In August 2016, a six-year-six-month-old female was referred to the Pediatric Dentistry Postgraduate Program Clinic for routine oral examination and possible treatment. The general and craniofacial features were suggestive of a rare syndrome, with retarded growth according to her chronological age, strange walking, and normal speech. The parents (father 26 and mother 29 years old, both healthy) reported that the patient was diagnosed with a nonsevere form of Morquio syndrome four years ago, confirmed by a significant increase in keratan sulfate in urine and a marked deficiency of galactosamine-6-sulfate activity in leucocyte cultures. No additional diagnostic tests (e.g., genetic analyses) for the condition were undertaken because the condition was diagnosed previously, and they represented a strong monetary outlay for the parents. At that time, the parents were recommended by the medical team to initiate treatment with enzyme replacement; however, the child had not received any type of therapy since the diagnosis by their own decision. The family history did not reveal any other relevant information. According to an exhaustive nutrition/diet questioning through the clinical history, her carbohydrate daily consumption (food and drinks) was considered low.\nThe clinical body examination manifested evident short stature and neck, slight scaphocephaly, bony deformities, protruded chest, stubby hands, kyphosis, and rotated legs. No neurological abnormalities were detected. The most important craniofacial features were brachycephaly, convex/biprotusive profile, flat nasal bridge, closed nasolabial angle, apparent large mouth, and broad lips (). Intraoral findings included a partially complete mixed dentition with several deep caries or cavities in all primary molars, ovoid arcades, anterior crossbite, mild open bite, macroglossia, and tongue thrusting; soft tissues were normal, but generalized moderate gingivitis was present; there also was high labial frenum (). Parents reported occasional oral abnormal habits such as onychophagia, thumb sucking, and mouth breathing. Both temporomandibular joints were diagnosed as normal, according to a clinical examination involving manual palpation of the masticatory muscles; mouth opening, closing, and lateral movements, or deviations of the mandible; and lateral and dorsal extra-auricular auscultation with the aid of a stethoscope for the determination of abnormal joint sounds. Oral hygiene was considered poor. The patient exhibited significant anxiety and difficulties in maintaining an adequate supine posture on the dental chair.\nAn adequate restorative/endodontic/orthodontic treatment plan was designed and presented to the parents. They agreed and signed an informed consent form. Initially, the patient was behaviorally managed employing persistently different noninvasive psychological techniques (e.g., conditioning, gradual desensitization, “tell-show-do,” and positive reinforcement), dental prophylaxis, and fluoride varnish applications; subsequently, a fair-to-good cooperation level was achieved. The restorative treatment consisted of the placement of preformed metallic crowns on all primary molars. Pulpotomies were performed in the right and left maxillary first and second primary molars, left mandibular first primary molar, and right mandibular second primary molar; and pulpectomies were performed in the left mandibular second primary molar and right mandibular first primary molar, under local anesthesia (lidocaine plus epinephrine) and rubber dam isolation. Pit and fissure sealants were also applied on the four permanent first molars (October 2016) (). Additionally, a preventive program was initiated and supervised. Anterior teeth exhibited some small enamel chalky irregularities ().\nThen, the orthodontic clinical and cephalometric diagnosis (Figures and ) was carried out, determining the presence of a pseudo-class III malocclusion. A Hyrax appliance was placed for four months (June 2017) (). In October 2017, Hawley and Schwartz removable orthodontic appliances were placed, and in November 2017, a laser labial frenulectomy was carried out. Nine months later, the Hawley appliance was substituted by a fixed Nance button with “Z” springs over the upper incisors for treating the anterior crossbite ().\nUnexpectedly, two months later, a gingival lesion appeared above the right upper central incisor, diagnosed presumptively as a “localized juvenile spongiotic gingival hyperplasia (LJSGH)” (); after discussing with the oral surgeon, it was decided only to observe the lesion, without any kind of treatment. It healed spontaneously during the next 10 months approximately ().\nIn July 2019, the occlusal problems have been almost resolved (). The last appointment was in September 2019 for clinical and radiographic control (). In this orthodontic stage, the cooperation and participation levels of the child and her parents were considered excellent. The patient will be closely followed in order to assess the evolution of the potential class III malocclusion.
Case four describes a seven-year-old male with mucopolysaccharidosis type IIIB (Sanfilippo Syndrome). Medical comorbidities included mitral and aortic valve dysplasia, intellectual disability, behavioral challenges, abnormal gait, and impaired hearing. On clinical examination, he had coarse facial features and hirsutism.\nHe was referred to the dental department for gross caries (Figure ) and received comprehensive dental treatment under general anesthesia. This included composite restoration and fissure sealant provision of the maxillary left second primary molar (ULE) (Figure ) and multiple extractions; mandibular primary molars and canines (LRE, LRD, LRC, LLC, LLD, LLE), mandibular left primary central incisor (LLA), maxillary right primary lateral incisor (URB), and left central incisor (ULA). He also received 22,600ppm fluoride application.