazza C, Peretti G
Eur Arch Otorhinolaryngol 2021 Jan;278(1):149-158. Epub 2020 Jul 23 doi: 10.1007/s00405-020-06210-5. PMID: 32705360Free PMC Article
Lawlor CM, Dombrowski ND, Nuss RC, Rahbar R, Choi SS
Otolaryngol Head Neck Surg 2020 Feb;162(2):234-240. Epub 2019 Dec 17 doi: 10.1177/0194599819893985. PMID: 31842676

Recent systematic reviews

Soloperto D, Spinnato F, Di Gioia S, Di Maro F, Pinter P, Bisceglia A, Marchioni D
Int J Pediatr Otorhinolaryngol 2021 Jan;140:110523. Epub 2020 Nov 26 doi: 10.1016/j.ijporl.2020.110523. PMID: 33261859
Jakobsen KK, Grønhøj C, Jensen DH, Fischer-Nielsen A, Hjuler T, von Buchwald C
PLoS One 2017;12(9):e0185283. Epub 2017 Sep 21 doi: 10.1371/journal.pone.0185283. PMID: 28934345Free PMC Article
Wentzel JL, Ahmad SM, Discolo CM, Gillespie MB, Dobbie AM, White DR
Laryngoscope 2014 Jul;124(7):1707-12. Epub 2014 Jan 15 doi: 10.1002/lary.24524. PMID: 24222273

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