The use of computer-aided design/manufacturing (CAD/CAM) technology to aid in the reconstruction of congenitally deficient pediatric mandibles: A case series.

BACKGROUND: Microvascular reconstruction of the pediatric mandible, particularly when necessitated by severe, congenital hypoplasia, presents a formidable challenge. Complex cases, however, may be simplified by computer-aided design/computer-aided manufacturing (CAD/CAM) assisted surgical planning. This series represents the senior authors' preliminary experiences with CAD/CAM assisted, microvascular reconstruction of the pediatric mandible. METHODS: Presented are two patients with hemifacial/bifacial microsomia, both with profound mandibular hypoplasia, who underwent CAD/CAM assisted reconstruction of their mandibles with vascularized fibula flaps. Surgical techniques, CAD/CAM routines employed, complications, and long-term outcomes are reported. RESULTS: Successful mandibular reconstructions were achieved in both patients with centralization of their native mandibles and augmentation of deficient mandibular subunits. No long-term complications were observed. CONCLUSIONS: CAD/CAM technology can be utilized in pediatric mandibular reconstruction, and is particularly beneficial in cases of profound, congenital hypoplasia requiring extensive, multi-planar, bony reconstructions.

Complications of mandibular distraction osteogenesis for congenital deformities: a systematic review of the literature and proposal of a new classification for complications.

A systematic review of English and non-English language articles on the complications of mandibular distraction osteogenesis (MDO) for patients with congenital deformities was performed, in accordance with the PRISMA statement. Search terms expressing distraction osteogenesis were used in 'AND' combination with search terms comprising 'mandible' and terms for complication, failure, and morbidity. A search using PubMed (National Library of Medicine, NCBI), EMBASE, and the Cochrane Central Register of Controlled Trials yielded 644 articles published between 1966 and mid October 2013. Clinical articles that reported complications related to MDO were included. Finally 81 articles on MDO in congenital deformities were eligible and were screened in detail. Complications including minor infection (6.0%), device-related problems (7.3%), skeletal open bite (2.4%), hypertrophic scar formation (2.1%), facial nerve palsy (1.8%), neurosensory disturbances of the inferior alveolar nerve (1.9%), and (fibrous) non-union (0.7%) were seen. A new index for more detailed classification of complications in MDO is proposed based on six categories that indicate the impact of the complication and its further treatment or final results. The proposed complication index may be a useful tool to classify complications related to MDO.

Reconstruction of mandibular defects - clinical retrospective research over a 10-year period -.

BACKGROUND: Functional and cosmetic defects in the maxillofacial region are caused by various ailments and these defects are addressed according to their need. Simplicity of procedure, intact facial function and esthetic outcome with the least possible donor site morbidity are the minimum requirements of a good reconstruction. Oro-mandibular reconstruction, although a challenge for the head and neck reconstructive surgeon, is now reliable and highly successful with excellent long-term functional and aesthetic outcomes with the use of autogenous bone grafts. Reconstruction of trauma- or mandibular oncologic defects with bony free flaps is considered the gold standard. However the the optimal reconstruction of mandibular defects is still controversial in regards to reconstructive options which include the donor site selection and the timing of surgery. The purpose of this study was to determine the outcome of different osseous reconstruction options using autogenous bone grafts for mandibular reconstructions. METHODS: This study was carried out on 178 patients with mandibular bone defects. They were reconstructed with autogenous bone grafts from different donor sites. At post operative visits they were evaluated for functional and cosmetic results. RESULTS: The success rate found in this study was around 90%. Only 7.6% of the cases showed poor results regarding facial contours and mouth opening. All other patients were satisfied with their cosmesis and mouth opening at the recipient sites was in the normal range during last follow-up visits. Donor sites were primarily closed in all cases and there was no hypertrophic scar. CONCLUSION: Based on this study, autogenous bone grafts are a reliable treatment modality for the reconstruction of mandibular bone defects with predictable aesthetic and functional outcomes. As the free vascularized fibular flap has the least resorption and failure rate, it should be the first choice for most cases of mandiblular reconstruction.

Early surgical treatment in unilateral coronoid hyperplasia and facial asymmetry.

Unilateral coronoid hyperplasia is a rare condition in the pediatric age. It may be an unrecognized cause of restricted mouth opening in children.The limited jaw movement is due to the enlargement of the coronoid process of the mandible that impinges on the zygomatic arch during mouth opening. This pathologic condition is still unknown and often misdiagnosed.Although in the past the term osteochondroma has been used to describe most of the unilateral and a few of the bilateral cases, there is no histologic evidence that the process has a neoplastic origin.Microscopic examination of the removed coronoid process has revealed hyperplastic compact bone covered with a thin layer of normal cartilage.There are multiple causes of mandibular hypomobility, each of them associated with different anatomic structures and etiologies, and a large number of cases, mostly bilateral, are idiopathic in nature.Several theories of pathogenesis have been proposed: temporomandibular joint dysfunctions, mandibular hypomobility, temporalis hyperactivity, hormonal stimulus, persistent cartilage growth center, genetic inheritance, and family factors.Unilateral coronoid hyperplasia is usually due to a trauma or a pathologic condition and is associated with facial asymmetry, being more frequently seen in women with histologic chondromatous or neoplastic changes. A thorough clinical history should include information about the onset and progression of pain and other subjective symptoms.In this study, we present a case of unilateral hyperplasia of the coronoid process in a 3 year-old female who, to the best of our knowledge, is the youngest patient so far reported with such anomaly.Our findings support the recommendation that early surgical treatment and aggressive postoperative physical therapy should be taken into account to allow for recovery of morphology and growth function in children.

Mandibuloacral dysplasia type A in childhood.

Mandibuloacral dysplasia type A (MADA) is characterized by growth retardation, postnatal onset of craniofacial anomalies with mandibular hypoplasia, progressive acral osteolysis, and skin changes including mottled pigmentation, skin atrophy, and lipodystrophy. Owing to its slowly progressive course, the syndrome has been recognized in adults, and pediatric case reports are scarce. We present the clinical case of two children in whom the diagnosis of MADA was made at an unusually early age. A 5-year-old boy presented with ocular proptosis, thin nose, and short and bulbous distal phalanges of fingers. A 4-year-old girl presented with round face and chubby cheeks, thin nose, bulbous fingertips, and type A lipodystrophy. In both, a skeletal survey showed wormian bones, thin clavicles, short distal phalanges of fingers and toes with acro-osteolysis. Both children were found to be homozygous for the recurrent missense mutation, c.1580G>A, (p.R527H) in exon 9 of the LMNA gene. Thus, the phenotype of MADA can be manifest in preschool age; diagnosis may be suggested by short and bulbous fingertips, facial features, and lipodystrophy, supported by the finding of acral osteolysis, and confirmed by mutation analysis.

Meta-analysis of mandibular distraction osteogenesis: clinical applications and functional outcomes.

BACKGROUND: Mandibular distraction osteogenesis has been used effectively to treat syndromic craniofacial deformities. In recent years, its scope of application has widened to include treatment of airway obstruction in adults and children and nonsyndromic class II mandibular hypoplasia. So far, there has been no evidence-based review of mandibular distraction osteogenesis for mandibular lengthening. METHODS: Two rounds of searches were performed by two independent assessors. The first-round PubMed search used the keywords "mandible" and "distraction osteogenesis." In the second-round search, the reference lists of the articles were retrieved. For both rounds, abstracts and then full articles were reviewed and selected on the basis of a set of inclusion and exclusion criteria. RESULTS: The 178 retrieved articles yielded 1185 mandibular distraction osteogenesis patients: 539 received unilateral mandibular distraction osteogenesis and 646 received bilateral mandibular distraction osteogenesis. Mandibular distraction osteogenesis was reported to improve facial asymmetry and retrognathia (50.1 percent), correct the slanted lip commissure (24.7 percent), and improve or level the mandibular occlusal plane (11.1 percent) in unilateral asymmetry cases, whereas bilateral mandibular distraction osteogenesis was shown to be effective in preventing tracheostomies for 91.3 percent of neonates or infants with respiratory distress, and in relieving symptoms of obstructive sleep apnea for 97.0 percent of children and 100 percent of adult patients. CONCLUSIONS: Mandibular distraction osteogenesis is effective in treating craniofacial deformities, but further clinical trials are required to assess the long-term stability and to compare the treatment with conventional treatment methods, especially in cases of obstructive sleep apnea or class II mandibular hypoplasia.

The osseous genioplasty.

Despite its existence for nearly half a century and its versatility in solving a complex range of chin deformities, osseous genioplasty through an intraoral approach remains a rusty tool in many surgeons' armamentarium. The osseous genioplasty is not solely within the domain of the maxillofacial or craniofacial surgeon; it is well within the reach of any surgeon whose practice involves facial aesthetics. The surgeon who masters this relatively simple procedure can solve a broad range of chin deformities that an implant cannot solve: a chin that is too long, too short, or asymmetric.

Basic guidelines for the surgical correction of mandibular anteroposterior deficiency and excess.

During the past decades, knowledge and understanding of all aspects of orthognathic surgery have increased greatly. Diagnostic skills and treatment planning have become more sophisticated and, through experience, surgical techniques have attained a level enabling the treatment of the most complex jaw deformities with confidence. In this article, guidelines for the treatment of mandibular anteroposterior dentofacial deformities are discussed. It should, however, always be kept in mind that the face and mouth are complex, three-dimensional structures and multifunctional in character. An artistic flair and the ability to think originally have become essential for the orthognathic surgeon, because no two dentofacial deformities are the same.

Distraction osteogenesis in the pediatric population.

OBJECTIVES: Distraction osteogenesis has been described routinely in the mandible for the advancement of bony segments. Complications, though infrequent, may include postoperative infection, implant extrusion, nonunion of the bony segments, device malfunction, cranial nerve paresis, and premature consolidation. METHODS: Seventy-eight distractions of the mandible were performed over 10 years. The technique in placement of these internal microdistraction devices involves making intraoral and extraoral incisions and beginning distraction after a latency period of 3 days. Following this latency period, distraction occurs at 1 mm/day. RESULTS: In this series of patients, distraction was accomplished successfully. There was a 2.6% rate of wound infection in this series; 2.6% also had bony nonunion; 3.8% had premature bony consolidation; and 3.8% had facial nerve complications. In the three patients with facial nerve paresis, we followed the facial nerve clinically and each paresis resolved within 6 months. CONCLUSIONS: Distraction of the mandible may be accomplished in the pediatric population. As with any intervention, inherent perioperative complications may still arise.

Distraction rate and latency: factors in the outcome of pediatric mandibular distraction.

BACKGROUND: Distraction osteogenesis was initially devised as a way to lengthen the lower extremity. All the basic tenets of the technique were developed based on this use. This includes both the supposed need for a latency period before distraction begins (3 to 5 days) and the ideal rate of distraction (1 mm/day). There is no evidence that these values apply to craniofacial distraction osteogenesis, particularly in children. Indeed, with the markedly improved blood supply in the face when compared with the lower extremity and the use of the technique in children versus adults, one might assume that a shorter latency period and a more rapid rate of distraction are possible. METHODS: This study is a retrospective review of 22 pediatric patients with mandibular hypoplasia undergoing mandibular distraction osteogenesis. All patients were operated on by a team consisting of an oral surgeon and a plastic surgeon. All the distraction devices used were external. All patients had a latency period, before activation, of less than 24 hours and a distraction rate of 2 mm/day. RESULTS: Two patients developed cellulitis at the pin sites, one patient developed premature healing, one patient developed a nonunion that required further surgery. This was the only patient in the series who had distraction of a previous bone graft. No patient undergoing distraction of native mandible developed a nonunion. CONCLUSIONS: Eliminating the latency period and rapidly distracting the mandible in pediatric cases has an acceptably low complication rate. This benefits the patient in terms of an overall reduction in the amount of time that the child needs to remain in the distraction device. The patient spends less time with the distraction device, thus reducing the potential morbidity and increased cost of the treatment. Potential explanations for why this variation in distraction technique is successful include the improved blood supply in the face of children and the failure of external devices to translate all the distracted movements to the bone interface. Some of this force is translated into both bending of the pins and migration of the pins through bone. Although a latency period might be necessary in the distraction of the lower extremities, the application of this concept to the craniofacial skeleton in children is erroneous.

Syngnathia and Van der Woude syndrome: a case report and literature review.

OBJECTIVE: Syngnathia is a rare anomaly involving soft tissue or bony adhesions between the maxilla and mandible. We review the literature and present a case of syngnathia associated with Van der Woude syndrome. Syngnathia can have very different etiologies, but this has rarely been reported in Van der Woude syndrome. Treatment of this condition is rarely discussed in the literature because of a paucity of case reports. RESULTS: Oral adhesion (syngnathia) in our patient was caused by a mandibular to maxillary fibrous band. Surgical treatment was successful. Clinical implications and review of literature for the treatment of this rare association of syngnathia and Van der Woude syndrome are discussed.

Tuberous sclerosis: clinicopathologic features and review of the literature.

INTRODUCTION: Tuberous sclerosis is a hamartoneoplastic syndrome, which may involve multiple organ systems. Oral hard tissue manifestations of the syndrome have been described in the literature only as recently as 1955. Patients who presented with clinical manifestations of tuberous sclerosis did not routinely undergo oral surveys to rule out 'lesions', and consequently data on 'lesions' in the maxillofacial complex is scant. Ten cases have been found in the English language literature, which describe maxillofacial 'lesions', which may be tumours, new growths, neoplasms or overgrowths occurring in patients diagnosed with tuberous sclerosis. PURPOSE: To review the literature for all maxillofacial lesions associated with tuberous sclerosis and to present an eleventh case of a patient with a maxillofacial lesion diagnosed as having tuberous sclerosis. RESULTS: Eleven cases were found with maxillofacial fibroblastic lesions associated with tuberous sclerosis. These lesions were all fibrous benign neoplasms found in the maxillofacial bony complex. CONCLUSIONS: Maxillofacial fibroblastic lesions in tuberous sclerosis have various histopathological presentations, some of which may be difficult to differentiate. Consequently, close microscopic examination of these lesions is necessary so that adequate surgical treatment is provided.

[The results of using a compression-distraction method for eliminating mandibular defects and deformities]. / Rezul'taty ispol'zovaniia komressionno-distraktsionnogo metoda dlia ustraneniia defektov i deformatsii nizhnei cheliusti.

Intraoral supraosseous compression-distraction devices (CDA) have been used for repair of mandibular underdevelopment and defects of different etiology at Central Institute of Stomatology since 1997. The distraction regenerates have been examined by ultrasonography, roentgenography, and echo-osteometry. These methods allow timely correction of the course of distraction and rule out formation of wrong hypotrophic distraction regenerates.

[Congenital infiltrating lipomatosis of the face. Presentation of a new clinical case]. / Lipomatose congénitale infiltrante de la face. Présentation d'un nouveau cas clinique.

A new case of this rarely described pathology is reported. It concerns a benign infiltrating lipomatosis, strictly located to the face. However, dento-musculo-squelettal structures are constantly involved, usually located to a hemiface. A young female patient has been followed up from her birth. Surgical procedures are reported. Diagnostic and therapeutic difficulties of this rare clinicopathologic entity are discussed.

[A case of facial fibrous dysplasia in brothers]. / A propos d'un cas de dysplasie fibreuse faciale dans une fratrie.

Fibrous dysplasia is a benign bone tumor. The cranio-facial localization concern 20% of fibrous dysplasia. A congenital etiology is evoked by all the authors. Our observations ask for the real etiology of this affection: Congenital? Familial? The diagnosis is done by anatomo-pathology, and surgery is the treatment for severe cases. Relapses are frequent.

Incidence of maxillofacial involvement in arthrogryposis multiplex congenita.

PURPOSE: This study determined the incidence of maxillofacial involvement in patients diagnosed with arthrogryposis multiplex congenita (AMC). PATIENTS AND METHODS: Twenty-three patients were evaluated by the pediatric physical medicine and rehabilitation, orthopedic surgery, and pediatric oral and maxillofacial surgery departments. Any patient in whom the diagnosis of AMC was in doubt was excluded from the study. All patients with limited mandibular function underwent computed tomography (CT) examination of their temporomandibular joints (TMJ). The results of physical therapy were followed. RESULTS: Five of the 23 patients diagnosed with AMC were found to have maxillofacial involvement, eg, presence of cleft palate, Robin-like sequence, high-arched palate, open-bite deformity, facial muscle weakness, esophageal dysfunction, and limited mandibular opening. No TMJ abnormalities were found by CT scan. Physical therapy was used for treatment of the limited opening, but relapse occurred quicky after therapy was discontinued. CONCLUSION: The incidence of maxillofacial findings is similar to that of most other reports. Treatment involves surgical correction of abnormal anatomy when possible (ie, cleft repair), symptomatic management (ie, esophageal dysfunction), and physical therapy.

Syngnathia: a report of two cases.

Syngnathia is a rare anomaly involving soft tissue or bony adhesions between the maxilla and mandible. Two cases of congenital syngnathia secondary to interalveolar soft tissue synechia are presented. These children present problems with airway protection and feeding in the immediate neonatal period. Most have multiple associated anomalies requiring concurrent management. Less than 50 cases have been described, most commonly in association with popliteal pterygium syndrome. Surgical management involves division of the adhesions in the first few days of life. Depending on the severity, these patients may present formidable anesthetic challenges. The prognosis is generally favorable in cases of soft tissue adhesions with eventual restoration of normal mandibular motion and function.