Palate development involves a genetic regulation through a complex molecular mechanism that may be disrupted by environmental factors, resulting in impaired fusion and cleft palate formation. An encounter with a case of cleft palate due to dorsal tongue hamartoma prompted us to perform this systematic review.
Objective
To review the clinical profile and management approach for a case with cleft palate and tongue hamartoma.
Design
A systematic literature search was conducted using keywords related to cleft palate and tongue hamartoma in PubMed, Scopus, MEDLINE, and Scielo databases through December 2021, with no time or language restrictions.
Patients, Participants
Studies reporting patients with cleft palate and tongue hamartoma were included.
Main outcome measure(s)
Information related to clinical profile, diagnostic tests, histopathology, management, and outcomes were extracted.
Results
Fourteen relevant publications were identified with 16 cases reported so far. Among them, thirteen patients were females (81.25%), and 3 were males (18.75%). The age of presentation varied from birth to 19 years. Oral-facial-digital syndrome (type II) was the most commonly associated syndrome.
Conclusion
Congenital tongue hamartoma with cleft palate is a rare presentation, which can present as an isolated entity or part of a syndrome. Genetic evaluation is warranted, particularly for multiple hamartomatous lesions. The preferred treatment is immediate excision of hamartoma while following a standard timeline for palatoplasty.
FergusonMWJ. Developmental mechanisms in normal and abnormal palate formation with particular reference to the aetiology, pathogenesis and prevention of cleft palate. Br J Orthod. 1981;8(3):115‐137.
GosainAKMooreFO. Embryology of the head neck. In: AstonSJBeaslyRVThroneCHM, (eds). Grabb and Smith’s Plastic Surgery. 5th ed. Philadelphia, PA: Lippincott-Raven Publishers;1997:223.
LanYXuJJiangR. Cellular and molecular mechanisms of palatogenesis. Curr Top Dev Biol. 2015;115:59‐84.
7.
BurgMLChaiYYaoCAMageeWFigueiredoJC. Epidemiology, etiology, and treatment of isolated cleft palate. Front Physiol. 2016;7:67.
8.
DursyE. Zur Entwicklungsgeschichte des Kopfes des Menschen und der höheren Wirbelthiere: Mit Holzschnitten und einem Atlas von neun Kupfertafeln mit erklärendem Texte. Laupp; 1869.
9.
HisW. Die entwicklung der menschlichen und thierischen physiognomien. Arch. fiir An at. u. Entwicklungsgesch. Anat. Abth. 1892
10.
VeauV. Hasenscharten menschlicher keimlinge auf der stufe 21–23 mm S. St. L. Zeitschrift für Anatomie und Entwicklungsgeschichte. 1938;108(3):459‐493.
11.
PattenBM. The normal development of the facial region. In: PruznznskyS, (ed). Congenital Anomalies of the Face and Associated Structures. Springfield, Ill: Charles C Thomas Publisher; 1961:11‐45.
12.
CarstensMH. Neural tube programming and craniofacial cleft formation. I. The neuromeric organization of the head and neck. Eur J Paediatr Neurol. 2004;8(4):181‐210.
13.
ChaiYMaxsonRE. Recent advances in craniofacial morphogenesis. Dev Dyn Off Publ Am Assoc Anat. 2006;235(9):2353‐2375.
14.
SmithTMLozanoffSIyyanarPPNazaraliAJ. Molecular signaling along the anterior-posterior axis of early palate development. Front Physiol. 2012;3:488.
15.
SchoenCAschrafiAThonissenMPoelmansGVon den HoffJWCarelsCEL. MicroRNAs in palatogenesis and cleft palate. Front Physiol. 2017;8:165.
16.
JohnstonMCSulikK. Embryology of the head neck. In SerafinDGeorgiadeNG, (eds). Pediatric Plastic Surgery. St Louis: Mosby; 1984:184‐215.
17.
HiebertJCJohnsonABTranHHYuZGladeRS. Congenital tongue mass with concomitant cleft palate and bifid tongue: a case report and review of the literature. Cleft Palate-Craniofacial J. 2016;53(2):245‐248.
18.
MoherDLiberatiATetzlaffJAltmanDG, The PRISMA Group. Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement. PLoS Med. 2009;6(7):e1000097.
19.
BhattacharyaVKhannaSAdil BashirSKumarUGarbyalRS. Cleft palate associated with hamartomatous bifid tongue. Report of two cases. J Plast Reconstr Aesthet Surg. 2009;62(11):1442‐1445.
20.
VelepicMSSassoAVelepicMMLusticaIStarcevicRKomljenovicD. Combined anomalies of the palate in Mohr syndrome: is preoperative electromyography of the palate useful?J Pediatr Surg. 2004;39(2):220‐222.
21.
IshiiTTakemoriSSuzukiJI. Hamartoma of the tongue: report of a case. Arch Otolaryngol. 1968;88(2):171‐173.
22.
DemuthRJJohnsDF. Recurrent aspiration pneumonitis in a cleft palate child with hamartoma of the tongue. Cleft Palate J. 1981;18(4):299‐303.
23.
MiyamotoYNagayamaMHayashiY. A cleft palate child with lobulated tongue and lingual hamartoma: report of a case. J Oral Maxillofac Surg. 1991;49(6):644‐646.
24.
HannaRArgenyiZBBendaJA. Hamartoma of the tongue in an infant with a primary diagnosis of ectrodactyly-ectodermal dysplasia-cleft lip and palate syndrome. J Cutan Pathol. 1994;21(2):173‐178.
25.
HaugKKhanSFuchsSKönigR. OFD II, OFD VI, and Joubert syndrome manifestations in 2 sibs. Am J Med Genet. 2000;91(2):135‐137. <135::aid-ajmg11>3.0.co;2-1
26.
TorielloHVLemireEG. Optic nerve coloboma, Dandy-Walker malformation, microglossia, tongue hamartomata, cleft palate and apneic spells: an existing oral-facial-digital syndrome or a new variant?: Clin Dysmorphol. 2002;11(1):19‐23.
27.
NoguchiTJinbuYItohHMatsumotoKSakaiOKusamaM. Epignathus combined with cleft palate, lobulated tongue, and lingual hamartoma: report of a case. Oral Surg Oral Med Oral Pathol Oral Radiol Endodontol. 2006;101(4):481‐486.
28.
JankSKeldererHWRaubenheimerEJPuelacherW. Medial tongue cleft associated with intraoral hamartoma—case report and review of literature. Int J Oral Maxillofac Surg. 2008;37(3):296‐299.
29.
DaramolaOOSuchiMChunRH. Lingual hamartoma associated with a cleft palate in a newborn. Ear Nose Throat J. 2014;93(10–11):E9‐E37.
30.
MbaUOnahI. Tongue harmatoma in association with cleft palate: case report. J Cleft Lip Palate Craniofacial Anom. 2017;4(2):168.
31.
YinSZhaoZ. Incomplete cleft palate, bifid tongue, and oral hamartomas: a rare congenital anomaly. J Craniofac Surg. 2020;31(2):e184‐e185.
32.
ZhanXCaoZZChenRRChenBB. [Giant teratoma of nasopharynx with cleft palate and lingual hamartoma in the newborn: a case report]. Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2019;54(9):695‐697.
33.
MartinelliMPalmieriACarinciFScapoliL. Non-syndromic cleft palate: an overview on human genetic and environmental risk factors. Front Cell Dev Biol. 2020 Oct 20;8:592271. .
34.
BushJOJiangR. Palatogenesis: morphogenetic and molecular mechanisms of secondary palate development. Dev Camb Engl. 2012;139(2):231‐243.
35.
LeslieEJMarazitaML. Genetics of cleft lip and cleft palate. Am J Med Genet C Semin Med Genet. 2013;163C(4):246‐258. . Epub 2013 Oct 4.
36.
HansonJWSmithDW. U-shaped palatal defect in the robin anomalad: developmental and clinical relevance. J Pediatr. 1975;87(1):30‐33.
37.
WidgerowAD. Klippel-Feil anomaly, cleft palate, and bifid tongue. Ann Plast Surg. 1990;25(3):216‐222.
38.
ChapmanMCSoaresBPLiY, et al. Congenital oral masses: an anatomic approach to diagnosis. RadioGraphics. 2019;39(4):1143‐1160.
39.
TakagiYMachidaJSatoHSakumaHNodaHOh-iwaI. Congenital bifid tongue with lingual hamartoma: a case report and review of the literature. J Oral Maxillofac Surg Med Pathol. 2016;28(2):133‐137.
40.
KreigerPAErnstLMEldenLMKazahayaKAlawiFRussoPA. Hamartomatous tongue lesions in children. Am J Surg Pathol. 2007;31(8):1186‐1190.
41.
GooldALKochBLWillgingJP. Lingual hamartoma in an infant: CT and MR imaging. Am J Neuroradiol. 2007;28(1):30‐31.
42.
DonnellyLFJonesBVStrifeJL. Imaging of pediatric tongue abnormalities. AJR Am J Roentgenol. 2000;175(2):489‐493.
43.
KhanILeventhalBL. Developmental delay. [updated 2021 Aug 4]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022. Available from: https://www.ncbi.nlm.nih.gov/books/NBK562231/
44.
LloydKMDennisM. Cowden’s disease. A possible new symptom complex with multiple system involvement. Ann Intern Med. 1963;58:136‐142.
45.
Mink van der MolenABvan BreugelJMMJanssenNG, et al. Clinical practice guidelines on the treatment of patients with cleft lip, alveolus, and palate: an executive summary. J Clin Med. 2021;10(21):4813.
