Abstract
Osteopathia striata combined with cranial sclerosis (OSCS) is a skeletal dysplasia, characterized by linear striation in the long bones and cranial osteosclerosis. OSCS leads to various abnormalities in the cranial and facial bones and disfigurements, including dental arch discrepancies. Here, we present a case of OSCS where dental arch discrepancies were corrected with bimaxillary orthognathic surgery. Boney sclerosis leads to an increase in bone density that can cause complications during and after surgery. Therefore, it is important to evaluate these patients thoroughly with cone beam computed tomography before performing surgery. Analyze of previous surgical procedures as well as adaptation of the surgical technique to the hard brittle bone must take place.
Highlights
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Osteopathia striata (OS) is a rare skeletal dysplasia, affecting cranial skeleton.
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Patients with OSCS have marked increases in bone density; that may cause intraoperative damage to surgical instruments.
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Dental arch discrepancies can be the result for OS-patients.
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The boney sclerosis in OS increases the surgical risk during orthognathic surgery.
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Evaluate OS thoroughly with cone beam computed tomography prior orthognathic surgery.
1
Introduction
Osteopathia striata combined with cranial sclerosis (OSCS) is a rare sclerosing bone dysplasia with variable clinical findings and specific radiological findings. OSCS is characterized by craniofacial hyperostosis and linear striations in the diaphysis and metaphysis of the long bones and the skull, due to increased osteoblastic activity [ , ]. The estimated prevalence of OSCS is less than 0.1 in 1 million, and a few more than 100 cases have been reported worldwide. OSCS is generally lethal in fetal and neonatal males, but affected females tend to survive with less severe phenotypes [ ].
Previous case studies typically described patients with craniofacial dysmorphisms, including macrocephaly, frontal bossing, hypertelorism, broad nasal bridge, conductive deafness, cleft palate, and mental retardation [ , ]. Nischiguchi et al. [ ] stated that we lack reports on dental findings in patients with OSCS. It has been reported that 30.7% of patients with OSCS have dental anomalies, including microdontia, unerupted teeth, abbreviated roots, pulp obliteration, malocclusion, and dental arch discrepancies [ , ].
Patients with OSCS have marked increases in bone density; bone descriptions have ranged from chalky to extremely hard. In some cases, high bone densities even caused intra-operative damage to surgical instruments. Changes in bone density can influence the time required for surgery and the risk of postsurgical complications, such as delayed union, nonunion, or infection [ , ].
Here, we describe a female patient with OSCS that underwent bimaxillary surgery to normalize a facial skeleton discrepancy. To the best of our knowledge, this study was the first to describe the surgical procedure and the pattern of bone healing in a patient with OSCS that underwent orthognathic surgery.
2
Case report
A female patient, born 1998, was referred to the Regional Cleft Team at the Umeå University Hospital in 2010. The patient was referred due to clinical status, which included hypertelorism, frontal bossing, impacted maxillary and mandibular premolars, an isolated cleft in the soft palate with bifid uvula, a small non-significant fistula on the border between the hard and soft palate, and a basal discrepancy with a negative overjet of 5 mm. The medical examination performed by the Regional Cleft Team included an examination in the department of Medical and Clinical Genetics to determine whether the condition was genetic. Heredity could not be determined, because both the patient’s parents were adopted. A differential diagnosis was considered in a multidisciplinary discussion. The OSCS diagnosis was determined in May, 2012. The isolated cleft in the soft palate and the small fistula on the border between the hard and soft palates did not require intervention from the cleft surgeon. The speech pathologist found no cleft palate-related speech characteristics (such as hypernasal speech or compensatory articulation), but some voice hyperfunction was noted. The oral and maxillofacial surgeon, together with the orthodontist, diagnosed a sagittal discrepancy that resulted in a negative overjet, which caused masticatory problems. The patient also experienced a delay in the eruption of the maxillary and mandibular permanent molars. A radiographic examination showed dense/hypermineralized bone in the facial skeleton, impacted premolars, and abnormal anatomy in the dental roots. Despite the malocclusion, the patient had a normal range of jaw movement with no tenderness in the muscle or temporomandibular joint.
A treatment plan was determined, with the goal of normalizing the patient’s basal and dental relationships between the maxilla and mandible. The risk of surgery-related complications was discussed and evaluated, keeping in mind the density of the facial skeleton.
The treatment started with surgical exposure and orthodontic traction, with a gold chain bonded onto the impacted teeth. This surgery was successful without complications as bone sequesters or local infections. After the teeth erupted to a normal position. The next step in rehabilitation was to align the dental arches. The presurgical orthodontic treatment included a fixed appliance in the mandible, maintained from August 2013 to September 2015 ( Fig. 1 ) for decompensation of the inclination of the anterior teeth. Impacted mandibular wisdom teeth were removed 2016.
