Cases reported "Facial Asymmetry"

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1/52. Simultaneous distraction osteogenesis and microsurgical reconstruction for facial asymmetry.

    Restoring facial balance in patients with severe facial asymmetry is a challenging problem for the craniofacial team. attention to bony reconstruction as well as soft-tissue contouring is required for patients with moderate to severe deformities. Traditionally, facial skeletal reconstruction was performed with osteotomies and bone grafting. More recently, distraction osteogenesis has proven to be successful in achieving bone lengthening. For select cases, distraction osteogenesis has lessened the need for major skeletal procedures and has allowed earlier surgical intervention. The reconstruction of the soft tissues in facial asymmetry has generally been performed as a second-stage procedure after skeletal reconstruction. The disadvantage of these traditional approaches is that it requires two separate major operative procedures, with the accompanying increased morbidity, hospital stay, and cost. We present a patient with hemifacial microsomia and a grade III mandibular deformity, in whom both the hard- and soft-tissue deficiencies were corrected in one surgical procedure with mandibular distraction osteogenesis and soft-tissue augmentation with a vascularized parascapular osteocutaneous flap. The technique and results at 1-year follow-up are presented.
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2/52. anesthesia for Freeman-Sheldon syndrome using a laryngeal mask airway.

    PURPOSE: To present a case of Freeman-Sheldon syndrome (FSS) with a previously unreported technique of anesthetic management, consisting of a malignant hyperthermia free anesthetic and laryngeal mask airway. CLINICAL FEATURES: Freeman-Sheldon syndrome (also known as whistling face syndrome, Windmill-Vane-hand syndrome, cranio-carpo-tarsal dysplasia and distal arthrogryposis type 2) is a rare congenital disorder defined by facial and skeletal abnormalities. The three basic abnormalities are microstomia with pouting lips, camptodactyly with ulnar deviation of the fingers and talipes equinovarus. patients with FSS frequently present for surgical correction of musculoskeletal or facial abnormalities. There are several anesthetic challenges including difficult airway, intravenous cannulation and regional technique. They may be at increased risk for malignant hyperthermia and postoperative pulmonary complications. We present a case of a two-year-old child with FSS undergoing elective unilateral inguinal hernia repair. A non-triggering anesthetic technique was used, consisting of 2 mg x kg(-1) propofol followed by a continuous infusion, nitrous oxide 50%/oxygen, and 3 microg x kg(-1) fentanyl. Intraoperative and postoperative analgesia was provided by an ilioinguinal nerve block with 10 ml bupivacaine 0.25% with epinephrine 1:200,000. The airway was maintained with a #2 laryngeal mask airway. The anesthetic was uneventful and there were no signs or symptoms of malignant hyperthermia. The patient was discharged home later the same day in good health. CONCLUSION: The use of a laryngeal mask airway and non-triggering anesthetic technique should be considered as options for anesthetic management in patients with FSS for short procedures that do not require neuromuscular blockade.
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3/52. Two-year follow-up of distraction osteogenesis: its effect on mandibular ramus height in hemifacial microsomia.

    Distraction osteogenesis has been used to lengthen the mandible in patients with hemifacial microsomia. Questions regarding soft tissue and skeletal growth after distraction osteogenesis have not been clearly elucidated in the literature. In this case report, a 2-year follow-up of distraction osteogenesis in a 7 year old boy is documented with lateral and posterior/anterior cephalometric analysis. The analysis was performed preoperatively and at specific postsurgical periods to evaluate the facial soft tissue and skeletal growth patterns. Objective analysis of this growing patient after distraction osteogenesis clearly demonstrates that the anteroposterior elongation of the mandible is relatively stable, whereas the vertical lengthening and soft tissue effects are minimal. Critical evaluation of other patients who have undergone distraction osteogenesis is needed to determine if this was an isolated incident or the expected result in similar patients.
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4/52. Unilateral coronal synostosis treated by internal forehead distraction.

    A 1-year-old infant with left hemicoronal synostosis was treated by distraction osteogenesis of the craniofacial skeleton using an internal distraction device. Surgery was performed through a coronal incision. The frontal bone and upper half of both orbits were first osteotomized en bloc after minimal epidural dissection of the supraorbital area and no epidural dissection around the coronal osteotomy site. The lateral one fourth of the frontal bone, including the right lateral half of the orbit, was left intact. The internal distraction device was fixed in the left temporal area. A 0.5-mm per day rate of distraction was performed up to an elongation of 17 mm after a 5-day latency period. The distraction device was removed after a consolidation period of 2 months. The results obtained were satisfactory, with symmetry of the forehead, orbit, and nose achieved without complications. The merits of this procedure are no extradural dead space after the operation (which prevents infection), shortened operative time, reduced blood loss, filling in the bone gap created by advancement with new bone, acceptable cosmesis by the parents during distraction, and no fixation device left after the second operation.
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5/52. Distraction osteogenesis of the ascending ramus for mandibular hypoplasia using extraoral or intraoral devices: a report of 8 cases.

    PURPOSE: This report presents the results of distraction osteogenesis using unidirectional extraoral and intraoral devices in 8 patients with different grades of vertical mandibular ramus hypoplasia. patients AND methods: Eight patients with hypoplastic mandibles underwent unilateral lengthening of the ascending ramus using unidirectional extraoral or intraoral devices. Intraoral mandibular distraction was performed on 5 patients with deficiencies of the vertical ramus up to 24 mm. External devices were used in 3 patients with more severe hypoplasias. An intraoral osteotomy was performed, and progressive distraction at rates of 0.5 mm/12 hours was initiated after 5 days. Once the desired length was reached, the device was maintained in place for 8 to 12 weeks. Three-dimensional computed tomography scans were taken in all the patients to plan the procedure and to compare the changes postoperatively. RESULTS: Successful distraction osteogenesis was achieved in all patients. The amount of mandibular lengthening ranged from 17 to 32 mm. Complications with the external devices such as rotation of the proximal bony fragment (2 cases) and loosening of the external screws at the end of the consolidation period (1 case) were observed. CONCLUSIONS: The results suggest that the intraoral device can be used as the method of choice for distraction osteogenesis of the ascending ramus of the mandible in patients with large deficiencies. Preoperative and postoperative 3-dimensional computed tomographic scans are essential in treatment planning.
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ranking = 3
keywords = operative
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6/52. Perioperative management of infants with the linear naevus sebaceous syndrome of Jadassohn: a report of two cases.

    Neurofibromatosis and tuberous sclerosis are the most well-recognized of the congential phakomatoses, a group of six hereditary neuro-oculo-cutaneous disorders. Although easily diagnosed at birth by a parasagittal line of facial sebaceous naevi, the linear naevus sebaceous syndrome (LNSS) of Jadassohn is the rarest phakomatosis, one often characterized by airway and anaesthetic considerations that do not apply to the other phakomatoses. In addition to its obvious cutaneous manifestations, LNSS is characterized by hemifacial asymmetry, an anatomic predictor of difficult trachael intubation, and intractable seizure activity, a condition that limits selection of anaesthetics. The perioperative management challenges of LNSS are depicted in the presentation of two cases of LNSS with different outcomes and contrasted with the major anaesthetic considerations in the perioperative management of other, more common phakomatoses.
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ranking = 6
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7/52. Distraction lengthening of the mandibular costochondral graft: a precautionary note.

    Distraction osteogenesis of bone-grafted mandibles has been shown to occur without undue difficulties in bone regeneration and healing. In the patient with hemifacial microsomia for which early costochondral graft reconstruction has been done, secondary distraction lengthening may still be needed eventually. It is important to assess preoperatively that the superior portion of the graft is properly positioned against the residual glenoid fossa to prevent excessive superior movement of the proximal mandibular segment during distraction. The case presented clearly illustrates this unique distraction phenomenon.
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8/52. One-stage facial augmentation with an intraoral groin adipose flap transfer.

    A free groin adipose flap using an intraoral approach was used to correct facial contour deformities instead of a groin dermis-fat flap. The major disadvantages of the latter flap are that multistage debulking procedures are required and there are wide postoperative donor scars because of the wide skin portion included in the flap. To overcome these weaknesses the authors developed a free groin, customized ("berry picked") adipose flap, which was transferred by an intraoral approach for reconstruction of congenital hemifacial (orbitozygomatic) hypoplasia. The advantages of this method include one-stage augmentation without secondary defatting, no skin grafting of the donor defect, a donor scar in a concealed area, and possible transfer through an intraoral approach that results in minimal invasive surgery with no scar at the graft site.
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9/52. Clinical application of injectable calcium sulfate on early bony consolidation in distraction osteogenesis for the treatment of craniofacial microsomia.

    The purpose of this project was to study the effect of calcium sulfate on early bony consolidation in distraction osteogenesis. A total of eight patients with craniofacial microsomia were treated between April 2000 and February 2001. The age of the patients ranged from 2 to 40 years, and all were male. The follow-up period was 1 to 15 months. The operative procedure in adults was based on Ortiz Monasterio's simultaneous mandibular and maxillary distraction technique. In children, osteotomy of the mandible was performed for distraction. On the fifth day after the operation, distraction was performed at a rate of 1 mm/d in three adults. In children, distraction was performed at a rate of 1 mm/d in one patient and 2 mm/d in four patients without a latent period. On the day of completion of distraction, calcium sulfate was implanted into the distracted zone. Radiographs showed bony consolidation at 4 weeks in one child at a rate of 1 mm/d, at 5 to 7 weeks in four children at a rate of 2 mm/d, and at 5 weeks in three adults after implantation of the calcium sulfate. In conclusion, these findings suggest that calcium sulfate is effective for early bony consolidation in distraction osteogenesis and that it would shorten the whole treatment period.
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10/52. Mandibular distraction in the absence of an ascending ramus and condyle.

    The introduction of distraction osteogenesis (DO) as a method of mandibular elongation has provided the craniofacial surgeon with a valuable tool in the approach to patients with mandibular deficiencies. A growing number of case reports and clinical series have proven the efficacy of mandibular DO as an augmentative technique in patients with hemifacial microsomia (HFM). Although DO has become a part of the treatment algorithm for many patients with HFM, surgeons have been reluctant to apply the technique in patients with complete agenesis of the ascending mandibular ramus and condyle (grade III HFM). In the following cases, mandibular DO was successfully performed in two patients with grade III HFM. Preoperative images and intraoperative findings confirmed that both patients lacked the entire ascending mandibular ramus and condyle on the affected side. After unilateral multiplanar DO, both patients showed new bone formation within the distraction gap and development of a pseudoarthrosis between the proximal segment of the mandible and the skull base. Facial symmetry improved dramatically, and speech and mandibular excursion were maintained. The patients have been followed for 1 to 2 years after distraction with durable functional and esthetic outcomes. Mandibular DO offers many advantages compared with rib grafting, including avoidance of donor site morbidity, application in patients who are not candidates for graft harvest, and use after rib graft failure. The following cases support the use of mandibular DO as a mechanism of endogenous tissue engineering in patients with complete agenesis of the ascending mandibular ramus and condyle.
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