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Radiology Quiz Case 2: Diagnosis

Radiology Quiz Case 2: Diagnosis Diagnosis: Imperforate right submandibular duct Infants may present with congenital masses in the floor of the mouth from a number of different causes.1 Rarely, a congenital imperforate submandibular duct (Wharton duct) orifice in a newborn can result in a cystic swelling with a bluish, translucent appearance. Failure of canalization of the terminal end of the duct is the cause of this kind of anomaly,2 and in most reported cases, a unilateral swelling is seen at birth.3 The differential diagnoses of this pathologic finding are extensive and include sialolithiasis, mucous retention phenomenon (ranula, mucocele), dermoid cyst, thyroglossal duct cyst, hemangioma, cystic hygroma, and lipoma.4 Cystic hygroma (or lymphangioma) is represented by individual, cystic cavities, which are often located unilaterally in the neonate's neck; it is a histologically benign congenital tumor of lymphatic origin. Endothelial membranes sprouting embryonically sequestered lymph vessels form outpouchings that penetrate into surrounding normal tissues, canalizing and producing large multiloculated cysts that are filled with serous secretions.5 Cystic hygroma presents as a tense elastic swelling that is covered by normal skin and can suddenly increase in volume. Patients can be asymptomatic, with only an aesthetic impact, or can have breathing difficulties and dysphagia owing to airway and digestive tract compression. The relationship of a cystic hygroma to the adjacent soft tissues of the neck is best demonstrated on MRI. The most common pattern is that of a mass with low or intermediate signal intensity on T1-weighted images and high signal intensity on T2-weighted images. Rarely, the lesion may be hyperintense on T1-weighted sequences, a finding that is associated with clotted blood or high lipid (chyle) content. In the case of hemorrhage, fluid-fluid levels may be observed.6 A ranula is a cystic indolent swelling of the floor of the mouth with a smooth surface that is caused by a traumatic or an inflammatory stenosis of the sublingual duct. The content may be serous or mucous. The symptoms include difficulty in articulation of words, dysphagia, reduced tongue mobility, and pain (in the case of infection).7 On MRI, the ranula's characteristic appearance is usually dominated by its high water content. Therefore, it has a low T1-weighted signal intensity, an intermediate proton density, and high T2-weighted signal intensity. This appearance, especially in a plunging ranula, may be similar to that of a lymphangioma (or cystic hygroma). However, if the protein concentration in the ranula's contents is high, the signal intensities can vary, often being high on all imaging sequences.8 Common features of the imperforate submandibular duct are persistent swelling in the anterior floor of the mouth, difficulty with feeding, or enlargement with crying. There is no clinically definitive way to distinguish these sialoceles from ranulas or cystic hygromas. However, they have some characteristic features, such as their congenital nature, their translucent, bluish hue, and the inability to milk saliva from the ipsilateral duct orifice. To date, to our knowledge, definitive MRI findings of atresia of the submandibular duct orifice have not been reported. Only a few authors have emphasized the usefulness of this kind of diagnostic feature for this imperforate Wharton duct.9,10 However, radiology can help in reaching a definite diagnosis, as in our case. In fact, MRI showed an extensive and uniform swelling throughout the course of the anterior branch of the right submandibular duct. It was 7 mm wide, and its course was tortuous up to the outlet in the floor of the mouth, where the lumen was narrowed, but without defects related to salivary calculi. Similar findings, though minor, were detected even in the rear section. There were no enlarged lymph nodes in the main stations in the neck. A thorough consideration of the imaging findings revealed some details that strongly pointed to the diagnosis of an imperforate submandibular duct: (1) the spiral-shape appearance, which was underlined by 3-dimensional MRI reconstruction; (2) location in the most anterior part of the oral cavity, in the expected position for the outlet of the submandibular duct in the lingual region; and (3) branching of the mass close to the gland. In our patient, a follow-up US obtained 10 months after the first scan showed that the cystic formation had regressed completely and spontaneously; this finding was confirmed by negative clinical examination results and regular leakage of saliva from the right Wharton duct. Return to Quiz Case. References 1. Rosow DE, Ward RF, April MM. Sialodochostomy as treatment for imperforate submandibular duct: a systematic literature review and report of two cases. Int J Pediatr Otorhinolaryngol. 2009;73(12):1613-161519380165PubMedGoogle ScholarCrossref 2. Amin MA, Bailey BM. Congenital atresia of the orifice of the submandibular duct: a report of 2 cases and review. Br J Oral Maxillofac Surg. 2001;39(6):480-48211735147PubMedGoogle ScholarCrossref 3. Pal K, Abdulla AM. Congenital imperforate submandibular duct in a newborn. Indian J Pediatr. 2007;74(7):687-68817699979PubMedGoogle ScholarCrossref 4. Addante RR. Congenital cystic dilatation of the submandibular duct. Oral Surg Oral Med Oral Pathol. 1984;58(6):656-6586594655PubMedGoogle ScholarCrossref 5. Gurulingappa , Awati MN, Aleem MA. Cystic hygroma: a difficult airway and its anaesthetic implications. Indian J Anaesth. 2011;55(6):624-62622223912PubMedGoogle ScholarCrossref 6. Koeller KK, Alamo L, Adair CF, Smirniotopoulos JG. Congenital cystic masses of the neck: radiologic-pathologic correlation. Radiographics. 1999;19(1):121-146, 152-1539925396PubMedGoogle ScholarCrossref 7. Arnold W, Ganzer U, Iurato S. Checklist Otorinolaringoiatria. Rome, Italy: CIC Edizione Internazionali; 2007:359-360 8. Gupta A, Karjodkar FR. Plunging ranula: a case report. ISRN Dent. 2011;2011:80692821991487PubMedGoogle Scholar 9. Ulualp SO, Rodriguez SC, Hernandez J, Hay M. Bilateral atresia of the submandibular duct orifices. Am J Otolaryngol. 2007;28(3):184-18617499136PubMedGoogle ScholarCrossref 10. Pownell PH, Brown OE, Pransky SM, Manning SC. Congenital abnormalities of the submandibular duct. Int J Pediatr Otorhinolaryngol. 1992;24(2):161-1691428595PubMedGoogle 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 138 (8) – Aug 1, 2012

Radiology Quiz Case 2: Diagnosis

Abstract

Diagnosis: Imperforate right submandibular duct Infants may present with congenital masses in the floor of the mouth from a number of different causes.1 Rarely, a congenital imperforate submandibular duct (Wharton duct) orifice in a newborn can result in a cystic swelling with a bluish, translucent appearance. Failure of canalization of the terminal end of the duct is the cause of this kind of anomaly,2 and in most reported cases, a unilateral swelling is seen at birth.3 The differential...
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Publisher
American Medical Association
Copyright
Copyright © 2012 American Medical Association. All Rights Reserved.
ISSN
0886-4470
eISSN
1538-361X
DOI
10.1001/archotol.138.8.774
Publisher site
See Article on Publisher Site

Abstract

Diagnosis: Imperforate right submandibular duct Infants may present with congenital masses in the floor of the mouth from a number of different causes.1 Rarely, a congenital imperforate submandibular duct (Wharton duct) orifice in a newborn can result in a cystic swelling with a bluish, translucent appearance. Failure of canalization of the terminal end of the duct is the cause of this kind of anomaly,2 and in most reported cases, a unilateral swelling is seen at birth.3 The differential diagnoses of this pathologic finding are extensive and include sialolithiasis, mucous retention phenomenon (ranula, mucocele), dermoid cyst, thyroglossal duct cyst, hemangioma, cystic hygroma, and lipoma.4 Cystic hygroma (or lymphangioma) is represented by individual, cystic cavities, which are often located unilaterally in the neonate's neck; it is a histologically benign congenital tumor of lymphatic origin. Endothelial membranes sprouting embryonically sequestered lymph vessels form outpouchings that penetrate into surrounding normal tissues, canalizing and producing large multiloculated cysts that are filled with serous secretions.5 Cystic hygroma presents as a tense elastic swelling that is covered by normal skin and can suddenly increase in volume. Patients can be asymptomatic, with only an aesthetic impact, or can have breathing difficulties and dysphagia owing to airway and digestive tract compression. The relationship of a cystic hygroma to the adjacent soft tissues of the neck is best demonstrated on MRI. The most common pattern is that of a mass with low or intermediate signal intensity on T1-weighted images and high signal intensity on T2-weighted images. Rarely, the lesion may be hyperintense on T1-weighted sequences, a finding that is associated with clotted blood or high lipid (chyle) content. In the case of hemorrhage, fluid-fluid levels may be observed.6 A ranula is a cystic indolent swelling of the floor of the mouth with a smooth surface that is caused by a traumatic or an inflammatory stenosis of the sublingual duct. The content may be serous or mucous. The symptoms include difficulty in articulation of words, dysphagia, reduced tongue mobility, and pain (in the case of infection).7 On MRI, the ranula's characteristic appearance is usually dominated by its high water content. Therefore, it has a low T1-weighted signal intensity, an intermediate proton density, and high T2-weighted signal intensity. This appearance, especially in a plunging ranula, may be similar to that of a lymphangioma (or cystic hygroma). However, if the protein concentration in the ranula's contents is high, the signal intensities can vary, often being high on all imaging sequences.8 Common features of the imperforate submandibular duct are persistent swelling in the anterior floor of the mouth, difficulty with feeding, or enlargement with crying. There is no clinically definitive way to distinguish these sialoceles from ranulas or cystic hygromas. However, they have some characteristic features, such as their congenital nature, their translucent, bluish hue, and the inability to milk saliva from the ipsilateral duct orifice. To date, to our knowledge, definitive MRI findings of atresia of the submandibular duct orifice have not been reported. Only a few authors have emphasized the usefulness of this kind of diagnostic feature for this imperforate Wharton duct.9,10 However, radiology can help in reaching a definite diagnosis, as in our case. In fact, MRI showed an extensive and uniform swelling throughout the course of the anterior branch of the right submandibular duct. It was 7 mm wide, and its course was tortuous up to the outlet in the floor of the mouth, where the lumen was narrowed, but without defects related to salivary calculi. Similar findings, though minor, were detected even in the rear section. There were no enlarged lymph nodes in the main stations in the neck. A thorough consideration of the imaging findings revealed some details that strongly pointed to the diagnosis of an imperforate submandibular duct: (1) the spiral-shape appearance, which was underlined by 3-dimensional MRI reconstruction; (2) location in the most anterior part of the oral cavity, in the expected position for the outlet of the submandibular duct in the lingual region; and (3) branching of the mass close to the gland. In our patient, a follow-up US obtained 10 months after the first scan showed that the cystic formation had regressed completely and spontaneously; this finding was confirmed by negative clinical examination results and regular leakage of saliva from the right Wharton duct. Return to Quiz Case. References 1. Rosow DE, Ward RF, April MM. Sialodochostomy as treatment for imperforate submandibular duct: a systematic literature review and report of two cases. Int J Pediatr Otorhinolaryngol. 2009;73(12):1613-161519380165PubMedGoogle ScholarCrossref 2. Amin MA, Bailey BM. Congenital atresia of the orifice of the submandibular duct: a report of 2 cases and review. Br J Oral Maxillofac Surg. 2001;39(6):480-48211735147PubMedGoogle ScholarCrossref 3. Pal K, Abdulla AM. Congenital imperforate submandibular duct in a newborn. Indian J Pediatr. 2007;74(7):687-68817699979PubMedGoogle ScholarCrossref 4. Addante RR. Congenital cystic dilatation of the submandibular duct. Oral Surg Oral Med Oral Pathol. 1984;58(6):656-6586594655PubMedGoogle ScholarCrossref 5. Gurulingappa , Awati MN, Aleem MA. Cystic hygroma: a difficult airway and its anaesthetic implications. Indian J Anaesth. 2011;55(6):624-62622223912PubMedGoogle ScholarCrossref 6. Koeller KK, Alamo L, Adair CF, Smirniotopoulos JG. Congenital cystic masses of the neck: radiologic-pathologic correlation. Radiographics. 1999;19(1):121-146, 152-1539925396PubMedGoogle ScholarCrossref 7. Arnold W, Ganzer U, Iurato S. Checklist Otorinolaringoiatria. Rome, Italy: CIC Edizione Internazionali; 2007:359-360 8. Gupta A, Karjodkar FR. Plunging ranula: a case report. ISRN Dent. 2011;2011:80692821991487PubMedGoogle Scholar 9. Ulualp SO, Rodriguez SC, Hernandez J, Hay M. Bilateral atresia of the submandibular duct orifices. Am J Otolaryngol. 2007;28(3):184-18617499136PubMedGoogle ScholarCrossref 10. Pownell PH, Brown OE, Pransky SM, Manning SC. Congenital abnormalities of the submandibular duct. Int J Pediatr Otorhinolaryngol. 1992;24(2):161-1691428595PubMedGoogle ScholarCrossref

Journal

Archives of Otolaryngology - Head & Neck SurgeryAmerican Medical Association

Published: Aug 1, 2012

Keywords: radiology specialty

References