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Laryngomalacia (literally, "soft larynx") is the most common cause of stridor in infancy, in which the soft, immature cartilage of the upper larynx collapses inward during inhalation, causing airway obstruction. It can also be seen in older patients, especially those with neuromuscular conditions resulting in weakness of the muscles of the throat. However, the infantile form is much more common.

Presentation[edit]

In infantile laryngomalacia, the supraglottic larynx (the part above the vocal cords) is tightly curled, with a short band holding the cartilage shield in the front (the epiglottis) tightly to the mobile cartilage in the back of the larynx (the arytenoids). These bands are known as the aryepiglottic folds; they create the movements that opens and closes the vocal cords for phonation. The shortened aryepiglottic folds cause the epiglottis to be curled on itself. This is the well known "omega shaped" epiglottis in laryngomalacia.

Laryngomalacia results in partial airway obstruction, most commonly causing a characteristic high-pitched squeaking noise on inhalation (inspiratory stridor). Some infants have feeding difficulties related to this problem. Rarely, children will have significant life threatening airway obstruction. The vast majority, however, will only have stridor without other more serious symptoms such as dyspnea (difficulty breathing).

Causes[edit]

Although laryngomalacia is not associated with a specific gene, there is evidence that some cases may be inherited.^[1]^[2] Relaxation or a lack of muscle tone in the upper airway may be a factor. It is often worse when the infant is on his or her back, because the floppy tissues can fall over the airway opening more easily in this position.^[3]

Diagnosis[edit]

The physician will ask some questions about the baby’s health problems and may recommend a flexible laryngoscopy to further evaluate the infant's condition.^[3]

Prognosis[edit]

Laryngomalacia becomes symptomatic after the first few months of life, and may get louder over the first year, as the child moves air more vigorously.

Treatment[edit]

Time is the only treatment necessary in more than 90% of infant cases.^[4] In other cases, surgery may be necessary.^[5]^[6]^[7] Most commonly, this involves cutting the aryepiglottic folds to let the supraglottic airway spring open. Treatment of gastroesophageal reflux disease can also help in the treatment of laryngomalacia, since gastric contents can cause the back part of the larynx to swell and collapse even further into the airway. In some cases, a temporary tracheostomy may be necessary.

References[edit]

^ Shulman JB, Hollister DW, Thibeault DW, Krugman ME (1976). "Familial laryngomalacia: a case report". Laryngoscope 86 (1): 84–91. doi:10.1288/00005537-197601000-00018. PMID 1256207.
^ Shohat M, Sivan Y, Taub E, Davidson S (1992). "Autosomal dominant congenital laryngomalacia". Am. J. Med. Genet. 42 (6): 813–4. doi:10.1002/ajmg.1320420613. PMID 1554019.
^ ^a ^b "Laryngomalacia – The Children's Hospital of Philadelphia". The Children's hospital of Philadelphia. Retrieved 31 August 2012.
^ Bye Michael R MD (September 13, 2007). "Laryngomalacia: Treatment & Medication". eMedicine from WebMD.
^ Holinger LD, Konior RJ (1989). "Surgical management of severe laryngomalacia". Laryngoscope 99 (2): 136–42. doi:10.1288/00005537-198902000-00004. PMID 2913424.
^ Zalzal GH (1989). "Stridor and airway compromise". Pediatr. Clin. North Am. 36 (6): 1389–402. PMID 2685719.
^ Solomons NB, Prescott CA (1987). "Laryngomalacia. A review and the surgical management for severe cases". Int. J. Pediatr. Otorhinolaryngol. 13 (1): 31–9. doi:10.1016/0165-5876(87)90005-X. PMID 3305399.

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1. 喉頭の先天異常 congenital anomalies of the larynx
2. 小児における吸気時喘鳴の評価 assessment of stridor in children
3. 血管輪 vascular rings
4. クループの臨床的特徴、評価、および診断 clinical features evaluation and diagnosis of croup
5. 小児における急性呼吸困難の原因 causes of acute respiratory compromise in children

English Journal

Bronchoscopy and airway management in patients with mucopolysaccharidoses (MPS).

Muhlebach MS, Shaffer CB, Georges L, Abode K, Muenzer J.SourceDivision of Pulmonology, Department of Pediatrics, University of North Carolina, Chapel Hill, North Carolina. marianne_muhlebach@med.unc.edu.
Pediatric pulmonology.Pediatr Pulmonol.2012 Sep 4. doi: 10.1002/ppul.22629. [Epub ahead of print]
INTRODUCTION: Mucopolysaccharidoses (MPS) are a group of lysosomal storage disorders characterized by tissue deposition of glycosaminoglycans (GAG). Their musculoskeletal abnormalities and the GAG storage in the airway result in increased risk for patients undergoing anesthesia. This study evaluates
PMID 22949390

A novel sclerosing skeletal dysplasia with mixed sclerosing bone dysplasia, characteristic syndromic features, and clinical and radiographic evidence of male-male transmission.

Murphy-Ryan M, Kirmani S, Thompson DM, Binkovitz LA, Thomas KB, Babovic-Vuksanovic D.SourceMayo Medical School, Rochester, Minnesota.
American journal of medical genetics. Part A.Am J Med Genet A.2012 Sep;158A(9):2292-6. doi: 10.1002/ajmg.a.35497. Epub 2012 Jul 20.
We report on a father and his 4-year-old son sharing a characteristic dysmorphic facial phenotype (including hyperteleroism, prominent forehead, and wide nasal bridge), macrocephaly, hearing loss, palatal clefting, developmental delay, hypotonia and bony abnormalities including marked cranial sclero
PMID 22821701

Japanese Journal

鼻咽腔閉鎖機能不全による摂食・嚥下障害を伴った第1第2鰓弓症候群の1例

玄景華,橋本岳英,片川吉尚,安田順一
岐阜歯科学会雑誌 37(3), 188-194, 2011-02-20
【緒言】第1第2鰓弓症候群は主に顔面、口腔および耳などに変形を生じる先天性疾患であるが、摂食・嚥下障害は少ないとされている。今回は著明な哺乳障害を伴った本症候群患児に対して、摂食機能療法を行い良好な結果を得たので報告する。【症例】4カ月女児。主訴:某大学病院小児科より嚥下リハビリテーション依頼。38週2,296g で帝王切開にて出生し、左耳介欠損、左耳孔閉鎖等を認め、本症候群と診断された。既往歴: …
NAID 110008138685

喉頭顕微鏡下喉頭形成術を行った左披裂軟骨脱臼をともなう喉頭軟化症の1例

福本弘二,漆原直人,鈴木孝明,松岡尚則,福澤宏明,川島章子,渡辺健太郎,長谷川史郎
日本小児外科学会雑誌 46(4), 734-737, 2010-06-20
症例は7歳の女児.出生体重690gの超低出生体重児で生後121日間挿管されており,抜管後より常に陥没呼吸気味であった.その後徐々に上気道閉塞症状が強くなり,4歳時の喉頭ファイバースコピーにて左披裂部の著明な軟化を認め,左披裂軟骨壊死による消失が疑われ気管切開術を施行した.6歳時に喉頭顕微鏡下に喉頭精査を行い,左披裂軟骨脱臼と左披裂部の余剰粘膜による喉頭軟化症と診断,喉頭顕微鏡下に左披裂部余剰粘膜部 …
NAID 110007657522

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リンク元

「喉頭軟化症」

Laryngomalacia
	Classification and external resources
	; Omega shaped epiglottis, seen in laryngomalacia
ICD-10	Q31.5
ICD-9	748.3
OMIM	150280
DiseasesDB	29421
eMedicine	ped/1280

　　[★]

英: laryngomalacia
関: 先天性喘鳴、気管軟化症

先天性喘鳴では最も頻度が高い。
病態としては、喉頭蓋などの喉頭壁構造の軟弱を基盤として、吸気により声門の方に引き込まれて喘鳴が生じる。
症状としては吸気時の喘鳴である。呼吸困難、チアノーゼ、呼吸停止に至る例は稀である。
予後は良好で2歳頃までに自然軽快する事が多いが、難治性の物がある。

参考

1. 喉頭・気管軟化症

http://mymed.jp/di/ixe.html

[1] Shulman JB, Hollister DW, Thibeault DW, Krugman ME (1976). "Familial laryngomalacia: a case report". Laryngoscope 86 (1): 84–91. doi:10.1288/00005537-197601000-00018. PMID 1256207.

[2] Shohat M, Sivan Y, Taub E, Davidson S (1992). "Autosomal dominant congenital laryngomalacia". Am. J. Med. Genet. 42 (6): 813–4. doi:10.1002/ajmg.1320420613. PMID 1554019.

[chop-3] "Laryngomalacia – The Children's Hospital of Philadelphia". The Children's hospital of Philadelphia. Retrieved 31 August 2012.

[4] Bye Michael R MD (September 13, 2007). "Laryngomalacia: Treatment & Medication". eMedicine from WebMD.

[5] Holinger LD, Konior RJ (1989). "Surgical management of severe laryngomalacia". Laryngoscope 99 (2): 136–42. doi:10.1288/00005537-198902000-00004. PMID 2913424.

[6] Zalzal GH (1989). "Stridor and airway compromise". Pediatr. Clin. North Am. 36 (6): 1389–402. PMID 2685719.

[7] Solomons NB, Prescott CA (1987). "Laryngomalacia. A review and the surgical management for severe cases". Int. J. Pediatr. Otorhinolaryngol. 13 (1): 31–9. doi:10.1016/0165-5876(87)90005-X. PMID 3305399.

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案内

laryngomalacia