Crouzon disease--a case report

The present case of a ten year old boy with craniofacial dysostosis with the features of midfacial hypoplasia is a disease known as Crouzon disease. This disease is characterised by cranial deformities, facial malformation, eye changes and occasional other associated abnormalities. The aim of this case is to discuss the clinical, radiographic features and management of the problems.     

Prosthetic rehabilitation of a Crouzon patient: A case report

Crouzon syndrome is a rare genetic disorder, which can be defined as a variation of craniofacial dysostosis caused by the premature obliteration and ossification of two or more sutures. The growth pattern results in pseudoprognathism and malocclusions including an overcrowded or a widely spaced dentition. Specifically maxillary arch is narrow, high, and V-shaped. Cleft palate and bifid uvula are other possible features in the oral cavity. This report describes a non-surgical treatment model to overcome the remaining significant Class III intermaxillary relation and excessive tooth loss to recover function and aesthetics for a 25-year-old Crouzon patient.     

Early orthodontic management of Crouzon Syndrome: a case report

Crouzon Syndrome is an autosomal dominant disorder with complete penetrance and variable expressivity. Described by a French neurosurgeon in 1912, it is a rare genetic disorder. Crouzon syndrome is caused by mutation in the fibroblast growth factor receptor 2 (FGFR2) gene. The disease is characterized by premature synostosis of coronal and sagittal sutures which begins in the first year of life. Once the sutures become closed, growth potential to those sutures is restricted. However, multiple sutural synostoses frequently extend to premature fusion of skull base causing midfacial hypoplasia, shallow orbit, maxillary hypoplasia and occasional upper airway obstruction. The case of a 7-year-old South African black boy with Crouzon Syndrome is presented. He presented with characteristic triad of cranial deformity, maxillary hypoplasia and exophthalmos. The clinical, cephalometric features and initial orthodontic management of this patient are discussed as part of multidisciplinary management.     

Crouzon syndrome with acanthosis nigricans: case report and mutational analysis.

OBJECTIVE: To describe the 22nd case of Crouzan syndrome with acanthosis nigricans, a hyperkeratotic skin disorder with hyperpigmentation. METHODS: DNA analysis and sequencing of the FGFR3 gene were performed. RESULTS: The 13-year-old Japanese boy described here also had dyspnea, facial palsy, sensorineural hearing loss, and skeletal and mental retardation. Examination of a skin biopsy specimen revealed the typical findings of acanthosis nigricans. Genetic analysis revealed the Ala391Glu mutation in one FGFR3 gene. CONCLUSIONS: Crouzon syndrome with acanthosis nigricans is a distinct clinical entity different from classic Crouzon syndrome.     

Familial Crouzon syndrome

Crouzon syndrome is an autosomal dominant condition of the craniosynostotic syndromes without syndactyly and with various dentofacial anomalies. Craniosynostosis, maxillary hypoplasia, shallow orbits, ocular proptosis and hypertelorism are the characteristic features of Crouzon syndrome. This report describes the variable clinical features in affected individuals over two generations of a family with dentofacial deformities and review of literature.     

A reflectable case of obstructive sleep apnea in an infant with Crouzon syndrome

Obstructive sleep apnea has recently drawn attention as a cause of sudden death among infants. Life-threatening obstruction of the upper airway is encountered in patients with syndromic craniosynostosis. Early definitive management of obstructive sleep apnea can conquer this critical situation. Although early tracheostomy can solve the problem, successful early midfacial distraction has been reported. In this report, a reflectable case of sudden death caused by a severe obstructive sleep apnea attack at home just before the midfacial distraction, during the waiting period for the surgery of midfacial distraction, is described. The authors stress the importance of preoperative care of the upper airway and the early definitive treatment using distraction osteogenesis for midfacial hypoplasia in infantile syndromic craniosynostosis.     

Orbitofrontal monobloc advancement for Crouzon syndrome

IntroductionUsually, patients suffering from Crouzon syndrome have synostosis of coronal sutures, exophthalmia, hypertelorism, and hypoplasia of the middle third of face. Sometimes maxillary retrusion is absent, so these patients have class I or II relationship. In these cases, frontofacial monobloc advancement, which is the gold standard, increases the maxillo-mandibular dysmorphia. Therefore we propose orbitofrontal monobloc advancement minus dental arch, without splits of the pterygoid plates.Case reportA 12-year-old girl with Crouzon syndrome had intracranial hypertension, exophthalmia, a middle third retrusion and a class II occlusion. We achieved orbitofrontal monobloc advancement which is frontofacial monobloc advancement minus maxillary dental arch. Four distractors KLS Martin were used. After 20 days of distraction, the final advancement was 10.2 mm for cranial distractors and 10.5 mm at fronto-zygomatic. Distractors were removed after 8 months.DiscussionWe offer patients suffering from Crouzon syndrome with class I or II relationship a change from the classic frontofacial monobloc advancement leaving the maxillary dental arch in place, thus avoiding the worsening of the maxillo-mandibular dysmorphia related to surgery. The idea of associating Le Fort I osteotomy with a frontofacial monobloc advancement or Le Fort III osteotomy has already been described, mainly by Tessier and Obwegeser, however they probably achieved a complete Le Fort I osteotomy while we don't split the pterygoid plates.The patient's morphology and his surgical history determine the choice between Le Fort III and monobloc advancement. Dental occlusion needs to be taken into account for surgical indication.     

Mandible magnitude in Crouzon syndrome

The authors describe Crouzon syndrome in 5 patients aged 9, 10, 13, 40 and 43 years. Typical image of Crouzon syndrome has been found in the woman and three children and its abortive form--in the man. The authors present their own studies on branch height, length of the body of the mandible and the angle value of the mandible according to the Costaras-Volarich and Pruzancky method in their paper. The ratio of the branch to the body is 1:1 in all patients under examination and it is not equal to the normal ratio of 2:3. The angle of the mandible is also greater than the normal value. The authors put forward a hypothesis that pseudomesioclusion in the sick with Crouzon syndrome can be conditioned not only by maxilla dysplasia but also by excessive height of the branch and increased angle value of the mandible basing on their own observations. This hypothesis, however, exact further radiocephalometric studies and close cooperation of obstetricians, paediatricians and orthodontists.     

Cephalometric analysis of hard and soft tissues in a 12-year-old syndromic child: a case report and update on dentofacial features of Crouzon syndrome

Crouzon syndrome or craniofacial dysostosis is a rare syndrome characterized by craniosynostosis, midfacial hypoplasia and exophthalmia. The abnormalities found in this syndrome change too much from case to case depending on the suture fusion order. We report a case of a 12 year old child and a mother showing variations in the dentofacial tissues clinically and radiographically. Subsequently, the application of digital software [Dolphin Imaging 11] enabled us to solve out the case as Crouzon syndrome by analyzing the skeletal and soft tissue alterations. An update of the effects of this syndrome on various systems and dentofacial features with emphasis on tooth abnormalities is documented.     

Dental agenesis patterns in Crouzon syndrome

Dental agenesis may be present in an isolated familiar manner, or occur as a part of a syndrome.To date, this clinical trait seems to have been overlooked in patients with Crouzon syndrome.The aim of the present study was to investigate dental agenesis and dental agenesis patterns in a population of persons with Crouzon syndrome in Sweden. Serial panoramic radiographs of 26 individuals with Crouzon syndrome (20 males, 6 females) were examined.Third molars were excluded from the assessment. The prevalence of agenesis for at least one tooth was 42.3%. Each affected patient was found to have up to 5 missing teeth. Upper and lower second premolars were the most frequently congenitally missing teeth. Eleven dental agenesis patterns of the entire dentition were identified, as described by the tooth agenesis code (TAC). All patterns were unique and asymmetric,with only one exception, a symmetric pattern of the maxillary and mandibular second premolars. In conclusion, persons with Crouzon syndrome were found to have a high prevalence of dental agenesis and a remarkable variability of dental agenesis patterns. It is important to be aware of this clinical situation, especially when orthodontic treatment planning for these patients is performed as early as in the mixed dentition.     

Crouzon twins with cloverleaf skull malformations.

We describe unique monozygotic twins with Crouzon's disease and cloverleaf-shaped skull deformities who have been closely followed since birth. Their abnormal skull shapes were identified during antenatal ultrasound examination. The twins had gross exophthalmos and hydrocephalus with papilledema, so early calvarial decompression surgery was required. Although born to healthy parents of normal appearance, a third cousin of the twins had a milder form of Crouzon's disease, and there was a family history of high-arched palate on the twins' paternal side. Because Crouzon's disease is an uncommon condition and the cloverleaf skull shape is unusual in patients with Crouzon's disease, identical twins with this constellation of deformities must be exceptionally rare.     

Algorithm to the treatment of Crouzon syndrome

Clinical reports regarding the entire surgical sequence in Crouzon syndrome from the neonatal period to the adult age are rare. The purpose of this study is to trace an operative algorithm with a long term follow up in a homogenous group of patients affected by Crouzon syndrome.A retrospective review was conducted for all patients affected by Crouzon syndrome who completed the entire surgical sequence. 7 Crouzon patients (4 females, 3 males) completed the entire surgical sequence at different ages: fronto-orbital advancement (0.9 years), LF III distraction osteogenesis (11.5 years) and orthognathic surgery (18 years). The mean age at the last follow up was 19.3 years; normalization of the face was obtained in all cases with improvement of the respiratory problems. After orthognathic surgery, all patients had stable occlusion. A one-year postoperative CBCT scan revealed almost complete ossification of all osteotomy sites.Frontoorbital advancement and modified Le Fort III distraction osteogenesis are reliable surgical procedures. SARME and conventional orthognathic surgery with reductive genioplasty and fat grafting are performed at the end of the surgical sequence to enhance facial aesthetics.     

Multidisciplinary management of Crouzon syndrome.

The comprehensive management of Crouzon syndrome in a 14-year-old girl has been presented. Because of the complexity of the facial and associated problems, a multidisciplinary approach is necessary to provide maximum functional and esthetic results; however, with such a cooperative interdisciplinary effort, the improvement and benefits derived for these patients are rewarding to all concerned.     

Audiodontics-a case report

A case is presented of a young boy with bilateral conductive deafness secondary to bilateral microtia. His ability to hear by means of a bone conduction receiver held between the teeth is a practical demonstration of ‘audiodontics’.