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CHAPTER 39

                          Salivary Gland Diseases in


                         Children and Adolescents


                                                  Sunday Olusegun Ajike
                                                       Kokila Lakhoo




                           Introduction                        Table 39.1: Classification of salivary gland diseases in children.
        Salivary glands are found in and around the oral cavity, and they are   Nonneoplastic tumours
        divided  into  major  and  minor  salivary  glands.  The  major  salivary             Congenital/developmental
        glands are the parotid, submandibular, and sublingual glands; the minor
        salivary glands are located in the lips, buccal mucosa, palate, and throat.     Agenesis/aplasia, hypogenesis/hypoplasia
        Generally,  salivary  gland  diseases  are  not  common  in  the  paediat-     Aberrant/ectopic salivary gland
        ric  population.  The  classification  of  salivary  gland  diseases  is  very      Haemangioma
        complex  because  it  encompasses  different  entities;  however,  precise      Lympangioma
        classification  and  terminology  are  necessary  for  accurate  diagnosis             Inflammatory and infection.
        and management. As in adults, diseases of the salivary glands may be
        nonneoplastic or neoplastic (tumours) (Table 39.1). The pattern of inci-     Acute sialadentis
        dence in the paediatric population differs greatly from that in the adult           Mumps, cytomegalovirus, Coxasackie A or B or parainfluenza virus)
        group. Most salivary gland lesions in children are either inflammatory           Human immunodeficiency virus (HIV)-associated salivary glands
        or  vascular  in  origin.  Of  the  developmental  salivary  gland  diseases,      Recurrent parotitis in children (RPC)
        haemangiomas are the most common. In the African paediatric popula-            Autoimmune
        tion, mumps is the most common in the inflammatory/infection group,
        but  in  the  developed  world,  only  sporadic  cases  of  mumps  are  now      Sjogren’s syndrome
        reported, and rhabdomyosarcomas are the most common nonodonto-            Cysts
        genic mesenchymal tumours in children.                         Ranula mucocele (mucous retention cyst)
           Neoplastic  changes  in  the  paediatric  population  are  very  rare      Salivary gland dysfunction
        compared to the inflammatory groups. In the population as a whole,           Xerostomia
        salivary gland neoplasms constitute 2.8% of all head and neck tumours,           Sialorrhea/ptyalism
        but in children it accounts for about 10% of all childhood neoplasms
        and between 3% and 22% of epithelial salivary gland neoplasms. The
        majority (88.5%) of salivary gland tumours are benign; the remaining   Neoplastic tumours
        11.5% being malignant. In children, the most common benign epithelial             Benign
        tumour  is  pleomorphic  adenoma,  and  the  most  common  malignant           Pleomorphic adenoma
        tumour is mucoepidermoid carcinoma.                               Warthin’s tumour
           Salivary  gland  tumours  in  children  have  the  same  clinical  and
        biologic  behavior  as  those  in  the  adult. The  majority  (76.7%)  occur             Malignant
        in the major glands, with the remainder in the minor glands, a ratio of           Mucoepidermoid carcinoma
        3.3:1. The ratio of occurrence of parotid to submandibular to sublingual           Acinic cell carcinoma
        tumours in the major salivary glands is 30:6:1. Globally, these tumours           Adenoid cystic carcinoma
        occur  predominantly  in  girls  and  at  any  childhood  age.  A  detailed             Mesenchymal tumours
        clinical history with imaging features narrows the differential diagnosis
        while  providing  useful  information  for  management  and  prognosis.           Neural tissue
        Incisional  biopsy  must  be  avoided  due  to  the  possibility  of  tumour           Neurofibroma
        spillage and facial nerve damage.                                   Muscular tissue
           The treatment of salivary gland diseases is categorised into medical           Rhabdomyosarcoma
        and  surgical,  depending  on  the  nature  of  the  disease  condition.  The
        neoplastic lesions usually require surgical intervention, with or without
        radiation and chemotherapy, whereas the nonneoplastic/inflammatory
        diseases are managed symptomatically and conservatively. A protracted
        conservative medical management is strongly advised, however, before
        surgical ablation is considered in children.
                          Investigations
        Salivary gland enlargement is a diagnostic challenge to the attending sur-
        geon because the glands could be involved in a wide spectrum of diseases.
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