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Unilateral submandibular depression
G Model ANORL-523; No. of Pages 3
ARTICLE IN PRESS European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2016) xxx–xxx
Available online at
ScienceDirect www.sciencedirect.com
What is your diagnosis?
Unilateral submandibular depression R. Bhoil a , K.A. Mistry a , R. Bhoil b,∗ , K. Thakur c a
Department of Radiodiagnosis, Dr. Rajendra Prasad Government Medical College, Kangra, HP, India Division of Prosthodontics, AFMC, Pune, India c Department of Otolaryngology, Dr. Rajendra Prasad Government Medical College, Kangra, HP, India b
1. Description A 20-year-old female complained of slight depression or “pit” in right submandibular region which was present since the past 4–5 years but has increased in size since 2 months (Fig. 1a). There was no history of pain, tenderness, sialadenitis or dysphagia; though she complained of occasional dryness in mouth but she never took any treatment for the same. Skin over both the
submandibular glands was normal and a slight depression was felt in right submandibular region. Oral cavity along with mucosa and submandibular papilla on both sides were normal. On massaging the right submandibular region, no saliva could be expressed out from the right duct orifice. The patient had caries in second and third molar teeth on right side. An ultrasound neck examination (Fig. 1b) and a CT scan of the neck (Fig. 1c and d) were performed.
∗ Corresponding author. 5-A The Mall Shimla, HP 171001, Shimla, India. Tel.: +91 9418012211. E-mail address: [email protected] (R. Bhoil). http://dx.doi.org/10.1016/j.anorl.2015.08.041 1879-7296/© 2016 Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Bhoil R, et al. Unilateral submandibular depression. European Annals of Otorhinolaryngology, Head and Neck diseases (2016), http://dx.doi.org/10.1016/j.anorl.2015.08.041
G Model ANORL-523; No. of Pages 3 2
ARTICLE IN PRESS R. Bhoil et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2016) xxx–xxx
Fig. 1. a: ultrasound of the neck right submandibular gland; b: ultrasound neck examination; c and d: axial and coranal CT scan images of the patient.
What is your diagnosis?
Please cite this article in press as: Bhoil R, et al. Unilateral submandibular depression. European Annals of Otorhinolaryngology, Head and Neck diseases (2016), http://dx.doi.org/10.1016/j.anorl.2015.08.041
G Model ANORL-523; No. of Pages 3
ARTICLE IN PRESS R. Bhoil et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2016) xxx–xxx
2. Answer On ultrasound of the neck right submandibular gland was not visualised (Fig. 1b). The left submandibular gland and bilateral parotids were normal. Computed-tomography revealed that the region of right submandibular gland was replaced by fat-density soft tissue contents (Fig. 1c and d). No significant lymphadenopathy was seen and the major neck vessels were normal. These imaging features suggested a diagnosis of unilateral submandibular gland aplasia. Congenital absence of major salivary glands is an extremely rare occurrence; with less than 40 cases reported [1]. Its cause is not clearly understood but may be related to arrest in organogenesis during early fetal life which may also be the reason for its association with other ectodermal malformations especially of the face like Treacher-Collins syndrome (mandibular facial dysotosis), congenitally malformed temporo-mandibular articulation or atresia of the lacrimal pucta [1–3]. In few of the reports familial cases have been described. Most frequently reported pattern is agenesis of all four major salivary glands, however, any of the glands or group of glands may be involved which can be unilateral or bilateral [2,3]. Patients may present with varied symptoms ranging from being completely asymptomatic to xerostomia, dysphagia and dental caries [3–5]. First-line treatment is symptomatic with hygiene and dietary rules. The mouth should be hydrated as often as possible [4]. If the patient continues to complain, saliva substitutes should
3
be added [5]. Preventive management of periodontal and dental disease is important. It is important for the clinician to be aware of the existence of this rare abnormality. Meticulous evaluation of patients with dryness of the mouth, poor oral hygiene and dental caries may lead to recognition of new cases and the prevention of the consequences particularly in the paediatric age group. Presence of other ectodermal defects should also raise the suspicion of this condition requiring further evaluation. Disclosure of interest The authors declare that they have no competing interest. References [1] Shipchandler TZ, Lorenz RR. Unilateral submandibular gland aplasia masquerading as cancer nodal metastasis. Am J Otolaryngol 2008;29:432–4. [2] Srinivasan A, Moyer JS, Mukherji SK. Unilateral submandibular gland aplasia associated with ipsilateral sublingual gland hypertrophy. AJNR Am J Neuroradiol 2006;27:2214–6. [3] Matsuda C, Matsui Y, Ohno K, Michi K. Salivary gland aplasia with cleft lip and palate: a case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;87:594–9. [4] Mathison CC, Hudgins PA. Bilateral submandibular gland aplasia with hypertrophy of sublingual glands. Otolaryngol Head Neck Surg 2008;138: 119–20. [5] Hahtanir A. CT and MR findings of bilateral submandibular gland aplasia associated with hypertrophied symmetrical sublingual glands herniated through mylohyoid defects. Dentomaxillofac Radiol 2012;41:79–83.
Please cite this article in press as: Bhoil R, et al. Unilateral submandibular depression. European Annals of Otorhinolaryngology, Head and Neck diseases (2016), http://dx.doi.org/10.1016/j.anorl.2015.08.041
ARTICLE IN PRESS European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2016) xxx–xxx
Available online at
ScienceDirect www.sciencedirect.com
What is your diagnosis?
Unilateral submandibular depression R. Bhoil a , K.A. Mistry a , R. Bhoil b,∗ , K. Thakur c a
Department of Radiodiagnosis, Dr. Rajendra Prasad Government Medical College, Kangra, HP, India Division of Prosthodontics, AFMC, Pune, India c Department of Otolaryngology, Dr. Rajendra Prasad Government Medical College, Kangra, HP, India b
1. Description A 20-year-old female complained of slight depression or “pit” in right submandibular region which was present since the past 4–5 years but has increased in size since 2 months (Fig. 1a). There was no history of pain, tenderness, sialadenitis or dysphagia; though she complained of occasional dryness in mouth but she never took any treatment for the same. Skin over both the
submandibular glands was normal and a slight depression was felt in right submandibular region. Oral cavity along with mucosa and submandibular papilla on both sides were normal. On massaging the right submandibular region, no saliva could be expressed out from the right duct orifice. The patient had caries in second and third molar teeth on right side. An ultrasound neck examination (Fig. 1b) and a CT scan of the neck (Fig. 1c and d) were performed.
∗ Corresponding author. 5-A The Mall Shimla, HP 171001, Shimla, India. Tel.: +91 9418012211. E-mail address: [email protected] (R. Bhoil). http://dx.doi.org/10.1016/j.anorl.2015.08.041 1879-7296/© 2016 Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Bhoil R, et al. Unilateral submandibular depression. European Annals of Otorhinolaryngology, Head and Neck diseases (2016), http://dx.doi.org/10.1016/j.anorl.2015.08.041
G Model ANORL-523; No. of Pages 3 2
ARTICLE IN PRESS R. Bhoil et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2016) xxx–xxx
Fig. 1. a: ultrasound of the neck right submandibular gland; b: ultrasound neck examination; c and d: axial and coranal CT scan images of the patient.
What is your diagnosis?
Please cite this article in press as: Bhoil R, et al. Unilateral submandibular depression. European Annals of Otorhinolaryngology, Head and Neck diseases (2016), http://dx.doi.org/10.1016/j.anorl.2015.08.041
G Model ANORL-523; No. of Pages 3
ARTICLE IN PRESS R. Bhoil et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2016) xxx–xxx
2. Answer On ultrasound of the neck right submandibular gland was not visualised (Fig. 1b). The left submandibular gland and bilateral parotids were normal. Computed-tomography revealed that the region of right submandibular gland was replaced by fat-density soft tissue contents (Fig. 1c and d). No significant lymphadenopathy was seen and the major neck vessels were normal. These imaging features suggested a diagnosis of unilateral submandibular gland aplasia. Congenital absence of major salivary glands is an extremely rare occurrence; with less than 40 cases reported [1]. Its cause is not clearly understood but may be related to arrest in organogenesis during early fetal life which may also be the reason for its association with other ectodermal malformations especially of the face like Treacher-Collins syndrome (mandibular facial dysotosis), congenitally malformed temporo-mandibular articulation or atresia of the lacrimal pucta [1–3]. In few of the reports familial cases have been described. Most frequently reported pattern is agenesis of all four major salivary glands, however, any of the glands or group of glands may be involved which can be unilateral or bilateral [2,3]. Patients may present with varied symptoms ranging from being completely asymptomatic to xerostomia, dysphagia and dental caries [3–5]. First-line treatment is symptomatic with hygiene and dietary rules. The mouth should be hydrated as often as possible [4]. If the patient continues to complain, saliva substitutes should
3
be added [5]. Preventive management of periodontal and dental disease is important. It is important for the clinician to be aware of the existence of this rare abnormality. Meticulous evaluation of patients with dryness of the mouth, poor oral hygiene and dental caries may lead to recognition of new cases and the prevention of the consequences particularly in the paediatric age group. Presence of other ectodermal defects should also raise the suspicion of this condition requiring further evaluation. Disclosure of interest The authors declare that they have no competing interest. References [1] Shipchandler TZ, Lorenz RR. Unilateral submandibular gland aplasia masquerading as cancer nodal metastasis. Am J Otolaryngol 2008;29:432–4. [2] Srinivasan A, Moyer JS, Mukherji SK. Unilateral submandibular gland aplasia associated with ipsilateral sublingual gland hypertrophy. AJNR Am J Neuroradiol 2006;27:2214–6. [3] Matsuda C, Matsui Y, Ohno K, Michi K. Salivary gland aplasia with cleft lip and palate: a case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;87:594–9. [4] Mathison CC, Hudgins PA. Bilateral submandibular gland aplasia with hypertrophy of sublingual glands. Otolaryngol Head Neck Surg 2008;138: 119–20. [5] Hahtanir A. CT and MR findings of bilateral submandibular gland aplasia associated with hypertrophied symmetrical sublingual glands herniated through mylohyoid defects. Dentomaxillofac Radiol 2012;41:79–83.
Please cite this article in press as: Bhoil R, et al. Unilateral submandibular depression. European Annals of Otorhinolaryngology, Head and Neck diseases (2016), http://dx.doi.org/10.1016/j.anorl.2015.08.041