Intralingual foregut duplication cyst in a newborn. (Original Article).

Abstract

Congenital lingual cystic masses are challenging entities that can be detected prenatally or discovered in various forms after birth. We report what we believe is only the ninth case of an intralingual fore gut duplication cyst that was lined with gastric mucosa. The preoperative evaluation of lingual cystic masses in newborns can include palpation, high-resolution sonography, computed tomography (CT), or magnetic resonance imaging (MRI). However, CT and MRI can pose a risk to the infant because they require sedation in order to properly position the patient for imaging. In our patient, we found that high-resolution sonography was most useful in revealing the nature of the mass as a nonvascular cystic lesion and in delineating its extension. We excised this lesion via bipolar cautery, which we prefer to cold-knife or [CO.sub.2] laser surgery.

Introduction

A lingual cystic mass in a newborn can be an interesting challenge for the otolaryngologist. Although most cases are brought to the otolaryngologist' s attention following birth, some are detected prenatally. Some of these lesions require emergency intervention. (1,2) Among the different lesions that can arise in the tongue, the foregut duplication cyst is of special interest because of its rarity, variable characteristics, and its puzzling embryogenesis. Until now, only eight cases of intralingual foregut duplication cysts lined with gastric mucosa have been reported in the literature (table). (3-9) In this article, we report a new case--one that illustrates the usefulness of noninvasive preoperative diagnostic tools and the advantages of bipolar cautery in excising this lesion.

Case report

A 6-day-old boy was brought to our clinic with an enlarged tongue, which had first been noticed at birth. He had no feeding or breathing difficulties or other associated abnormalities. On examination, the anterior two-thirds of his tongue was diffusely enlarged, and a bulge was noted on its ventral aspect. On palpation, a soft, nonpulsatile, and slightly compressible mass was felt in the body of the tongue. High-resolution sonography detected a 2.7 x 1.8-cm cystic mass confined to the tongue body; there was no extension into the floor of the mouth. Color Doppler ultrasonography detected no blood flow in the mass. At that point, we decided to take a wait-and-see approach.

The patient was brought back to our clinic at the age of 2 months because the size of the mass had increased and he began exhibiting difficulty feeding. The cyst was excised via a midline vertical incision at the ventral aspect of the tongue; both Wharton's ducts were preserved. The cyst wall was thick and adherent to the lingual muscles. Bipolar cautery was used throughout the dissection, and it resulted in a bloodless field and excellent visualization of the surrounding structures. The lesion extended to the base of the tongue, where it was attached to a stump-like structure; this structure was excised along with the cyst. The excised mass measured 3 x 1 x 1.2 cm. The wound was closed in layers, and the patient was extubated uneventfully. He did well overnight, and he was fed the next morning through a syringe. His postoperative period passed without difficulty.

On pathologic examination, the sectioned mass was found to contain a central tubular lesion 0.3 cm in diameter and 2 cm in length. Microscopic analysis revealed that patchy lingual skeletal muscle had surrounded the tubular structure. The structure's appearance was consistent with that of a segment of the gastrointestinal wall. The structure featured an outer smooth-muscle layer (muscularis propria, with circular and longitudinal layers), submucosa, and areas of mucosal lining that contained pits and glands and were consistent with gastric mucosa. Other areas of the mucosa were of the simple columnar type and focally denuded. An area of hemosiderin-laden macrophages indicated that there had been a previous hemorrhage (figure).

The patient's wound healed nicely, with no scarring and with normal tongue mobility. He resumed normal feeding, and at the 1-year follow-up, he exhibited no evidence of recurrence.

Discussion

A cystic mass in the anterior two-thirds of the tongue in a newborn can represent a dermoid cyst, an epidermoid cyst, a lymphangioma, a hemangioma, or a ranula. (4,10,11) Since first reported by Foderl (9) in 1895, cysts lined with gastric and/or intestinal mucosa have rarely been seen in the tongue or the floor of the mouth. These cysts have usually been reported along the alimentary tract, from the esophagus to the colon, (10'12) and in the gallbladder, pancreas, lungs, larynx, and urinary bladder. (13)

Congenital gastric and intestinal cysts of the oral cavity are more common in boys than in girls. (2,8,9,14) These masses involve the anterior aspect of the tongue in 60% of cases. (9,10) Some are asymptomatic, and some cause various degrees of feeding and breathing difficulties or manifest in unexpected ways, such as recurrent bleeding (13) or a brownish discharge from a lingual sinus. (10) Most of these cysts are solitary, but in some cases more than one cyst has been present. (14)

This lesion has been given several names, including cystic choristoma, heterotopic gastrointestinal cyst, enterocystoma, and duplication cyst. (9) The different terminology has led to some confusion regarding the nature of this lesion. Some of this uncertainty was resolved by Rickham et al, who established diagnostic criteria for duplication cysts. (15) These criteria include (1) the presence of a smooth-muscle coat, (2) an attachment of the cyst to a part of the alimentary tract, and (3) the presence of a mucosal lining from the alimentary tract. (4,7,8,11,15)

With these criteria in mind, we conducted an extensive search of the English-language literature to identify reports of lingual duplication cysts that were lined with gastric mucosa. We found that only eight such cases have been reported during the past 100 years. (3-9) The first was published by Duncan and Daniel in 1942. (3)

Lingual foregut duplication cysts are believed to arise from endodermal cells that become trapped during the fusion of the lateral lingual swelling (distal tongue bud) and the tuberculum impar (median tongue bud) in the 3- to 4-mm embryo. (2,10,14) These cells are believed to derive from either the stomodeum or the stomach, which is located in the neck close to the forming tongue at this stage of development. (16) These pluripotential cells can differentiate into various types of epithelium, includeing gastric, intestinal, colonic, and even respiratory.

In addition to a clinical examination, (10,13) the initial evaluation of a newborn with a lingual mass can include ultrasonography, computed tomography (CT), and/or magnetic resonance imaging (MRI). (8,9) Clinical examination alone cannot differentiate among the wide variety of possible lesions. Moreover, CT and MRI can be risky in some neonates (especially those who have large masses) because of the body positioning involved in these procedures and the need for sedation. (2)

In our patient, we found that high-resolution sonography was most useful in revealing the nature of the mass as a nonvascular cystic lesion and in delineating its extension. Thomas et al were the first to report the use of ultrasonography in assessing lesions in the oral cavity. (17) Ultrasound is inexpensive and readily available, and it can differentiate among solid, cystic, and vascular lesions, especially color Doppler ultrasound. Moreover, ultrasonography can detect these lesions prenatally, thereby allowing for adequate preparations to be made before delivery to secure the newborn's airway. (1)

Excising an intralingual cyst whose wall adheres to the surrounding muscles is a tedious exercise, especially in an infant with a large cyst. Conventional techniques, including sharp and blunt dissection, are associated with bleeding, limited visualization of nearby vessels and nerves, and substantial postoperative edema. The [CO.sub.2] laser provides for good hemostasis and better identification of surrounding normal anatomy, and it results in minimal tissue damage, less postoperative swelling, and less scarring. (18) However, it carries potential serious risks, which have been well documented in the literature. Therefore, we prefer bipolar cautery because it is a safer and less expensive alternative. The surgeon has several options in choosing the shape and direction of the initial surgical incision (e.g., vertical, transverse, or semilunar). We found that the vertical incision on the ventral aspect of the tongue was the most natural approach because it causes less scarring and allows the surgeon to preserv e the normal function of the tongue postoperatively. (1)

In conclusion, an intralingual foregut duplication cyst should be considered in the differential diagnosis of a lingual cystic mass in an infant. The use of ultrasonography as an initial assessment study might be sufficient, Early surgical excision in a symptomatic patient is desirable in order to avoid failure-to-thrive, respiratory distress, or more extensive dissection later if the lesion increases in size.

Table

Review of reported cases of intralingual foregut duplication cysts lined
with gastric mucosa

Case report                        Age/sex

Duncan and Daniel, 1942 (3)        2 mo/M

Lister and Zachary, 1968 (4)       11 mo/M
                                   Newborn/F

Willis, 1968 (5)                   4 mo/M

Harris and Courtemanche, 1974 (6)  6 mo/M

Brown and Kerr-Wilson, 1978 (7)    11 mo/M

Willner et al, 1991 (8)            8 mo/F

Said-Al-Naief et al, 1999 (9)      2 yr/F

El-Bitar et al, 2003 *             2 mo/M

Case report                        Cyst status at excision

Duncan and Daniel, 1942 (3)        Cyst had enlarged

Lister and Zachary, 1968 (4)       Emerging teeth damaged cyst
                                   Cyst caused tongue to protrude

Willis, 1968 (5)                   Not specified

Harris and Courtemanche, 1974 (6)  Cyst caused tongue to protrude

Brown and Kerr-Wilson, 1978 (7)    Cyst had enlarged

Willner et al, 1991 (8)            Cyst was asymptomatic and stable

Said-Al-Naief et al, 1999 (9)      Cyst affected speech and feeding

El-Bitar et al, 2003 *             Cyst affected feeding

* Present case.

References

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(2.) Grime PD. Giant enterocystoma within an infant's tongue. J Laryngol Otol 1990;104:814-18.

(3.) Duncan GW, Daniel RA, Jr. Cystic tumor of the tongue: Report of an unusual case. Arch Surg 1942;44: 164-9.

(4.) Lister J, Zachary RB. Cystic duplications in the tongue. J Pediatr Surg 1968;3:491-3.

(5.) Willis RA. Some unusual developmental heterotopias. Br Med J 1968;3:267-72.

(6.) Harris CN, Courtemanche AD. Gastric mucosal cyst of the tongue: Case report. Plast Reconstr Surg 1974;54:612-13.

(7.) Brown S, Kerr-Wilson R. Intra-oral duplication cyst. J Pediatr Surg 1978;13:95-6.

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(9.) Said-Al-Naief N, Fantasia JE, Sciubba JJ, et al. Heterotopic oral gastrointestinal cyst: Report of 2 cases and review of the literature. Oral Surg Oral Med Oral-Pathol Oral Radial Endod 1999; 88:80-6.

(10.) Lipsett J, Sparnon AL, Byard RW. Embryogenesis of enterocystomas-enteric duplication cysts of the tongue. Oral Surg Oral Med Oral Pathol 1993;75:626-30.

(11.) Mirchandani R, Sciubba J, Gloster ES. Congenital oral cyst with heterotopic gastrointestinal and respiratory mucosa. Arch Pathol Lab Med l989;113:1301-2.

(12.) 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.

(13.) Parikh DH, Ibrahim SK, Cook RC. Peptic ulceration in a lingual sinus. J Pediatr Surg 1991;26:99-100.

(14.) Mir R, Weitz J, Evans J, Coren C. Oral congenital cystic choristomas: A case report. Pediatr Pathol 1992;12:835-8.

(15.) Rickham PP, Lister J, Irving IM, eds. Neonatal Surgery. London: Butterworths, 1978.

(16.) Gorlin RJ, Kalnius V, Izant RJ. Occurrence of heterotopic gastric mucosa in the tongue. J Pediatr 1964;64:604-6.

(17.) Thomas MR. Nofal F, Cave AP. Dermoid cyst in the mouth: Value of ultrasound. J Laryngol Otol 1990;104:141-2.

(18.) Aydogan B, Kiroglu M, Soylu L, et al. Gastric cyst of the oral cavity. Int Pediatr Otorhinolaryngol 1995;45:255-8.

From the Department of Pediatric Otolaryngology-Head and Neck Surgery (Dr. El-Bitar) and the Department of Pathology (Dr. Kumar), Children's National Medical Center, George Washington University School of Medicine, Washington, D.C.; and the Department of Otolaryngology--Head and Neck Surgery, Georgetown University Medical School, Washington, D.C. (Dr. Milmoe).

Reprint requests: Mohamed A. El-Bitar, MD, Department of Otolaryngology--Head and Neck Surgery, American University of Beirut Medical Center, PO Box 11-0236, Beirut, Lebanon 1107-2020. Phone/fax: [phone]; e-mail: [e-mail]