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Ankyloglossia

MedGen UID:
56288
Concept ID:
C0152415
Congenital Abnormality
Synonym: Tongue tie
SNOMED CT: Tongue tie (67787004); Ankyloglossia (67787004); Tongue-tie (67787004)
 
HPO: HP:0010296
Monarch Initiative: MONDO:0007125
OMIM®: 106280

Definition

Ankyloglossia, commonly known as 'tongue-tie,' is a congenital anomaly that occurs predominantly in males and is characterized by an abnormally short lingual frenulum. The phenotype varies from absence of clinical significance to rare complete ankyloglossia where the ventral part of the tongue is fused to the floor of the mouth (Klockars, 2007). Some patients also exhibit absence of lower incisors (Acevedo et al., 2010). [from OMIM]

Clinical features

From HPO
Teeth, supernumerary
MedGen UID:
21210
Concept ID:
C0040457
Anatomical Abnormality
The presence of one or more teeth additional to the normal number.
Ankyloglossia
MedGen UID:
56288
Concept ID:
C0152415
Congenital Abnormality
Ankyloglossia, commonly known as 'tongue-tie,' is a congenital anomaly that occurs predominantly in males and is characterized by an abnormally short lingual frenulum. The phenotype varies from absence of clinical significance to rare complete ankyloglossia where the ventral part of the tongue is fused to the floor of the mouth (Klockars, 2007). Some patients also exhibit absence of lower incisors (Acevedo et al., 2010).

Term Hierarchy

Conditions with this feature

Ankyloglossia
MedGen UID:
56288
Concept ID:
C0152415
Congenital Abnormality
Ankyloglossia, commonly known as 'tongue-tie,' is a congenital anomaly that occurs predominantly in males and is characterized by an abnormally short lingual frenulum. The phenotype varies from absence of clinical significance to rare complete ankyloglossia where the ventral part of the tongue is fused to the floor of the mouth (Klockars, 2007). Some patients also exhibit absence of lower incisors (Acevedo et al., 2010).
Orofaciodigital syndrome I
MedGen UID:
307142
Concept ID:
C1510460
Disease or Syndrome
Oral-facial-digital syndrome type I (OFD1) is usually male lethal during gestation and predominantly affects females. OFD1 is characterized by the following features: Oral (lobulated tongue, tongue nodules, cleft of the hard or soft palate, accessory gingival frenulae, hypodontia, and other dental abnormalities). Facial (widely spaced eyes or telecanthus, hypoplasia of the alae nasi, median cleft or pseudocleft upper lip, micrognathia). Digital (brachydactyly, syndactyly, clinodactyly of the fifth finger; duplicated hallux [great toe]). Kidney (polycystic kidney disease). Brain (e.g., intracerebral cysts, agenesis of the corpus callosum, cerebellar agenesis with or without Dandy-Walker malformation). Intellectual disability (in ~50% of individuals).
Cleft palate with or without ankyloglossia, X-linked
MedGen UID:
375520
Concept ID:
C1844830
Disease or Syndrome
X-linked cleft palate and ankyloglossia is a rare, genetic developmental defect during embryogenesis syndrome characterized by the association of complete, partial or submucous cleft palate and ankyloglossia. Patients may also present abnormal uvula (e.g. absent, bifid, shortened or laterally deviated), short lingual frenulum and dental anomalies (e.g. buccal crossbite, absent and/or misshapen teeth). Digital abnormalities, such as mild clinodactyly and/or syndactyly, have also been reported.
Osteocraniostenosis
MedGen UID:
356331
Concept ID:
C1865639
Disease or Syndrome
Gracile bone dysplasia (GCLEB) is a perinatally lethal condition characterized by gracile bones with thin diaphyses, premature closure of basal cranial sutures, and microphthalmia (summary by Unger et al., 2013).
Orofaciodigital syndrome V
MedGen UID:
358131
Concept ID:
C1868118
Disease or Syndrome
Orofaciodigital syndrome V (OFD5) is an autosomal recessive disorder characterized by cleft palate/uvula, lobulated tongue, frontal bossing, hypertelorism, postaxial polydactyly, and impaired intellectual development (summary by Faily et al., 2017).
17q11.2 microduplication syndrome
MedGen UID:
501218
Concept ID:
C3495679
Disease or Syndrome
Syndrome that has characteristics of dysmorphic features and intellectual deficit. It has been described in seven patients within one family. 17q11.2 microduplication encompasses the NF1 region. The underlying mechanism may be non-allelic homologous recombination. The study of pedigree suggests that this microduplication segregates within the family for at least two generations. Two patients displayed a normal clinical presentation, suggesting an autosomal dominant pattern of inheritance with incomplete penetrance.
ADNP-related multiple congenital anomalies - intellectual disability - autism spectrum disorder
MedGen UID:
862975
Concept ID:
C4014538
Disease or Syndrome
ADNP-related disorder is characterized by hypotonia, severe speech and motor delay, mild-to-severe intellectual disability, and characteristic facial features (prominent forehead, high anterior hairline, wide and depressed nasal bridge, and short nose with full, upturned nasal tip) based on a cohort of 78 individuals. Features of autism spectrum disorder are common (stereotypic behavior, impaired social interaction). Other common findings include additional behavioral problems, sleep disturbance, brain abnormalities, seizures, feeding issues, gastrointestinal problems, visual dysfunction (hypermetropia, strabismus, cortical visual impairment), musculoskeletal anomalies, endocrine issues including short stature and hormonal deficiencies, cardiac and urinary tract anomalies, and hearing loss.
Intellectual disability, autosomal dominant 29
MedGen UID:
863578
Concept ID:
C4015141
Mental or Behavioral Dysfunction
SETBP1 haploinsufficiency disorder (SETBP1-HD) is characterized by hypotonia and mild motor developmental delay; intellectual abilities ranging from normal to severe disability; speech and language disorder; behavioral problems (most commonly attention/concentration deficits and hyperactivity, impulsivity), and refractive errors and strabismus. Typically children with SETBP1-HD whose intellect is in the normal or borderline range (IQ 80-90) were diagnosed following genetic testing for behavioral problems and/or severe speech and language disorders (respectively: the inability to produce sounds in words correctly, and deficits in the understanding and/or expression of words and sentences). To date, 47 individuals with SETBP1-HD have been reported.
Tetraamelia syndrome 2
MedGen UID:
1648284
Concept ID:
C4747923
Disease or Syndrome
Tetraamelia syndrome-2 (TETAMS2) is characterized by rudimentary appendages or complete absence of the limbs, usually symmetric, as well as bilateral agenesis of the lungs. There are abnormalities of the pulmonary vasculature and dysmorphic features, including bilateral cleft lip/palate, ankyloglossia, mandibular hypoplasia, microretrognathia, and labioscrotal fold aplasia (Szenker-Ravi et al., 2018). For a discussion of genetic heterogeneity of TETAMS, see 273395.
KINSSHIP syndrome
MedGen UID:
1779339
Concept ID:
C5543317
Disease or Syndrome
KINSSHIP syndrome (KINS) is an autosomal dominant disorder characterized by a recognizable pattern of anomalies including developmental delay, impaired intellectual development, seizures, mesomelic dysplasia, dysmorphic facial features, horseshoe or hypoplastic kidney, and failure to thrive (summary by Voisin et al., 2021).
Ataxia, intention tremor, and hypotonia syndrome, childhood-onset
MedGen UID:
1787902
Concept ID:
C5543478
Disease or Syndrome
Childhood-onset ataxia, intention tremor, and hypotonia syndrome (ATITHS) is a neurodevelopmental disorder characterized by delayed walking due to ataxia, intention tremor, and hypotonia apparent from early childhood. Affected individuals have global developmental delay with mildly impaired intellectual development and speech delay or learning disabilities. Eye movement abnormalities may also be present. Brain imaging shows cerebellar atrophy in some patients (summary by Webb et al., 2021).
Neuroocular syndrome
MedGen UID:
1790414
Concept ID:
C5551362
Disease or Syndrome
Developmental delay, impaired speech, and behavioral abnormalities
MedGen UID:
1794167
Concept ID:
C5561957
Disease or Syndrome
Developmental delay, impaired speech, and behavioral abnormalities (DDISBA) is characterized by global developmental delay apparent from early childhood. Intellectual disability can range from mild to severe. Additional variable features may include dysmorphic facial features, seizures, hypotonia, motor abnormalities such as Tourette syndrome or dystonia, and hearing loss (summary by Cousin et al., 2021).
Congenital disorder of glycosylation, type IIw
MedGen UID:
1794196
Concept ID:
C5561986
Disease or Syndrome
Congenital disorder of glycosylation type IIw (CDG2W) is an autosomal dominant metabolic disorder characterized by liver dysfunction, coagulation deficiencies, and profound abnormalities in N-glycosylation of serum specific proteins. All reported patients carry the same mutation (602671.0017) (summary by Ng et al., 2021). For an overview of congenital disorders of glycosylation, see CDG1A (212065) and CDG2A (212066).
Chilton-Okur-Chung neurodevelopmental syndrome
MedGen UID:
1803276
Concept ID:
C5677022
Disease or Syndrome
Chilton-Okur-Chung neurodevelopmental syndrome (CHOCNS) is characterized mainly by global developmental delay with variably impaired intellectual development and occasional speech delay. Most patients have behavioral abnormalities, including autism spectrum disorder, ADHD, and aggression. About half of patients have dysmorphic facial features, and about half have nonspecific brain abnormalities, including thin corpus callosum. Rare involvement of other organ systems may be present. At least 1 child with normal development at age 2.5 years has been reported (Chilton et al., 2020).
Branchial arch abnormalities, choanal atresia, athelia, hearing loss, and hypothyroidism syndrome
MedGen UID:
1824056
Concept ID:
C5774283
Disease or Syndrome
Branchial arch abnormalities, choanal atresia, athelia, hearing loss, and hypothyroidism syndrome (BCAHH) is an autosomal dominant disorder characterized by choanal atresia, athelia or hypoplastic nipples, branchial sinus abnormalities, neck pits, lacrimal duct anomalies, hearing loss, external ear malformations, and thyroid abnormalities. Additional features may include developmental delay, impaired intellectual development, and growth failure/retardation (summary by Cuvertino et al., 2020 and Baldridge et al., 2020).
Tessadori-Van Haaften neurodevelopmental syndrome 3
MedGen UID:
1824083
Concept ID:
C5774310
Disease or Syndrome
Tessadori-Bicknell-van Haaften neurodevelopmental syndrome-3 (TEBIVANED3) is characterized by global developmental delay with poor overall growth, impaired intellectual development, and speech difficulties. More variable features include hypotonia, microcephaly, and dysmorphic facies. The severity and manifestations of the disorder are highly variable (Tessadori et al., 2022). For a discussion of genetic heterogeneity of Tessadori-Bicknell-van Haaften neurodevelopmental disorder, see TEBIVANED1 (619758).
Intellectual developmental disorder, autosomal dominant 73
MedGen UID:
1841272
Concept ID:
C5830636
Mental or Behavioral Dysfunction
Autosomal dominant intellectual developmental disorder-73 (MRD73) is a highly variable neurodevelopmental disorder characterized by impaired intellectual development that ranges from mild to severe, speech delay, behavioral abnormalities, and nonspecific dysmorphic facial features (Janssen et al., 2022).

Professional guidelines

PubMed

Messner AH, Walsh J, Rosenfeld RM, Schwartz SR, Ishman SL, Baldassari C, Brietzke SE, Darrow DH, Goldstein N, Levi J, Meyer AK, Parikh S, Simons JP, Wohl DL, Lambie E, Satterfield L
Otolaryngol Head Neck Surg 2020 May;162(5):597-611. Epub 2020 Apr 14 doi: 10.1177/0194599820915457. PMID: 32283998
Brzęcka D, Garbacz M, Micał M, Zych B, Lewandowski B
Dev Period Med 2019;23(1):79-87. doi: 10.34763/devperiodmed.20192301.7985. PMID: 30954985Free PMC Article
Walsh J, Tunkel D
JAMA Otolaryngol Head Neck Surg 2017 Oct 1;143(10):1032-1039. doi: 10.1001/jamaoto.2017.0948. PMID: 28715533

Recent clinical studies

Etiology

Cordray H, Mahendran GN, Tey CS, Nemeth J, Raol N
Am J Speech Lang Pathol 2023 Nov 6;32(6):3048-3063. Epub 2023 Aug 22 doi: 10.1044/2023_AJSLP-23-00169. PMID: 37606583
Cordray H, Mahendran GN, Tey CS, Nemeth J, Sutcliffe A, Ingram J, Raol N
Acta Paediatr 2023 Mar;112(3):347-357. Epub 2022 Dec 9 doi: 10.1111/apa.16609. PMID: 36437565
Cruz PV, Souza-Oliveira AC, Notaro SQ, Occhi-Alexandre IGP, Maia RM, De Luca Canto G, Bendo CB, Martins CC
J Am Dent Assoc 2022 Nov;153(11):1026-1040.e31. doi: 10.1016/j.adaj.2022.07.011. PMID: 36307175
Messner AH, Walsh J, Rosenfeld RM, Schwartz SR, Ishman SL, Baldassari C, Brietzke SE, Darrow DH, Goldstein N, Levi J, Meyer AK, Parikh S, Simons JP, Wohl DL, Lambie E, Satterfield L
Otolaryngol Head Neck Surg 2020 May;162(5):597-611. Epub 2020 Apr 14 doi: 10.1177/0194599820915457. PMID: 32283998
Walsh J, Tunkel D
JAMA Otolaryngol Head Neck Surg 2017 Oct 1;143(10):1032-1039. doi: 10.1001/jamaoto.2017.0948. PMID: 28715533

Diagnosis

Cruz PV, Souza-Oliveira AC, Notaro SQ, Occhi-Alexandre IGP, Maia RM, De Luca Canto G, Bendo CB, Martins CC
J Am Dent Assoc 2022 Nov;153(11):1026-1040.e31. doi: 10.1016/j.adaj.2022.07.011. PMID: 36307175
Costa-Romero M, Espínola-Docio B, Paricio-Talayero JM, Díaz-Gómez NM
Arch Argent Pediatr 2021 Dec;119(6):e600-e609. doi: 10.5546/aap.2021.eng.e600. PMID: 34813240
Messner AH, Walsh J, Rosenfeld RM, Schwartz SR, Ishman SL, Baldassari C, Brietzke SE, Darrow DH, Goldstein N, Levi J, Meyer AK, Parikh S, Simons JP, Wohl DL, Lambie E, Satterfield L
Otolaryngol Head Neck Surg 2020 May;162(5):597-611. Epub 2020 Apr 14 doi: 10.1177/0194599820915457. PMID: 32283998
Campanha SMA, Martinelli RLC, Palhares DB
Codas 2019 Feb 25;31(1):e20170264. doi: 10.1590/2317-1782/20182018264. PMID: 30810632
Walsh J, Tunkel D
JAMA Otolaryngol Head Neck Surg 2017 Oct 1;143(10):1032-1039. doi: 10.1001/jamaoto.2017.0948. PMID: 28715533

Therapy

Cordray H, Mahendran GN, Tey CS, Nemeth J, Raol N
Am J Speech Lang Pathol 2023 Nov 6;32(6):3048-3063. Epub 2023 Aug 22 doi: 10.1044/2023_AJSLP-23-00169. PMID: 37606583
Cordray H, Mahendran GN, Tey CS, Nemeth J, Sutcliffe A, Ingram J, Raol N
Acta Paediatr 2023 Mar;112(3):347-357. Epub 2022 Dec 9 doi: 10.1111/apa.16609. PMID: 36437565
Bruney TL, Scime NV, Madubueze A, Chaput KH
Acta Paediatr 2022 May;111(5):940-947. Epub 2022 Feb 21 doi: 10.1111/apa.16289. PMID: 35150472
Wang J, Yang X, Hao S, Wang Y
Int J Paediatr Dent 2022 Mar;32(2):144-156. Epub 2021 May 22 doi: 10.1111/ipd.12802. PMID: 33964037
O'Shea JE, Foster JP, O'Donnell CP, Breathnach D, Jacobs SE, Todd DA, Davis PG
Cochrane Database Syst Rev 2017 Mar 11;3(3):CD011065. doi: 10.1002/14651858.CD011065.pub2. PMID: 28284020Free PMC Article

Prognosis

Wei EX, Meister KD, Balakrishnan K, Cheng AG, Qian ZJ
Otolaryngol Head Neck Surg 2023 Oct;169(4):1020-1027. Epub 2023 Mar 30 doi: 10.1002/ohn.332. PMID: 36994937
Guinot F, Carranza N, Ferrés-Amat E, Carranza M, Veloso A
J Clin Pediatr Dent 2022 Nov;46(6):33-39. Epub 2022 Nov 1 doi: 10.22514/jocpd.2022.023. PMID: 36624902
Baxter R, Merkel-Walsh R, Baxter BS, Lashley A, Rendell NR
Clin Pediatr (Phila) 2020 Sep;59(9-10):885-892. Epub 2020 May 28 doi: 10.1177/0009922820928055. PMID: 32462918
Varadan M, Chopra A, Sanghavi AD, Sivaraman K, Gupta K
J Stomatol Oral Maxillofac Surg 2019 Dec;120(6):549-553. Epub 2019 Jun 27 doi: 10.1016/j.jormas.2019.06.003. PMID: 31255827
Wright JE
J Paediatr Child Health 1995 Aug;31(4):276-8. doi: 10.1111/j.1440-1754.1995.tb00810.x. PMID: 7576881

Clinical prediction guides

Cordray H, Mahendran GN, Tey CS, Nemeth J, Sutcliffe A, Ingram J, Raol N
Acta Paediatr 2023 Mar;112(3):347-357. Epub 2022 Dec 9 doi: 10.1111/apa.16609. PMID: 36437565
Wang J, Yang X, Hao S, Wang Y
Int J Paediatr Dent 2022 Mar;32(2):144-156. Epub 2021 May 22 doi: 10.1111/ipd.12802. PMID: 33964037
Campanha SMA, Martinelli RLC, Palhares DB
Codas 2019 Feb 25;31(1):e20170264. doi: 10.1590/2317-1782/20182018264. PMID: 30810632
Walsh J, Tunkel D
JAMA Otolaryngol Head Neck Surg 2017 Oct 1;143(10):1032-1039. doi: 10.1001/jamaoto.2017.0948. PMID: 28715533
O'Shea JE, Foster JP, O'Donnell CP, Breathnach D, Jacobs SE, Todd DA, Davis PG
Cochrane Database Syst Rev 2017 Mar 11;3(3):CD011065. doi: 10.1002/14651858.CD011065.pub2. PMID: 28284020Free PMC Article

Recent systematic reviews

Cordray H, Mahendran GN, Tey CS, Nemeth J, Raol N
Am J Speech Lang Pathol 2023 Nov 6;32(6):3048-3063. Epub 2023 Aug 22 doi: 10.1044/2023_AJSLP-23-00169. PMID: 37606583
Cordray H, Mahendran GN, Tey CS, Nemeth J, Sutcliffe A, Ingram J, Raol N
Acta Paediatr 2023 Mar;112(3):347-357. Epub 2022 Dec 9 doi: 10.1111/apa.16609. PMID: 36437565
Cruz PV, Souza-Oliveira AC, Notaro SQ, Occhi-Alexandre IGP, Maia RM, De Luca Canto G, Bendo CB, Martins CC
J Am Dent Assoc 2022 Nov;153(11):1026-1040.e31. doi: 10.1016/j.adaj.2022.07.011. PMID: 36307175
González Garrido MDP, Garcia-Munoz C, Rodríguez-Huguet M, Martin-Vega FJ, Gonzalez-Medina G, Vinolo-Gil MJ
Int J Environ Res Public Health 2022 Sep 28;19(19) doi: 10.3390/ijerph191912347. PMID: 36231647Free PMC Article
Wang J, Yang X, Hao S, Wang Y
Int J Paediatr Dent 2022 Mar;32(2):144-156. Epub 2021 May 22 doi: 10.1111/ipd.12802. PMID: 33964037

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