Volume 4, Issue 1 , Pages 17-20, January 2009
Colonic duplication in the submandibular region of the neck
Article Outline
Summary
We present a case of an unusual colonic duplication cyst which was located in the submandibular region of the neck. To our knowledge, this is the first reported case of a submandibular cyst lined with colonic mucosa. This type of cystic mass is felt to be due to either displacement of primitive intestinal epithelium or abnormal differentiation of primitive endodermal cells into colonic mucosa in an abnormal location. Diagnosis was made with a combination of CT scan, ultrasound, and ultimately surgical pathology. Treatment is with surgical excision, and the prognosis is excellent with complete excision.
Keywords: Pediatric neck mass, Gastrointestinal duplication cyst
1. Introduction
Gastrointestinal duplication cysts of the head and neck are rare. These are benign cystic structures lined by intestinal epithelium that may be gastric or intestinal in appearance and also may include squamous or respiratory epithelium [1], [2], [3]. There is commonly a thin layer of smooth muscle surrounding the cyst [1], [2], [3], [4]. Gastrointestinal duplication cysts may occur anywhere along the alimentary tract, from mouth to anus. Many other terms exist to describe similar findings including gastric mucosa choristomas, choriostomatic cysts, and heterotopic gastrointestinal cyst of the oral cavity [2]. The majority of these cysts are discovered prior to 2 years of age with a significant portion being discovered during infancy [1], [2]. The location and size of the cyst often determines whether the patient will experience any difficulty with feeding, breathing, or inability to close the mouth. Approximately 30% of reported cases have been symptomatic upon diagnosis [1], [2].
2. Case report
A 5-year-old boy was referred to the Pediatric Otolaryngology service with a 7-month history of a left sided submandibular mass, which had initially begun after a viral infection. He was observed for about 2 months prior to referral. The child was healthy otherwise and had not been experiencing any dysphagia, dyspnea, or fevers. Past surgical history was significant for bilateral myringotomy with tube placement and adenotonsillectomy performed 2 years prior. Physical examination was essentially normal except for a palpable left level I and II neck mass measuring approximately 2
cm×3cm. The mass was somewhat firm and not easily compressible. An ultrasound was performed in the office that revealed a heterogeneous cystic mass with a few areas of increased density within (Fig. 1). A CT scan of the neck with contrast revealed a 1.3cm×2.9cm left submandibular mass with a few punctate calcifications along the anterior border (Fig. 2). The mass was felt to be most likely representative of a dermoid cyst and therefore the patient was taken to operating room for surgical excision after informed consent had been obtained.
Fig. 1.
Ultrasound of the left submandibular mass displays heterogeneous cystic structure deep to the platysma.
Fig. 2.
Axial CT image of the neck displays a left sided cystic submandibular mass with small calcifications seen anteriorly, at the deep margin of the submandibular gland and lateral to the mylohoid muscle.
The operation was performed with no complications. Intraoperative findings were that of a left cystic mass adherent to the left submandibular gland and left hypoglossal nerve. The mass was removed carefully without any damage to surrounding neurovascular structures. The specimen was then sent to pathology in formalin, and the patient was sent home the same day. The specimen was measured by pathology to be 2.7cm×2.5cm×1.4cm. When bisected, there was thick, mucoid material noted within a thin-walled capsule. The cyst wall demonstrated all layers of colonic mucosa, from the mucous-secreting epithelium to the variably developed muscularis propria. Ganglion cells of both the Meissner's and Auerbach's plexi were present (Fig. 3). The diagnosis of gastrointestinal duplication cyst with colonic mucosa was made. At his follow up appointment, the patient was doing well and did not have any postoperative complications or recurrence.
Fig. 3.
The cyst wall demonstrates all layers of colonic mucosa, from the mucous-secreting epithelium to the variably developed muscularis propria. Ganglion cells of both the Meissner's and Auerbach's plexi are present (H&E, 100×). Inset: ganglion cells of the Meissner's plexus (H&E, 400×).
3. Discussion
Gastrointestinal duplication cysts of the head and neck region most frequently involve the tongue and floor of mouth, with approximately 60% of reported cases being found within some portion of the tongue [1], [2], [3], [5]. The incidence has been reported to be higher in males with a ratio of approximately 3:1. There have only been around 40 reported cases of head and neck gastrointestinal duplication cysts, though some have stated that they feel these lesions are more common than the literature would indicate [6]. Of the known cases, the large majority have been lined by gastric mucosa and found within the tongue or floor of mouth near the midline [1]. To our knowledge, there have only been a few reported cases of gastric duplication cysts in the submandibular region, none of which were lined with colonic type mucosa. Other causes of cystic structures in these regions would include dermoid cysts, lymphatic malformations, thyroglossal ducts cysts, plunging ranulas, and branchial cleft cysts among others [2], [7].
Histopathologically, these cysts are characterized by gastric or other intestinal mucosa either alone or in combination with other types of epithelium, such as squamous or respiratory type. The most prevalent type of epithelium has been reported to be gastric, followed by gastric and squamous, intestinal, and mixture of gastric and intestinal [1], [2]. There is usually a serosal layer and a thin layer of muscle tissue surrounding enteric duplication cysts [8]. Alternatively, choristomas are frequently used synonymously with gastrointestinal duplication cysts of the oral cavity, however, these lesions are defined as a mass of tissue that is histologically typical of an organ or part of the body remote from the site where it is normally found [4]. These lesions may consist of neural tissue, cartilaginous tissue, and intestinal tissue among others.
There are numerous theories as to how duplication cysts arise in the head and neck. Some authors have postulated that gastric remnants get trapped within the tongues lateral swellings in the midline at the region of the tuberculum impar around the 4th week of embryogenesis [9]. This theory would more likely explain the midline cysts involving the tongue lined with gastric muscosa, but are less likely to explain the colonic lined submandibular type. Another theory proposed by Daley et al. is that the lesions could develop from nests of endodermal cells that lined the primitive stomodeum and became trapped during the fusion of embryonic processes [10]. Woolgar and Smith stated that undifferentiated endodermal cells could give rise to these cysts, with different factors affecting the differentiation into different types of muscosal lining [11]. This view appears to be supported by histochemical studies that have been done indicating the presence of lectin binding pattern to mucin in the cysts that is different that would be expected in normal gastrointestinal epithelium [1].
While colonic cysts are rare in the head and neck region, they do tend to occur more commonly in other parts of the body. Gastrointestinal duplications are most commonly found in the region of the ileum (60%) and esophagus [12]. Colonic duplications account for approximately 4–18% of those reported [12]. When they occur, they most likely involve the cecum and may present with signs of obstruction, bleeding, pain, or constipation [8]. The cystic form of colonic duplications are more common that tubular form and are thought to commonly arise from the mesenteric border of the bowel wall [8]. Treatment for abdominal colonic cysts is surgical excision when the patient becomes symptomatic. They are usually able to be fully excised and do not tend to recur.
In conclusion, colonic duplication cysts are rare lesions found in the head and neck that may mimic other cystic pathology such as dermoid cysts or lymphatic malformations. Colonic duplication cysts are part of a family of enteric cysts that more commonly involve the tongue or floor of mouth. These are benign lesions that may cause significant concern and symptoms to involved patients and their families. While they are rare, they must be considered in the differential diagnosis of cystic lesions of the oral cavity and neck.
References
- . Congenital heterotopic gastrointestinal cyst of the oral cavity in a neonate: case report and review of literature. Int. J. Pediatr. Otorhinolaryngol. 1996;36(1 (June)):69–77
- . Heterotopic oral gastrointestinal cyst: report of 2 cases and review of the literature. Oral Surg. Oral Med. Oral Pathol. Oral Radiol. Endod. 1999;88(1 (July)):80–86
- . Gastric heterotopia in the nasopharynx. Int. J. Pediatr. Otorhinolaryngol. 2002;64(1 (May 31)):65–67
- . Gastric mucosal choristoma of the tongue and floor of the mouth. J. Oral Maxillofac. Surg. 2001;59(2 (February)):210–212
- . Heterotopic gastrointestinal cyst partially lined with dermoid cyst epithelium. Oral Surg. Oral Med. Oral Pathol. Oral Radiol. Endod. 2001;91(6 (June)):686–688
- . Heterotopic gastric mucosa of the oral cavity: a rare entity. Int. J. Pediatr. Otorhinolaryngol. 2002;66(2 (November 11)):139–142
- . Gastric choristoma of the midline neck in a newborn: a case report and review of the literature. J. Pediatr. Surg. 2003;38(7 (July)):E1–3
- . Colonic duplication in adults: report of two cases presenting with rectal bleeding. World J. Gastroenterol. 2005;11:5072–5074
- . Occurence of heterotopic gastric mucosa in the tongue. J. Pediatr. 1964;64:604–606
- . Hetertopic gastric cyst of the oral cavity. Head Neck Surg. 1984;7:168–171
- . Heterotopic gastrointestinal cyst of oral cavity; developmental lesion?. Oral Surg. Oral Med. Oral Pathol. 1988;66:223–225
- . Gastrointestinal duplications. J. Pediatr. Surg. 2003;38:740–744
PII: S1871-4048(08)00033-6
doi:10.1016/j.pedex.2008.05.001
© 2008 Elsevier Ireland Ltd. All rights reserved.
Volume 4, Issue 1 , Pages 17-20, January 2009
