Craniofacial disorders.

The complexity of the craniofacial patient mandates the cooperation of a multidisciplinary team that can systematically evaluate each individual and ensure that a protocol-driven pathway is undertaken for the best patient care. Oral and maxillofacial surgeons contribute to surgical care in this setting with specific knowledge of growth and development of the face. This enables optimum timing for early skeletal correction where appropriate, and definitive surgery following the cessation of growth to maximize function and aesthetics. This chapter will describe the major principles in managing patients with specific craniofacial anomalies and provide examples of the outcomes possible.

Carpal Coalitions and Metacarpal Synostoses: A Review.

Background: Carpal coalition and metacarpal synostosis are uncommon congenital anomalies of the carpus and hand. Methods: A comprehensive review of the literature was performed to help guide surgical and non-surgical treatment of carpal coalition and metacarpal synostosis. Results: The embryology, epidemiology, medical and surgical management, and associated outcomes are detailed. Conclusions: Most patients with these disorders will likely benefit from conservative measures. Surgery should be considered in patients with pain and limitations in wrist and hand function.

Successful Bone Healing of Nonunion After Ulnar Shortening Osteotomy for Smokers Treated With Teriparatide.

Ulnar shortening osteotomy is widely performed as the standard surgical treatment for ulnar impaction syndrome and has a high percentage of success for pain relief. However, delayed union and nonunion of the osteotomy site remain the most concerning complications. In particular, smokers have a higher incidence of nonunion, which amounts to 30% of cases. For the treatment of nonunion, secondary surgical interventions such as bone grafting will be necessary but are extremely challenging. Recently, teriparatide (recombinant human parathyroid hormone [PTH 1-34]) administration has been reported in several clinical studies as a noninvasive pharmacological systemic treatment for fracture healing or nonunion. The authors present 2 cases of smokers, a 62-year-old man and a 42-year-old woman, with nonunion after ulnar shortening osteotomy and fixation with 6-hole non-locking plate for ulnar impaction syndrome. For treatment of nonunion, noninvasive therapy with teriparatide (20-µg, subcutaneous injection) in addition to low-intensity pulsed ultrasound was underwent. In both cases, partial bone union began to be observed on radiographs after the first 4 weeks of teriparatide administration and successful bone healing without additional surgical interventions was achieved after 10 and 6 months of treatment with teriparatide, respectively. The current case reports showed that non-invasive combination therapy of teriparatide and low-intensity pulsed ultrasound were a possible alternative to surgical intervention. In the future, teriparatide therapy might be applied actively to patients who have risk factors for delayed union, such a heavy smoking habit, and are expected to experience nonunion after ulnar shortening osteotomy.

Inherited thrombocytopenias: an approach to diagnosis and management.

Inherited thrombocytopenias vary in their presentation, associated features, and molecular etiologies. An accurate diagnosis is important to provide appropriate therapy as well as counseling for the individual and their family members. As the genetic basis of more disorders is understood, it will be possible to diagnose a greater fraction of patients as well as learn more about the process of megakaryopoiesis and platelet production.

Management of posttraumatic radioulnar synostosis.

Posttraumatic radioulnar synostosis is a rare complication following fracture of the forearm and elbow. Risk factors for synostosis are related to the initial injury and surgical management of the fracture. Typically, patients present with complete loss of active and passive forearm pronation and supination. Evidence of bridging heterotopic bone between the radius and ulna can be seen on plain radiographs. Although nonsurgical management is sufficient in some cases, surgical excision is typically required. The timing of surgical intervention remains controversial. However, early resection between 6 and 12 months after the initial injury can be performed safely in patients with radiographic evidence of bony maturation. Surgical management consists of complete resection of the synostosis with optional interposition of biologic or synthetic materials to restore forearm rotation. A low recurrence rate can be achieved following primary radioulnar synostosis excision without the need for routine adjuvant prophylaxis.

Prevention of recurrent radioulnar heterotopic ossification by combined indomethacin and a dermal/silicone sheet implant: case report.

A 27-year-old, right-handed man developed severe radioulnar synostosis at the distal radius 7 months after open reduction and internal fixation of his distal radius fracture. Heterotopic ossification formed at the radius fracture site, requiring excision of the heterotopic bone and plate removal. A bilayer, dermal substitute and silicone sheet was placed between the radius and ulna in the interosseous space to prevent recurrence of the heterotopic ossification. After surgery, the patient was treated with indomethacin 25 mg orally 3 times daily for 6 weeks. At 1 year after surgery, he has retained full pronation and near normal supination. Radiographs demonstrate no new heterotopic bone formation.

A neonate with severe thrombocytopenia and radio-ulnar synostosis.

Bone marrow failure syndromes can be associated with abnormalities of the forearms. We observed a neonate with congenital thrombocytopenia who had bilateral radio-ulnar synostosis and fifth finger clinodactly. We performed an evaluation of the mechanism causing the thrombocytopenia using a combination of direct and indirect measures of thrombopoiesis. These tests indicated decreased platelet production. This entity of congenital hyporegenerative thrombocytopenia with bilateral radio-ulnar synostosis and fifth-finger clinodactly is an uncommon but easily recognizable form of congenital amegakaryocytic thrombocytopenia (CAMT). This entity can be distinguished from the TAR syndrome (thrombocytopenia and absent radii) by the distinctive orthopedic issues, different underlying genetic mutations, and a more worrisome prognosis for CAMT than for TAR.

Occipital plagiocephaly.

The diagnosis of occipital plagiocephaly has remained a complex and controversial issue in the field of craniofacial surgery. Over the past 30 years, numerous studies have been published describing the management and treatment for 'posterior plagiocephaly', 'plagiocephaly without synostosis', 'deformational plagiocephaly' and 'occipital plagiocephaly', with surgical 'correction' being chosen as the primary modality of treatment irrespective of the patency status of the lambdoid sutures. Two hundred and four patients with unilateral occipital plagiocephaly have been seen at the Australian Craniofacial Unit over the past 16 years. Each patient was evaluated by a craniofacial surgeon, paediatric neurosurgeon and paediatric geneticist. All children underwent plain radiographs of the skull to define the sutural anatomy. In those patients where the sutural anatomy was equivocal, 2-D and 3-D CT scans were performed. Only two of the 204 patients (approximately 1%) manifested the clinical, radiographic and pathological features of true unilambdoid synostosis. There was radiographic evidence of sutural fusion on plain films, 2-D and 3-D CT scans. Pathology specimens showed bony sutural fusion. Two hundred and two patients presented with unilateral occipital deformities and patent sutures on radiography. These patients with occipital plagiocephaly in the absence of true synostosis were initially managed conservatively (head positioning, and physiotherapy in those patients with torticollis). Those patients who underwent surgical correction in infancy (21/204) included patients with severe plagiocephaly not responding to conservative therapy (19/204) and the two patients with true unilambdoid synostosis (2/204).One hundred and ninety-one of the total patients (94%) were noted by their parents to have acceptable improvement in their head shape. Thirteen patients were seen within the past year and are too early to assess. Two surgical patients (one fronto-orbital advancement, one occipital craniectomy) and one patient followed conservatively were judged by their parents to be without notable improvement. In our series it is apparent that the majority of cases of occipital plagiocephaly are not secondary to true synostosis and can be managed by conservative positional measures.

[Congenital tarsal synostosis]. / Les synostoses congénitales du tarse.

Tarsal coalition results from the fusion of at least two tarsal bones. The clinical and radiological pictures depend on the anatomic location of the coalition. Calcaneonavicular and talonavicular coalitions are the most frequent features which may result in peroneal spastic flat foot; however they are symptomless in most cases. Tarsal coalitions are usually evident on plain X-ray films using appropriate incidences. In selected cases, partial talocalcaneal coalition requires CT scan or MRI. No treatment is needed for asymptomatic conditions. Painful spastic flat foot must be treated conservatively by limiting sport activity as a first step treatment. In case of persistent symptoms, cast with correction of deformities under general anesthaesia is recommended. Surgery is needed only in case of failure of previous treatment, requiring resection of the bony bridge and sometimes triple foot arthrodesis.

Management of antebrachial deformity and shortening secondary to a synostosis in a dog.

A nine-week-old labrador was presented with a deformed left forelimb causing obvious lameness. Radiographs suggested that the dog had suffered a fracture involving the radius and ulna which had healed resulting in a synostosis. There was a concurrent radial physeal injury. The dog was treated initially by osteotomy of the radius and ulna stabilised using a static external skeletal fixator. At a subsequent surgery lengthening of the limb was achieved by using an Ilizarov apparatus. Worsening carpal hyperflexion was managed by bandaging in forced extension. A successful outcome was achieved with a final result of a 24 per cent discrepancy in length between the two radii at 17 months of age. An increase in length of the ipsilateral humerus was seen in association with the shortened forelimb.

[Carpal bone protuberance]. / Wynioslosc kostna nadgrastka.

The term "carpe bossu" has been coined to bony eminence at the base of II or III metatcarpal, sometimes expanding to the trapezoid and capitate dorsally. The condition is usually symptom free. Pain is spontaneous or elicited by overuse and aggravated by palmar flexion of the wrist. The subluxation of extensors tendons overlying the boss may occur in more cases. Bony eminence, regular in shape is evident on lateral radiographs. Conservative treatment usually renders remission of symptoms but if it fails surgery is indicated.

Treatment of traumatic radioulnar synostosis by excision and postoperative low-dose irradiation.

Post-traumatic radioulnar synostosis can have a profound effect on upper extremity function. Prior reports of excision, with and without interposition material, have demonstrated frequent recurrence and disappointing results. Based on a favorable experience with radiation prophylaxis of heterotopic ossification following total hip arthroplasty, this modality has been used in the management of post-traumatic forearm synostosis. Four cases using excision of bony synostosis followed by single-fraction, low-dose (800 cGy), limited-field irradiation are presented. With a follow-up period of 1-4 years after excision and irradiation, all four patients had total arcs of forearm rotation between 115 degrees and 120 degrees. Each patient noted sustained functional improvement, and there was no x-ray film evidence of recurrent synostosis formation. Single fraction irradiation did not require ongoing patient compliance nor did it complicate rehabilitative efforts. Furthermore, soft tissue and bony healing were not impaired.

Tarsal coalitions: an instructional review.

Tarsal coalitions, the majority of which are congenital, are abnormal unions between two or more tarsal bones. Acquired tarsal coalitions may result from trauma or from rheumatic conditions. Tarsal coalition may cause peroneal spastic flatfoot or rigid valgus foot without peroneal spasm. Surgery is not always essential in the treatment of tarsal coalitions; some patients will respond to conservative treatment.