The Cleft Palate–Craniofacial Journal 53(2) pp. 245–248 March 2016 Ó Copyright 2016 American Cleft Palate–Craniofacial Association
CASE REPORT Congenital Tongue Mass With Concomitant Cleft Palate and Bifid Tongue: A Case Report and Review of the Literature Jared C. Hiebert, M.D., Adam B. Johnson, M.D., H. Henry Tran, M.D., Zhongxin Yu, M.D., Robert S. Glade, M.D. A case report of a congenital, lingual, salivary gland choristoma with bifid tongue and cleft palate is presented. The patient was born with airway obstruction in supine positioning. Laryngoscopy revealed a midline tongue mass that extended into the hypopharyx and pathological examination showed a congenital ectopic salivary gland. The bifid tongue was repaired at the time of surgical excision. Literature review revealed nine additional cases of congenital lingual mass, bifid tongue, and cleft palate. The most common tongue mass reported was hamartoma (40%), but the differential diagnoses include hamartoma, teratoma, and salivary choristoma. KEY WORDS:
bifid tongue, ectopic salivary gland, tongue mass
The patient was taken to the operating room on the third day of life for an examination under anesthesia and direct laryngoscopy and bronchoscopy and controlled intubation in preparation for magnetic resonance imaging (MRI). The airway examination revealed no synchronous lesions or other oral anomalies. The midline mass could be pulled outside the oral cavity, which extended the tongue and opened the airway. The MRI revealed no additional abnormalities. The next day the patient underwent surgery to excise the lingual mass and glossoplasty (Fig. 1). The patient was nasally reintubated in the operating room, the mass was excised under direct visualization, and the medial mucosa of the bifid tongue was excised with the CO2 laser. The tongue was then approximated with dissolvable sutures. The patient was left intubated secondary to anticipated tongue swelling. The tongue specimen consisted of a well-circumscribed polypoid mass partially covered by glistening tan mucosa. It measured 2.1 3 1.3 cm. The cut surfaces revealed homogeneous white-tan tissue with a vaguely nodular appearance in the central portion of the tissue. There was no necrosis, hemorrhage, or evidence of malignancy. Histologically, the mass was predominately composed of lobular salivary gland tissue that was partially surrounded by skeletal muscles and squamous epithelium of the tongue (Fig. 2A). The squamous epithelium and skeletal muscle were unremarkable. The salivary gland tissue had a normal lobular architecture (Fig. 2B) and was composed predominately of serous acini with associated excretory ducts (Fig. 2C and 2D) resembling the histology of a normal major salivary gland. The pathological diagnosis was salivary gland choristoma, that is, salivary gland tissue within the parenchyma of the tongue (choristoma means ‘‘the right tissue with the right architecture in the wrong place’’). Genetic testing, comparative genomic hybridization, and florescence in situ hybridization also revealed no abnormalities.
Congenital tongue lesions associated with cleft palate and bifid tongue are rare; only nine other cases have been adequately described in the literature. We report a case of a congenital salivary gland choristoma with concomitant bifid tongue and cleft palate, only the third reported. A 3.13-kg girl born at 41 weeks was transferred on the second day of life to our neonatal intensive care unit for evaluation of cleft palate, midline tongue mass, and bifid tongue. At birth, the patient was noted to have respiratory difficulty and oxygen desaturations requiring supplemental oxygen when lying supine or using a pacifier. Respiratory status improved when the patient was placed prone. The otorhinolaryngology service was consulted, and examination revealed a midline incomplete, secondary, U-shaped cleft palate, bifid tongue and a pedunculated, 2-cm mass that extended from the midline base of the tongue. Because of multiple congenital anomalies, an echocardiogram was performed and revealed a ventricular septal defect, a patent foramen ovale, and patent ductus arteriosus for which the cardiology department was consulted.
Dr. Hiebert MD is Resident Physician, Department of Otorhinolaryngology, University of Oklahoma Health Sciences Center; Dr. Johnson is Resident Physician, Department of Otorhinolaryngology, University of Oklahoma Health Sciences Center; Dr. Tran is Resident Physician, Department of Pathology, University of Oklahoma Health Sciences Center; Dr. Yu is Assistant Professor, Department of Pathology, University of Oklahoma Health Sciences Center; and Dr. Glade is Assistant Professor, Department of Otorhinolaryngology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma. Presented as a poster presentation at the American Cleft PalateCraniofacial Association 72nd Annual Meeting, Palm Springs, California, April 21–25, 2015. Submitted February 2015; Revised May 2015; Accepted May 2015. Address correspondence to: Dr. Jared C. Hiebert, Department of Otorhinolaryngology, University of Oklahoma Health Sciences Center, 920 Stanton L. Young Boulevard, WP 1290, Oklahoma City, OK 73014. E-mail [email protected]. DOI: 10.1597/15-062 245
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FIGURE 1 A: Pedunculated tongue mass before excision. B: Excised tongue mass. C: Bifid tongue after excision of tongue mass. D: Glossoplasty. E: Secondary cleft palate.
The patient was started on nasogastric feeding on postoperative day 1 (day of life 5). Before this she had been on total parenteral nutrition administered by the neonatal intensive care service. She was evaluated by the speech pathologist on postoperative day 6 and cleared for oral feeding; to this end she was trained to use a pigeon nipple. The child was being completely orally fed by day 12 and was discharged home. The patient’s weight had increased to 3.25 kg at discharge. The 2-month follow-up revealed that the patient was doing well and using a pigeon nipple; she was gaining weight appropriately and now weighed 4.13 kg. There were no further complaints of obstruction. Her tongue was mobile and well healed. Repair of her ventricular septal defect was done at 4 months of age, and her palate was
repaired at 11 months of age. By all accounts, at 12 months of age, the patient was doing well and had no evidence of further developmental abnormalities or delays. DISCUSSION Congenital lingual masses are rare and have broad differential diagnoses, including hamartoma; teratoma; hemangioma; rhabdomyosarcoma; lingual cyst; ectopic salivary, glial, or thyroid tissue; and fibrolipoma. The presence of congenital lingual mass with synchronous bifid tongue and cleft palate is rarer still. A review of the literature for similar reports of a triad of cleft palate, bifid tongue, and midline lingual mass revealed nine additional cases. Only reports with adequate description and pathol-
Hiebert et al., TONGUE MASS WITH CLEFT PALATE AND BIFID TONGUE
247
FIGURE 2 A: Histologically, the mass is predominately composed of lobular salivary gland tissue (right lower portion of the picture, indicated by double black arrows), which is partially covered by skeletal muscles (middle portion of the picture, indicated by an orange arrow), and squamous epithelium (left upper portion of the picture, indicated by a blue arrow) of the tongue (H&E stain). B: The salivary gland has a lobular architecture with acini and associated excretory ducts, which are in the center of the lobules (indicated by black arrows), histologically resembling a normal salivary gland (H&E stain). C: Most of the acini are composed of serous epithelial cells with a basal nucleus and dense cytoplasm packed with basophilic zymogen granules (H&E stain). D: These zymogen granules are positive for PAS stain. The arrow indicates one of the acini with cells filled with PAS-positive zymogen granules (PAS stain).
ogy were included. Clinical data are presented in Table 1. Pathology of the lingual masses included hamartoma (Vandenhaute et al., 2000; Noguchi et al., 2006; Jank et al., 2008; Bhattacharya et al., 2009), teratoma (Bonet et al., 2012), and salivary tissue (Britto et al., 2000; Haghigi et al., 2004). Six patients had additional congenital anomalies, including oronasal-pharyngeal teratomas, nasal dermoid cyst, dorsal hemivertebra, vitreous hemorrhage, and cardiac anomalies. The present case is unique in that pathology of the tongue mass was salivary choristoma; thus it represents only the third case reported in the pediatric literature (Britto et al., 2000; Haghigi et al., 2004). Ectopic salivary tissue is rare, and cases have been described involving multiple areas of the head and neck, including the tonsil (Wise et al., 2005), gingiva (Ledesma-Montes et al., 1998), neck (Guerrissi, 2000), and even the middle ear (Lee et al., 2006). These findings are likely the result of aberrant embryologic development of the minor/major salivary glands (Guerrissi, 2000; Wise et al., 2005). The cleft palate associated with lingual masses has been suggested to occur as a result of the developmental sequence of the tongue and palate (Bhattacharya et al., 2009). A large tongue or tongue mass developing before palate formation prevents the palatal shelves from fusing in the midline. Similarly, the tongue mass prevents fusion of
the embryologic tongue buds. Instances of cleft formation as a result of tongue obstruction are also seen in PierreRobin sequence or such congenital anomalies as KlippelFeil syndrome (Widgerow, 1990). Although our patient tested negative for any genetic abnormalities, patients presenting with a triad of cleft palate, bifid tongue, and midline lingual mass should be evaluated by genetic testing to assist with diagnostic and prognostic information. At 12 months of age, our patient has shown no evidence of developmental delay, and the literature is deficient in description of developmental progress in this group of patients. However, cleft palate and midline defects can be associated with a variety of syndromes, including oro-facial-digital syndrome (Gurrieri et al., 2007). When counseling concerned parents, it is reasonable to discuss that in the absence of a genetic diagnosis or other significant anomalies, there is no good evidence to suggest further cognitive or developmental abnormalities but the patient should be followed closely. Additionally, it is prudent to obtain preoperative imaging (computed tomography or MRI) in these patients to assess for other head and neck pathology and evaluate the extent of the lesion. Investigators have also obtained technetium (Bhattacharya et al., 2009) and radioactive iodine (Britto et
Hamartoma Salivary choristoma Hamartoma
Salivary choristoma
Soybean-size 2.1 3 1.3
2.5 3 1.7 None
99m
18 d Birth M 10 (Britto et al., 2000)
* AP ¼ age at presentation; AS ¼ age at surgery; F ¼ female; ductus arteriosus. † Time of glossoplasty for bifid tongue. ‡ Present case.
Palatal teratoma, VSD VSD, PFO, PDA None
Respiratory difficulty Respiratory and feeding difficulty Respiratory difficulty F F F 7 (Noguchi et al., 2006) 8‡ 9 (Jank et al., 2008)
Birth Birth Birth
Tc ¼ technetium; M ¼ male; CT ¼ computed tomography; MRI ¼ magnetic resonance imaging; VSD ¼ ventricular septal defect; PFO ¼ patent foramen ovale; PDA ¼ patent
Salivary choristoma 232 M 6 (Haghigi et al., 2004)
Birth
20 d, 6 mo† F 5 (Bonet et al., 2012)
Birth
4 wk, 8 wk, 6 mo† 9d 4d 3 mo
Respiratory difficulty, failed oral feeds
Vomer teratoma Nasal dermoid cyst, posterior pharyngeal wall teratoma Dorsal hemivertebra, vitreous hemorrhage Nasopharyngeal teratoma Respiratory difficulty 24 d, 6 mo† 2 d, 6 mo† F M 3 (Bonet et al., 2012) 4 (Bonet et al., 2012)
Birth Birth
None Speech impairment 19 y F 2 (Bhattacharya et al., 2009)
19 y
9 mo F 1 (Bhattacharya et al., 2009)
9 mo
MRI, Iodine-131
Teratoma 232
CT, MRI, radiolabeled iodine scan CT, postoperative MRI MRI MRI
Teratoma Teratoma 1.5 3 2 2.5 3 2
Hamartoma
2.5 3 3 Tc pertechnetate thyroid scan Tc pertechnetate thyroid scan
99m
None Choking with feedings
Associated Malformation Reported Clinical Symptoms AS AP Gender Case Number and Reference
Data of Patients With Clinical Triad of Cleft Palate, Bifid Tongue, and Lingual Mass* TABLE 1
99m
Tongue Mass Size (cm) Imaging
Hamartoma
Cleft Palate–Craniofacial Journal, March 2016, Vol. 53 No. 2
Tongue Mass Histology
248
al., 2000; Haghigi et al., 2004) scans to rule out ectopic thyroid tissue. Congenital lingual masses are a rare clinical entity and may cause significant airway and feeding difficulties. Our patient had significant airway obstruction in the supine position that resolved after a single-stage procedure of mass resection and tongue reconstruction. We advocate for prompt evaluation and treatment of these patients in order to provide a safe airway and allow for early oral feeding. CONCLUSION Our patient presented at birth with a symptomatic lingual salivary choristoma and concomitant bifid tongue and cleft palate. These were isolated findings with no associated genetic abnormality. Immediate resection and reconstruction resulted in an excellent outcome. This is an unusual case, and salivary choristoma should be in the differential diagnosis when evaluating patients with cleft palate, bifid tongue, and a midline lingual mass. REFERENCES Bhattacharya V, Khanna S, Bashir SA, Kumar U, Garbyal RS. Cleft palate associated with hamartomatous bifid tongue. Report of two cases. J Plast Reconstr Aesthet Surg. 2009;62:1442–1445. Bonet C, Penarrocha-Oltra D, Minguez JM, Vera-Sirera B, Penarrocha-Diago M, Penarrocha-Diago M. Oral teratomas: a report of 5 cases. J Oral Max Surg. 2012;70:2809–2813. Britto JA, Ragoowansi RH, Sommerlad BC. Double tongue, intraoral anomalies, and cleft palate—case reports and a discussion of developmental pathology. Cleft Palate Craniofac J. 2000;37:410–417. Guerrissi JO. Cervical tumor by ectopic salivary gland. J Craniofac Surg. 2000;11:394–397. Lee DK, Kim JH, Cho Y, Chung W, Hong SH. Salivary gland choristoma of the middle ear in an infant: a case report. Int J Pediatr Otorhinolaryngol. 2006;70:167–170. Gurrieri F, Franco B, Toriello H, Neri G. Oral–facial–digital syndromes: review and diagnostic guidelines. Am J Med Genet Part A. 2007;143A:3314–3323. Haghigi K, Milles M, Cleveland D, Ziccardi V. Epignathus teratoma with bifid tongue and median glossal salivary mass: report of a case. J Oral Maxillofac Surg. 2004;62:379–383. Jank S, Kelderer HW, Raubenheimer EJ, Puelacher W. Medial tongue cleft associated with intraoral hamartoma—case report and review of the literature. Int J Oral Maxillofac Surg. 2008;37:296–299. ´ ´ ´ız M, PortillaLedesma-Montes C, Ferna´ndez-Lopez R, Garces-Ort Robertson J, Herna´ndez-Guerrero JC. Gingival salivary gland choristoma. A case report. J Periodontol. 1998;69:1164–1166. Noguchi T, Jinbu Y, Itoh H, Matsumoto K, Sakai O, Kusama M. Epignathus combined with cleft palate, lobulated tongue, and lingual hamartoma: report of a case. Oral Pathol Oral Radiol Endod. 2006;101:481–486. Vandenhaute B, Leteurtre E, Lecomte-Houcke M, Pellerin P, Nuyts JP, Cuisset JM, Soto-Ares G. Epignathus teratoma: report of three cases with a review of the literature. Cleft Palate Craniofac J. 2000;37:83–91. Widgerow AD. Klippel-Feil anomaly, cleft palate, and bifid tongue. Ann Plast Surg. 1990;25:216–22. Wise JB, Sehgal K, Guttenberg M, Shah UK. Ectopic salivary tissue of the tonsil: a case report. Int J Pediatr Otorhinolaryngol. 2005;69:567–571.
CASE REPORT Congenital Tongue Mass With Concomitant Cleft Palate and Bifid Tongue: A Case Report and Review of the Literature Jared C. Hiebert, M.D., Adam B. Johnson, M.D., H. Henry Tran, M.D., Zhongxin Yu, M.D., Robert S. Glade, M.D. A case report of a congenital, lingual, salivary gland choristoma with bifid tongue and cleft palate is presented. The patient was born with airway obstruction in supine positioning. Laryngoscopy revealed a midline tongue mass that extended into the hypopharyx and pathological examination showed a congenital ectopic salivary gland. The bifid tongue was repaired at the time of surgical excision. Literature review revealed nine additional cases of congenital lingual mass, bifid tongue, and cleft palate. The most common tongue mass reported was hamartoma (40%), but the differential diagnoses include hamartoma, teratoma, and salivary choristoma. KEY WORDS:
bifid tongue, ectopic salivary gland, tongue mass
The patient was taken to the operating room on the third day of life for an examination under anesthesia and direct laryngoscopy and bronchoscopy and controlled intubation in preparation for magnetic resonance imaging (MRI). The airway examination revealed no synchronous lesions or other oral anomalies. The midline mass could be pulled outside the oral cavity, which extended the tongue and opened the airway. The MRI revealed no additional abnormalities. The next day the patient underwent surgery to excise the lingual mass and glossoplasty (Fig. 1). The patient was nasally reintubated in the operating room, the mass was excised under direct visualization, and the medial mucosa of the bifid tongue was excised with the CO2 laser. The tongue was then approximated with dissolvable sutures. The patient was left intubated secondary to anticipated tongue swelling. The tongue specimen consisted of a well-circumscribed polypoid mass partially covered by glistening tan mucosa. It measured 2.1 3 1.3 cm. The cut surfaces revealed homogeneous white-tan tissue with a vaguely nodular appearance in the central portion of the tissue. There was no necrosis, hemorrhage, or evidence of malignancy. Histologically, the mass was predominately composed of lobular salivary gland tissue that was partially surrounded by skeletal muscles and squamous epithelium of the tongue (Fig. 2A). The squamous epithelium and skeletal muscle were unremarkable. The salivary gland tissue had a normal lobular architecture (Fig. 2B) and was composed predominately of serous acini with associated excretory ducts (Fig. 2C and 2D) resembling the histology of a normal major salivary gland. The pathological diagnosis was salivary gland choristoma, that is, salivary gland tissue within the parenchyma of the tongue (choristoma means ‘‘the right tissue with the right architecture in the wrong place’’). Genetic testing, comparative genomic hybridization, and florescence in situ hybridization also revealed no abnormalities.
Congenital tongue lesions associated with cleft palate and bifid tongue are rare; only nine other cases have been adequately described in the literature. We report a case of a congenital salivary gland choristoma with concomitant bifid tongue and cleft palate, only the third reported. A 3.13-kg girl born at 41 weeks was transferred on the second day of life to our neonatal intensive care unit for evaluation of cleft palate, midline tongue mass, and bifid tongue. At birth, the patient was noted to have respiratory difficulty and oxygen desaturations requiring supplemental oxygen when lying supine or using a pacifier. Respiratory status improved when the patient was placed prone. The otorhinolaryngology service was consulted, and examination revealed a midline incomplete, secondary, U-shaped cleft palate, bifid tongue and a pedunculated, 2-cm mass that extended from the midline base of the tongue. Because of multiple congenital anomalies, an echocardiogram was performed and revealed a ventricular septal defect, a patent foramen ovale, and patent ductus arteriosus for which the cardiology department was consulted.
Dr. Hiebert MD is Resident Physician, Department of Otorhinolaryngology, University of Oklahoma Health Sciences Center; Dr. Johnson is Resident Physician, Department of Otorhinolaryngology, University of Oklahoma Health Sciences Center; Dr. Tran is Resident Physician, Department of Pathology, University of Oklahoma Health Sciences Center; Dr. Yu is Assistant Professor, Department of Pathology, University of Oklahoma Health Sciences Center; and Dr. Glade is Assistant Professor, Department of Otorhinolaryngology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma. Presented as a poster presentation at the American Cleft PalateCraniofacial Association 72nd Annual Meeting, Palm Springs, California, April 21–25, 2015. Submitted February 2015; Revised May 2015; Accepted May 2015. Address correspondence to: Dr. Jared C. Hiebert, Department of Otorhinolaryngology, University of Oklahoma Health Sciences Center, 920 Stanton L. Young Boulevard, WP 1290, Oklahoma City, OK 73014. E-mail [email protected]. DOI: 10.1597/15-062 245
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Cleft Palate–Craniofacial Journal, March 2016, Vol. 53 No. 2
FIGURE 1 A: Pedunculated tongue mass before excision. B: Excised tongue mass. C: Bifid tongue after excision of tongue mass. D: Glossoplasty. E: Secondary cleft palate.
The patient was started on nasogastric feeding on postoperative day 1 (day of life 5). Before this she had been on total parenteral nutrition administered by the neonatal intensive care service. She was evaluated by the speech pathologist on postoperative day 6 and cleared for oral feeding; to this end she was trained to use a pigeon nipple. The child was being completely orally fed by day 12 and was discharged home. The patient’s weight had increased to 3.25 kg at discharge. The 2-month follow-up revealed that the patient was doing well and using a pigeon nipple; she was gaining weight appropriately and now weighed 4.13 kg. There were no further complaints of obstruction. Her tongue was mobile and well healed. Repair of her ventricular septal defect was done at 4 months of age, and her palate was
repaired at 11 months of age. By all accounts, at 12 months of age, the patient was doing well and had no evidence of further developmental abnormalities or delays. DISCUSSION Congenital lingual masses are rare and have broad differential diagnoses, including hamartoma; teratoma; hemangioma; rhabdomyosarcoma; lingual cyst; ectopic salivary, glial, or thyroid tissue; and fibrolipoma. The presence of congenital lingual mass with synchronous bifid tongue and cleft palate is rarer still. A review of the literature for similar reports of a triad of cleft palate, bifid tongue, and midline lingual mass revealed nine additional cases. Only reports with adequate description and pathol-
Hiebert et al., TONGUE MASS WITH CLEFT PALATE AND BIFID TONGUE
247
FIGURE 2 A: Histologically, the mass is predominately composed of lobular salivary gland tissue (right lower portion of the picture, indicated by double black arrows), which is partially covered by skeletal muscles (middle portion of the picture, indicated by an orange arrow), and squamous epithelium (left upper portion of the picture, indicated by a blue arrow) of the tongue (H&E stain). B: The salivary gland has a lobular architecture with acini and associated excretory ducts, which are in the center of the lobules (indicated by black arrows), histologically resembling a normal salivary gland (H&E stain). C: Most of the acini are composed of serous epithelial cells with a basal nucleus and dense cytoplasm packed with basophilic zymogen granules (H&E stain). D: These zymogen granules are positive for PAS stain. The arrow indicates one of the acini with cells filled with PAS-positive zymogen granules (PAS stain).
ogy were included. Clinical data are presented in Table 1. Pathology of the lingual masses included hamartoma (Vandenhaute et al., 2000; Noguchi et al., 2006; Jank et al., 2008; Bhattacharya et al., 2009), teratoma (Bonet et al., 2012), and salivary tissue (Britto et al., 2000; Haghigi et al., 2004). Six patients had additional congenital anomalies, including oronasal-pharyngeal teratomas, nasal dermoid cyst, dorsal hemivertebra, vitreous hemorrhage, and cardiac anomalies. The present case is unique in that pathology of the tongue mass was salivary choristoma; thus it represents only the third case reported in the pediatric literature (Britto et al., 2000; Haghigi et al., 2004). Ectopic salivary tissue is rare, and cases have been described involving multiple areas of the head and neck, including the tonsil (Wise et al., 2005), gingiva (Ledesma-Montes et al., 1998), neck (Guerrissi, 2000), and even the middle ear (Lee et al., 2006). These findings are likely the result of aberrant embryologic development of the minor/major salivary glands (Guerrissi, 2000; Wise et al., 2005). The cleft palate associated with lingual masses has been suggested to occur as a result of the developmental sequence of the tongue and palate (Bhattacharya et al., 2009). A large tongue or tongue mass developing before palate formation prevents the palatal shelves from fusing in the midline. Similarly, the tongue mass prevents fusion of
the embryologic tongue buds. Instances of cleft formation as a result of tongue obstruction are also seen in PierreRobin sequence or such congenital anomalies as KlippelFeil syndrome (Widgerow, 1990). Although our patient tested negative for any genetic abnormalities, patients presenting with a triad of cleft palate, bifid tongue, and midline lingual mass should be evaluated by genetic testing to assist with diagnostic and prognostic information. At 12 months of age, our patient has shown no evidence of developmental delay, and the literature is deficient in description of developmental progress in this group of patients. However, cleft palate and midline defects can be associated with a variety of syndromes, including oro-facial-digital syndrome (Gurrieri et al., 2007). When counseling concerned parents, it is reasonable to discuss that in the absence of a genetic diagnosis or other significant anomalies, there is no good evidence to suggest further cognitive or developmental abnormalities but the patient should be followed closely. Additionally, it is prudent to obtain preoperative imaging (computed tomography or MRI) in these patients to assess for other head and neck pathology and evaluate the extent of the lesion. Investigators have also obtained technetium (Bhattacharya et al., 2009) and radioactive iodine (Britto et
Hamartoma Salivary choristoma Hamartoma
Salivary choristoma
Soybean-size 2.1 3 1.3
2.5 3 1.7 None
99m
18 d Birth M 10 (Britto et al., 2000)
* AP ¼ age at presentation; AS ¼ age at surgery; F ¼ female; ductus arteriosus. † Time of glossoplasty for bifid tongue. ‡ Present case.
Palatal teratoma, VSD VSD, PFO, PDA None
Respiratory difficulty Respiratory and feeding difficulty Respiratory difficulty F F F 7 (Noguchi et al., 2006) 8‡ 9 (Jank et al., 2008)
Birth Birth Birth
Tc ¼ technetium; M ¼ male; CT ¼ computed tomography; MRI ¼ magnetic resonance imaging; VSD ¼ ventricular septal defect; PFO ¼ patent foramen ovale; PDA ¼ patent
Salivary choristoma 232 M 6 (Haghigi et al., 2004)
Birth
20 d, 6 mo† F 5 (Bonet et al., 2012)
Birth
4 wk, 8 wk, 6 mo† 9d 4d 3 mo
Respiratory difficulty, failed oral feeds
Vomer teratoma Nasal dermoid cyst, posterior pharyngeal wall teratoma Dorsal hemivertebra, vitreous hemorrhage Nasopharyngeal teratoma Respiratory difficulty 24 d, 6 mo† 2 d, 6 mo† F M 3 (Bonet et al., 2012) 4 (Bonet et al., 2012)
Birth Birth
None Speech impairment 19 y F 2 (Bhattacharya et al., 2009)
19 y
9 mo F 1 (Bhattacharya et al., 2009)
9 mo
MRI, Iodine-131
Teratoma 232
CT, MRI, radiolabeled iodine scan CT, postoperative MRI MRI MRI
Teratoma Teratoma 1.5 3 2 2.5 3 2
Hamartoma
2.5 3 3 Tc pertechnetate thyroid scan Tc pertechnetate thyroid scan
99m
None Choking with feedings
Associated Malformation Reported Clinical Symptoms AS AP Gender Case Number and Reference
Data of Patients With Clinical Triad of Cleft Palate, Bifid Tongue, and Lingual Mass* TABLE 1
99m
Tongue Mass Size (cm) Imaging
Hamartoma
Cleft Palate–Craniofacial Journal, March 2016, Vol. 53 No. 2
Tongue Mass Histology
248
al., 2000; Haghigi et al., 2004) scans to rule out ectopic thyroid tissue. Congenital lingual masses are a rare clinical entity and may cause significant airway and feeding difficulties. Our patient had significant airway obstruction in the supine position that resolved after a single-stage procedure of mass resection and tongue reconstruction. We advocate for prompt evaluation and treatment of these patients in order to provide a safe airway and allow for early oral feeding. CONCLUSION Our patient presented at birth with a symptomatic lingual salivary choristoma and concomitant bifid tongue and cleft palate. These were isolated findings with no associated genetic abnormality. Immediate resection and reconstruction resulted in an excellent outcome. This is an unusual case, and salivary choristoma should be in the differential diagnosis when evaluating patients with cleft palate, bifid tongue, and a midline lingual mass. REFERENCES Bhattacharya V, Khanna S, Bashir SA, Kumar U, Garbyal RS. Cleft palate associated with hamartomatous bifid tongue. Report of two cases. J Plast Reconstr Aesthet Surg. 2009;62:1442–1445. Bonet C, Penarrocha-Oltra D, Minguez JM, Vera-Sirera B, Penarrocha-Diago M, Penarrocha-Diago M. Oral teratomas: a report of 5 cases. J Oral Max Surg. 2012;70:2809–2813. Britto JA, Ragoowansi RH, Sommerlad BC. Double tongue, intraoral anomalies, and cleft palate—case reports and a discussion of developmental pathology. Cleft Palate Craniofac J. 2000;37:410–417. Guerrissi JO. Cervical tumor by ectopic salivary gland. J Craniofac Surg. 2000;11:394–397. Lee DK, Kim JH, Cho Y, Chung W, Hong SH. Salivary gland choristoma of the middle ear in an infant: a case report. Int J Pediatr Otorhinolaryngol. 2006;70:167–170. Gurrieri F, Franco B, Toriello H, Neri G. Oral–facial–digital syndromes: review and diagnostic guidelines. Am J Med Genet Part A. 2007;143A:3314–3323. Haghigi K, Milles M, Cleveland D, Ziccardi V. Epignathus teratoma with bifid tongue and median glossal salivary mass: report of a case. J Oral Maxillofac Surg. 2004;62:379–383. Jank S, Kelderer HW, Raubenheimer EJ, Puelacher W. Medial tongue cleft associated with intraoral hamartoma—case report and review of the literature. Int J Oral Maxillofac Surg. 2008;37:296–299. ´ ´ ´ız M, PortillaLedesma-Montes C, Ferna´ndez-Lopez R, Garces-Ort Robertson J, Herna´ndez-Guerrero JC. Gingival salivary gland choristoma. A case report. J Periodontol. 1998;69:1164–1166. Noguchi T, Jinbu Y, Itoh H, Matsumoto K, Sakai O, Kusama M. Epignathus combined with cleft palate, lobulated tongue, and lingual hamartoma: report of a case. Oral Pathol Oral Radiol Endod. 2006;101:481–486. Vandenhaute B, Leteurtre E, Lecomte-Houcke M, Pellerin P, Nuyts JP, Cuisset JM, Soto-Ares G. Epignathus teratoma: report of three cases with a review of the literature. Cleft Palate Craniofac J. 2000;37:83–91. Widgerow AD. Klippel-Feil anomaly, cleft palate, and bifid tongue. Ann Plast Surg. 1990;25:216–22. Wise JB, Sehgal K, Guttenberg M, Shah UK. Ectopic salivary tissue of the tonsil: a case report. Int J Pediatr Otorhinolaryngol. 2005;69:567–571.
