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Radiology Quiz Case 2: Diagnosis

Radiology Quiz Case 2: Diagnosis Diagnosis: Cervical foregut duplication cyst The embyological foregut is the portion of the alimentary canal that extends from the pharynx to the duodeunum and the hepatobiliary tree. Foregut duplications are rare congenital anomalies that can occur at any point along the embyological foregut. They represent about one-third of alimentary tract duplications, with an overall incidence of only 1 in 13 000.1 They are usually asymptomatic cysts and most commonly occur in the abdomen or the thorax. They are rarely identified in the head and neck region. Of those found in the head and neck, most are associated with the tongue.2 To be considered an alimentary tract duplication, the cyst must typically meet 3 criteria: (1) the presence of a well-developed coat of smooth muscle; (2) an epithelial lining of some portion of the alimentary tract; and (3) an attachment to some portion of the gastrointestinal tract.1 The present case describes the findings of a cervical foregut duplication. In this case, there was no gross connection to the pharynx or the esophagus. Histologic sections revealed a cyst wall lined mostly by squamous epithelium, with an underlying histiocytic reaction and a clearly demarcated smooth muscle layer. There was 1 focal area of tall columnar mucinous epithelium with underlying tightly packed fundic glands, consistent with gastric-type epithelium (Figure 2). The presence of the gastric-type epithelium with an underlying distinct smooth muscle layer met the histologic criteria for a diagnosis of foregut duplication cyst and would not be characteristic of other benign cysts of the region, such as branchial cleft cysts. Mucin-secreting epithelial cells account for the radiographic appearance of relatively increased attenuation compared with water that is less commonly seen with many other uncomplicated developmental neck cysts. View LargeDownload Figure 2. While in many cases cervical foregut duplication cysts present as asymptomatic lesions, associated symptoms vary with the location of the cyst and may include breathing or swallowing difficulties. Pathologically, the cysts are benign but have the potential to become infected and swell, which may result in airway compromise or sepsis. Ultrasonography, magnetic resonance imaging, and computed tomography can all be useful in defining the lesion. There have also been reports of ultrasonography being used to detect alimentary tract duplications prenatally.3 Coincidence of a foregut duplication cyst and Horner syndrome has been previously described but is not a common presentation.4 The differential diagnosis of a unilateral cystic neck mass in a child should include branchial cleft cyst, thymic cyst, and thymopharyngeal duct cyst. While a thyroglossal duct cyst is the most common congenital cyst in the pediatric population, it would be an unlikely consideration in this case because the cyst was too large and too far off midline. The final diagnosis is typically only confirmed by examination of histologic sections after excision, as shown in Figure 2, with demonstration of smooth muscle as well as nests of gastric mucosal tissue. Once the cervical foregut duplication cyst is identified, the definitive treatment is surgical excision, as was performed in this case. In all reported cases, to our knowledge, excision has been curative, with no reports of recurrence after excision.5 Although rare, foregut duplication cyst should be included in the differential diagnosis of a cystic neck mass in a pediatric patient. Return to Quiz Case 2. References 1. Qi BQ, Beasley SW, Williams AK. Evidence of a common pathogenesis for foregut duplications and esophageal atresia with tracheo-esophageal fistula. Anat Rec. 2001;264(1):93-10011505375PubMedGoogle ScholarCrossref 2. Edwards J, Pearson S, Zalzal G. Foregut duplication cyst of the hypopharynx. Arch Otolaryngol Head Neck Surg. 2005;131(12):1112-111516365227PubMedGoogle ScholarCrossref 3. el-Bitar MA, Milmoe G, Kumar S. Intralingual foregut duplication cyst in a newborn. Ear Nose Throat J. 2003;82(6):454-45612861873PubMedGoogle Scholar 4. Robinson GC, Dikrainian DA, Roseborough GF. Congenital Horner's syndrome and heterochromia iridum: their association with congenital foregut and vertebral anomalies. Pediatrics. 1965;35:103-10714223208PubMedGoogle Scholar 5. Fraser L, Howatson AG, MacGregor FB. Foregut duplication cyst of the pharynx. J Laryngol Otol. 2008;122(7):754-75618086334PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Otolaryngology - Head & Neck Surgery American Medical Association

Radiology Quiz Case 2: Diagnosis

Archives of Otolaryngology - Head & Neck Surgery , Volume 137 (7) – Jul 18, 2011

Radiology Quiz Case 2: Diagnosis

Abstract

Diagnosis: Cervical foregut duplication cyst The embyological foregut is the portion of the alimentary canal that extends from the pharynx to the duodeunum and the hepatobiliary tree. Foregut duplications are rare congenital anomalies that can occur at any point along the embyological foregut. They represent about one-third of alimentary tract duplications, with an overall incidence of only 1 in 13 000.1 They are usually asymptomatic cysts and most commonly occur in the abdomen or the thorax....
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Publisher
American Medical Association
Copyright
Copyright © 2011 American Medical Association. All Rights Reserved.
ISSN
0886-4470
eISSN
1538-361X
DOI
10.1001/archoto.2011.96-b
Publisher site
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Abstract

Diagnosis: Cervical foregut duplication cyst The embyological foregut is the portion of the alimentary canal that extends from the pharynx to the duodeunum and the hepatobiliary tree. Foregut duplications are rare congenital anomalies that can occur at any point along the embyological foregut. They represent about one-third of alimentary tract duplications, with an overall incidence of only 1 in 13 000.1 They are usually asymptomatic cysts and most commonly occur in the abdomen or the thorax. They are rarely identified in the head and neck region. Of those found in the head and neck, most are associated with the tongue.2 To be considered an alimentary tract duplication, the cyst must typically meet 3 criteria: (1) the presence of a well-developed coat of smooth muscle; (2) an epithelial lining of some portion of the alimentary tract; and (3) an attachment to some portion of the gastrointestinal tract.1 The present case describes the findings of a cervical foregut duplication. In this case, there was no gross connection to the pharynx or the esophagus. Histologic sections revealed a cyst wall lined mostly by squamous epithelium, with an underlying histiocytic reaction and a clearly demarcated smooth muscle layer. There was 1 focal area of tall columnar mucinous epithelium with underlying tightly packed fundic glands, consistent with gastric-type epithelium (Figure 2). The presence of the gastric-type epithelium with an underlying distinct smooth muscle layer met the histologic criteria for a diagnosis of foregut duplication cyst and would not be characteristic of other benign cysts of the region, such as branchial cleft cysts. Mucin-secreting epithelial cells account for the radiographic appearance of relatively increased attenuation compared with water that is less commonly seen with many other uncomplicated developmental neck cysts. View LargeDownload Figure 2. While in many cases cervical foregut duplication cysts present as asymptomatic lesions, associated symptoms vary with the location of the cyst and may include breathing or swallowing difficulties. Pathologically, the cysts are benign but have the potential to become infected and swell, which may result in airway compromise or sepsis. Ultrasonography, magnetic resonance imaging, and computed tomography can all be useful in defining the lesion. There have also been reports of ultrasonography being used to detect alimentary tract duplications prenatally.3 Coincidence of a foregut duplication cyst and Horner syndrome has been previously described but is not a common presentation.4 The differential diagnosis of a unilateral cystic neck mass in a child should include branchial cleft cyst, thymic cyst, and thymopharyngeal duct cyst. While a thyroglossal duct cyst is the most common congenital cyst in the pediatric population, it would be an unlikely consideration in this case because the cyst was too large and too far off midline. The final diagnosis is typically only confirmed by examination of histologic sections after excision, as shown in Figure 2, with demonstration of smooth muscle as well as nests of gastric mucosal tissue. Once the cervical foregut duplication cyst is identified, the definitive treatment is surgical excision, as was performed in this case. In all reported cases, to our knowledge, excision has been curative, with no reports of recurrence after excision.5 Although rare, foregut duplication cyst should be included in the differential diagnosis of a cystic neck mass in a pediatric patient. Return to Quiz Case 2. References 1. Qi BQ, Beasley SW, Williams AK. Evidence of a common pathogenesis for foregut duplications and esophageal atresia with tracheo-esophageal fistula. Anat Rec. 2001;264(1):93-10011505375PubMedGoogle ScholarCrossref 2. Edwards J, Pearson S, Zalzal G. Foregut duplication cyst of the hypopharynx. Arch Otolaryngol Head Neck Surg. 2005;131(12):1112-111516365227PubMedGoogle ScholarCrossref 3. el-Bitar MA, Milmoe G, Kumar S. Intralingual foregut duplication cyst in a newborn. Ear Nose Throat J. 2003;82(6):454-45612861873PubMedGoogle Scholar 4. Robinson GC, Dikrainian DA, Roseborough GF. Congenital Horner's syndrome and heterochromia iridum: their association with congenital foregut and vertebral anomalies. Pediatrics. 1965;35:103-10714223208PubMedGoogle Scholar 5. Fraser L, Howatson AG, MacGregor FB. Foregut duplication cyst of the pharynx. J Laryngol Otol. 2008;122(7):754-75618086334PubMedGoogle ScholarCrossref

Journal

Archives of Otolaryngology - Head & Neck SurgeryAmerican Medical Association

Published: Jul 18, 2011

Keywords: diagnostic radiologic examination,radiology specialty

References