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Congenital lingual cysts
Journal of Pediatric Surgery (2007) 42, E25 – E27
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Congenital lingual cysts Oliver Karama,*, Riccardo E. Pfistera, Philippe Extermannb, Giorgio C. La Scalac a
Department of Paediatrics, Neonatology and Pediatric Intensive Care Service, University of Geneva’s Hospital, 1211 Geneva, Switzerland b Dianecho Ultrasound Centre, 1205 Geneva, Switzerland c Department of Paediatrics, Pediatric Surgery Service, University of Geneva’s Hospital, 1211 Geneva, Switzerland Index words: Infant; Newborn; Tongue; Tongue/surgery; Mucocele; Mucocele/surgery; Operative surgical procedure; Prenatal diagnosis; Ultrasonography; Prenatal
Abstract Congenital lingual cysts are rare congenital tumors of the oropharynx, which should be suspected when the tongue appears enlarged by a mass. Prenatal diagnosis by ultrasound allows for an optimal management at the time of delivery because the mass can obstruct the airways. Unless the cyst enlarges and hinders feeding or breathing, surgical treatment is indicated only a few months after birth because spontaneous regression can sometimes be observed. D 2007 Elsevier Inc. All rights reserved.
Oral and upper cervical tumors are quite uncommon in the perinatal period, yet they may have severe consequences after birth. Although most of these lesions are benign, their localization can have significant repercussions. Large masses that hinder respiration at birth are usually announced by the presence of a polyhydramnios on prenatal ultrasound and generally require an immediate neonatal attention. However, masses may continue to grow after birth and subsequently lead to severe respiratory obstruction, particularly with the occurrence of upper airway infections. Smaller tumors may not produce airway obstruction but can still interfere with feeding.
1. Case presentation We present the case of a male infant born by vaginal delivery at 39 weeks of gestational age. At 20 weeks of * Corresponding author. Tel.: +41 22 382 3311; fax: +41 22 382 4624. E-mail address: [email protected] (O. Karam). 0022-3468/$ – see front matter D 2007 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2007.01.060
gestational age, lingual cysts were discovered on a routine morphological ultrasound. These masses were followed by ultrasound (Fig. 1A), and their size increased during the last trimester of pregnancy, growing to up to 9 mm in diameter at 36 weeks (Fig. 1B). The mother’s medical and obstetric history was unremarkable. There was no family history of any congenital anomalies. At birth, adaptation was excellent, Apgar scores were 9, 10, and 10 at 1, 5, and 10 minutes; cord pH was 7.26/7.41. On clinical examination, the infant had a protruding tongue with 2 bluish cysts bulging on its inferior aspect (Fig. 1C). The patient was admitted to the special care nursery for respiratory monitoring. Ultrasound imaging confirmed the presence of 2 hypoechoic cysts having a maximum diameter of 12 and 16 mm, respectively, possibly communicating. There was no evidence of an underlying vascular malformation or hemangioma. The patient did not have breathing difficulties and was able to bottle feed. However, breast-feeding could not be achieved because the child was unable to latch sufficiently on the nipple.
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O. Karam et al.
Fig. 1 A, Antenatal ultrasound scan at 22 weeks of gestational age (arrows point to the cysts). B, 3-Dimensional ultrasound scan at 36 weeks of gestational age (arrow points to the protruding cysts). C, Initial clinical appearance of the cysts protruding out of the mouth. D, Appearance at operation on the inferior aspect of the tongue.
Surgery was not initially planned for the neonatal period; however, on the request of the mother of the patient, who wished to breast-feed, the patient was scheduled for surgical resection of the cysts (Fig. 1D) on day 3 of life. The histologic analysis confirmed communicating cysts of 20 10 6 mm (Fig. 2A) covered by a mixed pattern of epithelium. Some sections were covered by a transitional epithelium (Fig. 2B), whereas others had a pseudostratified, ciliated epithelium or a cylindrical epithelium (Fig. 2C); 2 islets of accessory salivary glands were found in the cyst wall. Postoperatively, dexamethasone was administered for 48 hours to prevent local swelling. The hospital course was then uneventful, and the child was discharged home 5 days after surgery fully breast-fed. At follow-up appointments in the pediatric surgery clinic at 4 weeks and 6 months, the child was healthy, showed a normal-sized tongue, and had no feeding difficulties.
2. Discussion Congenital lingual cysts are an uncommon anomaly seen in newborns. Their prevalence is still unknown, and only
small series have been published so far. A congenital sublingual cyst is often secondary to a dilatation of a minor salivary gland on the inferior aspect of the tongue, or it can occur because of a dilatation of the actual sublingual gland, which is localized anteriorly on the mouth’s floor between the mylohyoideus and hyoglossus muscles [1,2]. Clinically, a fluid-filled cyst elevates the tongue or the mouth’s floor; if sublingual in origin, this cyst may plunge under the mylohyoideus muscle with a cervical extension, producing also a cervical mass, which can mimic a lymphangioma. Those cysts may go unnoticed at birth but can progressively grow to represent a real threat to the airway causing stridor and dyspnoea [3]. When small at birth, they are often diagnosed in late childhood when big enough to be noticed; reports indicate that they may also be acquired and occur after a tongue trauma [2,4,5]. The differential diagnosis of lingual and sublingual cysts includes lymphatic malformations (solid-cystic masses with indistinct margins on ultrasound); vascular malformations and hemangiomas; fetal cervical tumors such as teratomas (solid-cystic tumors with mixed hypoechoic and hyperechoic areas on ultrasound examination, often associated with polyhydramnios), thymomas, and thyroid tumors;
Congenital lingual cysts
E27 postnatal ultrasound determined the exact anatomy of the cyst and confirmed its polycystic nature. The microscopic examination of the cyst in the reported case revealed an epithelium-lined cyst; usually, congenital sublingual cysts are epithelium-free, and they develop from a mucus leak after disruption of a salivary gland and are therefore considered pseudocysts. However, 7.5% of sublingual cysts are mucosal retention cysts covered with epithelium [6], and the glands involved may then frequently display metaplasia of the epithelial lining. Because the histologic analysis of our patient’s cyst demonstrated 3 different types of epithelia, the final diagnosis was a benign epithelial cyst with metaplasia of the epithelial lining. This finding is in keeping with a foregut duplication cyst, which are described to contain both respiratory and gastrointestinal epithelium [7,8]. Different techniques are described to treat lingual cysts. As mentioned, spontaneous resolution has been sometimes observed [5], but usually, a surgical approach is recommended [4,5,9,10]. Urgent surgical treatment is only recommended when the cyst hinders feeding or breathing, to allow for potential spontaneous regression of the cyst. After an observation period of a few months however, in case of persistence of the cyst, excision is suggested. This can involve marsupialization or unroofing of the cyst, simple excision of the cyst, or excision combined with removal of the ipsilateral sublingual gland in case of a cyst extending to the floor of the mouth. The recurrence rate in epithelium-free cyst seems to be lower after excision including the ipsilateral sublingual gland [10].
References
Fig. 2 A, Macroscopic aspect of the excised specimen showing 2 communicating cysts. B, Passage from a transitional epithelium to a cylindrical epithelium. C, Transition from a pseudostratified, ciliated epithelium to a cylindrical epithelium.
ectopic thyroglossal duct cyst (although quite unusual in the floor of the oral cavity); foregut duplication cysts such as heterotopic gastric cysts and enterocystomas. An ultrasound will differentiate between solid and liquid tumors; a more complete investigation of the nature and anatomical relationships of the mass can be obtained by magnetic resonance imaging even in utero. In our case, the
[1] Fernandez Moya JM, Cifuentes Sulzberger S, Diaz Recasens J, et al. Antenatal diagnosis and management of a ranula. Ultrasound Obstet Gynecol 1998;11:147 - 8. [2] Ugboko VI, Hassan O, Prasad S, et al. Congenital ranula. A report of two cases. ORL J Otorhinolaryngol Relat Spec 2002;64:294 - 6. [3] Kaneko K, Takahashi K, Unno A, et al. Lingual cyst in infancy: importance of palpation for diagnosis. Acta Paediatr Jpn 1997;39:475 - 7. [4] Morton RP, Bartley JR. Simple sublingual ranulas: pathogenesis and management. J Otolaryngol 1995;24:253 - 4. [5] Pandit RT, Park AH. Management of pediatric ranula. Otolaryngol Head Neck Surg 2002;127:115 - 8. [6] Oliveira DT, Consolaro A, Freitas FJ. Histopathological spectrum of 112 cases of mucocele. Braz Dent J 1993;4:29 - 36. [7] Eaton D, Billings K, Timmons C, et al. Congenital foregut duplication cysts of the anterior tongue. Arch Otolaryngol Head Neck Surg 2001; 127:1484 - 7. [8] Ohbayashi Y, Miyake M, Nagahata S. Gastrointestinal cyst of the tongue: a possible duplication cyst of foregut origin. J Oral Maxillofac Surg 1997;55:626 - 8 [discussion 629-30]. [9] Burkart CM, Brinkman JA, Willging JP, et al. Lingual cyst lined by squamous epithelium. Int J Pediatr Otorhinolaryngol 2005;69: 1649 - 53. [10] Yoshimura Y, Obara S, Kondoh T, et al. A comparison of three methods used for treatment of ranula. J Oral Maxillofac Surg 1995;53:280 - 2.
www.elsevier.com/locate/jpedsurg
Congenital lingual cysts Oliver Karama,*, Riccardo E. Pfistera, Philippe Extermannb, Giorgio C. La Scalac a
Department of Paediatrics, Neonatology and Pediatric Intensive Care Service, University of Geneva’s Hospital, 1211 Geneva, Switzerland b Dianecho Ultrasound Centre, 1205 Geneva, Switzerland c Department of Paediatrics, Pediatric Surgery Service, University of Geneva’s Hospital, 1211 Geneva, Switzerland Index words: Infant; Newborn; Tongue; Tongue/surgery; Mucocele; Mucocele/surgery; Operative surgical procedure; Prenatal diagnosis; Ultrasonography; Prenatal
Abstract Congenital lingual cysts are rare congenital tumors of the oropharynx, which should be suspected when the tongue appears enlarged by a mass. Prenatal diagnosis by ultrasound allows for an optimal management at the time of delivery because the mass can obstruct the airways. Unless the cyst enlarges and hinders feeding or breathing, surgical treatment is indicated only a few months after birth because spontaneous regression can sometimes be observed. D 2007 Elsevier Inc. All rights reserved.
Oral and upper cervical tumors are quite uncommon in the perinatal period, yet they may have severe consequences after birth. Although most of these lesions are benign, their localization can have significant repercussions. Large masses that hinder respiration at birth are usually announced by the presence of a polyhydramnios on prenatal ultrasound and generally require an immediate neonatal attention. However, masses may continue to grow after birth and subsequently lead to severe respiratory obstruction, particularly with the occurrence of upper airway infections. Smaller tumors may not produce airway obstruction but can still interfere with feeding.
1. Case presentation We present the case of a male infant born by vaginal delivery at 39 weeks of gestational age. At 20 weeks of * Corresponding author. Tel.: +41 22 382 3311; fax: +41 22 382 4624. E-mail address: [email protected] (O. Karam). 0022-3468/$ – see front matter D 2007 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2007.01.060
gestational age, lingual cysts were discovered on a routine morphological ultrasound. These masses were followed by ultrasound (Fig. 1A), and their size increased during the last trimester of pregnancy, growing to up to 9 mm in diameter at 36 weeks (Fig. 1B). The mother’s medical and obstetric history was unremarkable. There was no family history of any congenital anomalies. At birth, adaptation was excellent, Apgar scores were 9, 10, and 10 at 1, 5, and 10 minutes; cord pH was 7.26/7.41. On clinical examination, the infant had a protruding tongue with 2 bluish cysts bulging on its inferior aspect (Fig. 1C). The patient was admitted to the special care nursery for respiratory monitoring. Ultrasound imaging confirmed the presence of 2 hypoechoic cysts having a maximum diameter of 12 and 16 mm, respectively, possibly communicating. There was no evidence of an underlying vascular malformation or hemangioma. The patient did not have breathing difficulties and was able to bottle feed. However, breast-feeding could not be achieved because the child was unable to latch sufficiently on the nipple.
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O. Karam et al.
Fig. 1 A, Antenatal ultrasound scan at 22 weeks of gestational age (arrows point to the cysts). B, 3-Dimensional ultrasound scan at 36 weeks of gestational age (arrow points to the protruding cysts). C, Initial clinical appearance of the cysts protruding out of the mouth. D, Appearance at operation on the inferior aspect of the tongue.
Surgery was not initially planned for the neonatal period; however, on the request of the mother of the patient, who wished to breast-feed, the patient was scheduled for surgical resection of the cysts (Fig. 1D) on day 3 of life. The histologic analysis confirmed communicating cysts of 20 10 6 mm (Fig. 2A) covered by a mixed pattern of epithelium. Some sections were covered by a transitional epithelium (Fig. 2B), whereas others had a pseudostratified, ciliated epithelium or a cylindrical epithelium (Fig. 2C); 2 islets of accessory salivary glands were found in the cyst wall. Postoperatively, dexamethasone was administered for 48 hours to prevent local swelling. The hospital course was then uneventful, and the child was discharged home 5 days after surgery fully breast-fed. At follow-up appointments in the pediatric surgery clinic at 4 weeks and 6 months, the child was healthy, showed a normal-sized tongue, and had no feeding difficulties.
2. Discussion Congenital lingual cysts are an uncommon anomaly seen in newborns. Their prevalence is still unknown, and only
small series have been published so far. A congenital sublingual cyst is often secondary to a dilatation of a minor salivary gland on the inferior aspect of the tongue, or it can occur because of a dilatation of the actual sublingual gland, which is localized anteriorly on the mouth’s floor between the mylohyoideus and hyoglossus muscles [1,2]. Clinically, a fluid-filled cyst elevates the tongue or the mouth’s floor; if sublingual in origin, this cyst may plunge under the mylohyoideus muscle with a cervical extension, producing also a cervical mass, which can mimic a lymphangioma. Those cysts may go unnoticed at birth but can progressively grow to represent a real threat to the airway causing stridor and dyspnoea [3]. When small at birth, they are often diagnosed in late childhood when big enough to be noticed; reports indicate that they may also be acquired and occur after a tongue trauma [2,4,5]. The differential diagnosis of lingual and sublingual cysts includes lymphatic malformations (solid-cystic masses with indistinct margins on ultrasound); vascular malformations and hemangiomas; fetal cervical tumors such as teratomas (solid-cystic tumors with mixed hypoechoic and hyperechoic areas on ultrasound examination, often associated with polyhydramnios), thymomas, and thyroid tumors;
Congenital lingual cysts
E27 postnatal ultrasound determined the exact anatomy of the cyst and confirmed its polycystic nature. The microscopic examination of the cyst in the reported case revealed an epithelium-lined cyst; usually, congenital sublingual cysts are epithelium-free, and they develop from a mucus leak after disruption of a salivary gland and are therefore considered pseudocysts. However, 7.5% of sublingual cysts are mucosal retention cysts covered with epithelium [6], and the glands involved may then frequently display metaplasia of the epithelial lining. Because the histologic analysis of our patient’s cyst demonstrated 3 different types of epithelia, the final diagnosis was a benign epithelial cyst with metaplasia of the epithelial lining. This finding is in keeping with a foregut duplication cyst, which are described to contain both respiratory and gastrointestinal epithelium [7,8]. Different techniques are described to treat lingual cysts. As mentioned, spontaneous resolution has been sometimes observed [5], but usually, a surgical approach is recommended [4,5,9,10]. Urgent surgical treatment is only recommended when the cyst hinders feeding or breathing, to allow for potential spontaneous regression of the cyst. After an observation period of a few months however, in case of persistence of the cyst, excision is suggested. This can involve marsupialization or unroofing of the cyst, simple excision of the cyst, or excision combined with removal of the ipsilateral sublingual gland in case of a cyst extending to the floor of the mouth. The recurrence rate in epithelium-free cyst seems to be lower after excision including the ipsilateral sublingual gland [10].
References
Fig. 2 A, Macroscopic aspect of the excised specimen showing 2 communicating cysts. B, Passage from a transitional epithelium to a cylindrical epithelium. C, Transition from a pseudostratified, ciliated epithelium to a cylindrical epithelium.
ectopic thyroglossal duct cyst (although quite unusual in the floor of the oral cavity); foregut duplication cysts such as heterotopic gastric cysts and enterocystomas. An ultrasound will differentiate between solid and liquid tumors; a more complete investigation of the nature and anatomical relationships of the mass can be obtained by magnetic resonance imaging even in utero. In our case, the
[1] Fernandez Moya JM, Cifuentes Sulzberger S, Diaz Recasens J, et al. Antenatal diagnosis and management of a ranula. Ultrasound Obstet Gynecol 1998;11:147 - 8. [2] Ugboko VI, Hassan O, Prasad S, et al. Congenital ranula. A report of two cases. ORL J Otorhinolaryngol Relat Spec 2002;64:294 - 6. [3] Kaneko K, Takahashi K, Unno A, et al. Lingual cyst in infancy: importance of palpation for diagnosis. Acta Paediatr Jpn 1997;39:475 - 7. [4] Morton RP, Bartley JR. Simple sublingual ranulas: pathogenesis and management. J Otolaryngol 1995;24:253 - 4. [5] Pandit RT, Park AH. Management of pediatric ranula. Otolaryngol Head Neck Surg 2002;127:115 - 8. [6] Oliveira DT, Consolaro A, Freitas FJ. Histopathological spectrum of 112 cases of mucocele. Braz Dent J 1993;4:29 - 36. [7] Eaton D, Billings K, Timmons C, et al. Congenital foregut duplication cysts of the anterior tongue. Arch Otolaryngol Head Neck Surg 2001; 127:1484 - 7. [8] Ohbayashi Y, Miyake M, Nagahata S. Gastrointestinal cyst of the tongue: a possible duplication cyst of foregut origin. J Oral Maxillofac Surg 1997;55:626 - 8 [discussion 629-30]. [9] Burkart CM, Brinkman JA, Willging JP, et al. Lingual cyst lined by squamous epithelium. Int J Pediatr Otorhinolaryngol 2005;69: 1649 - 53. [10] Yoshimura Y, Obara S, Kondoh T, et al. A comparison of three methods used for treatment of ranula. J Oral Maxillofac Surg 1995;53:280 - 2.