A 4-year seven-month-old, Tunisian boy was referred to the Pediatric Dentistry Department at the Faculty of Dental Medicine of Monastir (Tunisia) complaining of pain related to poor oral and dental condition.
The patient' medical history revealed that the young boy was initially diagnosed with SSS by his pediatrician who relied on clinical features, consanguineous factor, and biochemical measures to confirm the diagnosis.
The patient was born to consanguineous parents and had no other siblings.
He had history of full-term birth by caesarean section with a birth weight of 2 kg, a birth height of 42 cm, and a head circumference of 31 cm.
The patient's medical history reported also episodes of tetany and convulsions associated with signs and symptoms of hypocalcemia from the 15th day of birth. The seizures lasted a few minutes and were resolved without intravenous antiepileptic drug administration.
The initial patient's biochemical examinations showed hyperphosphatemia (2.5 mmol/L), hypocalcemia (1.8 mmol/L), and very low levels of parathyroid hormone (4.2 pg/L) (Table 1).
Vitamin D and oral calcium preparations were prescribed by the patient's pediatrician during clinical follow-ups, but due to recurrence of hypocalcemia, second episodes of seizures were observed at the age of 13 months.
A16 months, the pediatrician general examination revealed characteristic facial dysmorphism with microcephaly (-3 standard deviation [SD]) and severe growth retardation (-4 SD for height, -3 SD for weight).
At this age, the patient height was 80 cm and weight was 14 kg.
The general examination demonstrated also intrauterine growth retardation (IUGR), oligohydramnios, and fetal distress. His hands and feet were small with thin and short digits, which gave him a very short stature.
No congenital heart abnormality was reported.
The genetic testing revealed 12 bp (155-166 del) within the TBCE gene in exon 3 confirming the diagnosis of SSS.
The neurological examination revealed an intellectual disability, with a mental retardation, a psychomotor delay, and a severe speech delay which made the dental examination and treatment very difficult to perform.
Extraoral head and neck examination confirmed the diagnosis of SSS and reported typical craniofacial features of this syndrome such as microcephaly, large set ears, deep-set eyes, prominent forehead, beaked nose, thin lips, and marked philtrum (Figure 1).
Intraoral examination revealed micrognathic mandible and maxilla, an arched palate, and small dental arches with an open bite.
All the maxillary and mandibular teeth were severely decayed due to the poor oral hygiene, the plaque accumulation, and the enamel hypoplasia (Figure 2).
Radiographic examinations were not possible due to the patient behavior.
The proposed treatment plan was discussed and approved by the patient's parents.
Written informed consent was obtained from the child's parents for all imaging exams, treatment modalities, and data publications.
The full dental treatment was planned and summarized in Table 2.
A pulpotomy was performed on teeth #62 and #52 after removal of the coronal pulp using a sterile excavator. The pulp chamber was then rinsed with normal saline, and the bleeding was controlled using a small cotton pellet.
A zinc oxide-eugenol mixed to a thick consistency was then applied and condensed over the imputed pulp of the prepared cavity.
Finally, the rest of the cavity was partially filled with type IX glass ionomer cement (GIC) (Riva self-cure, SDI Australia) and then restored with resin composite (3M  FZ100 MP-USA).
A root canal therapy was performed on teeth #51, #61, #54, #55, #64, #65, #74, #75, #84, and #85, by the same pediatric dentist using the same procedure.
After administration of local anesthesia, coronal access was performed with a #02 round high-speed carbide burr without isolation using a rubber dam since isolation was difficult to perform for this patient.
Then, after identification of the canal orifices, the root canals were manually prepared using stainless steel manual K-files (Dentsply-Maillefer, Ballaigues, Switzerland) in a step back manner through size #35 by quarter-turn-pull technique. The root length was determined approximately given the impossibility of taking X-rays. The pulp chamber was abundantly irrigated with 2.5% sodium hypochlorite.
All the root canals were dried using paper points and sealed using zinc oxide-eugenol. Finally the access cavities were restored with type IX GIC (glass ionomer cement) (Riva self-cure, SDI Australia).
Composite resin restorations (3M  FZ100 MP-USA) were placed on the maxillary and mandibular incisors and stainless-steel crowns (3M  ESPE -USA) on all maxillary and mandibular primary molars.
All the dental treatments were carried out over several sessions under local anesthesia because the patient's parents refused categorically general anesthesia.
 Figure 3 shows the postoperative restorative treatments.
Dietary advice with instructions on oral hygiene was given for the parents, and regular follow-up visits were scheduled every 6 months and showed a very satisfactory clinical condition of all the restorative treatments.
Regular follow-up visits were scheduled, and dental prophylaxis was performed at each visit.