Frontofacial Monobloc Advancement Research Articles

Blindness as a Complication of Monobloc Frontofacial Advancement With Distraction

The monobloc frontofacial osteotomy provides aesthetic and functional improvement in the treatment of various craniofacial deformities. This procedure, through highly complex, has had some significant associated complication, such as cerebrospinal fluid leakage, hematoma, infection, and bone resorption. Distraction has been successfully used to gradually elongate bone and soft tissue. This method seems to provide improved results over conventional surgery, with less morbidity. We present a case of a patient with Apert syndrome who underwent monobloc advancement using the Rigid External Device (RED) device and who developed a transient bilateral amaurosis on the fourth postoperative day before distraction. A second procedure was performed to push back the frontal bandeau, maintaining the device in position. The blindness was resolved with this procedure as well as treatment with systemic steroids. The distraction was started thereafter, and the desired improvement was acquired. To our knowledge, this is the first case of transient bilateral amaurosis in a patient undergoing monobloc distraction.

Monobloc distraction in an infant, using the rigid external distractor: Problems and solutions—A case report

Patients with craniofacial dysostosis frequently develop functional problems including raised intracranial pressure, ocular dysfunction, obstructive sleep apnoeas and failure to thrive. These functional problems can be treated by a number of different techniques. The monobloc frontofacial advancement has the ability to correct all of these functional problems in one procedure, but can be associated with high morbidity particularly in the young infant. A case report of a 4-month-old infant with Pfeiffers syndrome and severe functional problems treated by monobloc osteotomy and distraction using the rigid external distractor is reported. The management, problems and complications encountered with this patient are discussed together with the role of monobloc distraction in the infant. Adaptation of the rigid external distraction (RED) technique using two titanium mesh sheets to prevent penetration of the skull by the cranial pins is described. Monobloc frontofacial advancement in the very young can be successfully achieved using the RED frame, thus treating exophthalmus, raised intracranial pressure and upper airway problems in one operation. However, it may be associated with significant complications and should only be used for those extreme cases where the severity of the functional problems prevent treatment being delayed.

Palpebral Fissure Changes After Monobloc Frontofacial Advancement in Faciocraniosynostosis

There is virtually no literature on the effect of correction of syndromal faciocraniosynostosis with monobloc advancement on the palpebral fissure shape. Using image processing software, we measured the effect of monobloc advancement on the position of the upper and lower eyelids as well as the palpebral fissure slant in a series of 18 patients with syndromal faciocraniosynostosis who had undergone surgery for correction of orbital and midface hypoplasia. For both eyes of each patient, 3 variables were measured on the pre- and postoperative photographs: the linear distances between the upper and lower eyelid margins, the pupil center and the angle between the inner and outer canthi. The globe protrusion was also measured on axial computed tomography scans before and after surgery. The results indicate that the exorbitism reduction induced by monobloc advancement is accompanied by a diminution of the distance between both eyelids and the pupil center. However, the downward slant of the palpebral fissure is increased after surgery. The data suggest that the lower and upper eyelid retraction seen preoperatively in the majority of patients with faciocraniosynostosis tend to be corrected when the frontofacial region is advanced by the monobloc. On the other hand, the surgery tends to lower the outer canthus, increasing the negative slant of the fissure. The postoperative changes induced by the frontofacial monobloc advancement need to be taken into account when the surgery is going to be performed.

Reduction of Morbidity of the Frontofacial Monobloc Advancement in Children by the Use of Internal Distraction

Surgical treatment of faciocraniosynostosis is a complex problem that includes both function and aesthetics. Treatment goals are to prevent further neurologic disorders and to correct the morphologic impairment. Thirty-six patients with faciocraniosynostosis (mean age, 5.2 years) were evaluated prospectively after frontofacial monobloc advancement and quadruple internal distraction. Four distractors were used in combination with a frontofacial monobloc advancement osteotomy. Complications and advancement were evaluated clinically and radiographically. Respiratory status was evaluated by polysomnography. Relapse was evaluated by comparing results at the time of distractor removal to 6-month values. The mean follow-up was 30 months. Distraction was completed in 35 patients. Twenty-eight patients (80 percent) completed their distraction uneventfully in the initial period. In seven patients, a problem related to the distraction devices required revision surgery, and subsequently six of them completed the distraction. One patient died the day after surgery from acute brain tonsillar herniation before distraction was begun. The exorbitism was corrected clinically in all patients in whom distraction was completed (n = 34). A class I occlusal relationship was obtained in 28 of 35 patients (80 percent). When respiratory impairment was present, it was corrected in all but two cases (14 of 16). A septic frontal osteonecrosis occurred in one patient 2 months after distraction was completed (frontal bone loss in one of 35 patients). Reossification at time of distractor removal was limited. Relapse has been observed predominantly at the occlusal level (six of 35) and more frequently in Pfeiffer syndrome. Internal distraction allows for early correction of respiratory impairment and exorbitism of faciocraniosynostosis, and limits the major risks of frontofacial monobloc advancement. Previous operations performed before the frontofacial monobloc advancement increased its morbidity.

Exorbitism Correction of Faciocraniosynostoses by Monobloc Frontofacial Advancement With Distraction Osteogenesis

Monobloc frontofacial advancement with internal distraction osteogenesis is still a relatively new technique, and the literature on exorbitism correction of syndromal faciocraniosynostosis with monobloc advancement is scarce. Using a image processing software, we measured on axial computed tomographic scans the effect of monobloc advancement on the correction of exorbitism on a large series of patients (n=38) with syndromal faciocraniosynostosis who underwent surgery for correction of cranial and facial hypoplasia. A population reference of 23 patients without faciocraniosynostoses was also measured. For each orbit, globe protrusion was expressed as the ratio between the length of the globe that is above the interzygomatic line and eye size. The results indicate that monobloc has a symmetrical effect on both eyes that is correlated to the preoperative degree of exorbitism. Longitudinal data indicate that exorbitism correction changes with time. A mean preoperative globe protrusion of 0.86 dropped sharply to 0.54 at 6 months after distraction and increased to 0.61 at 16 months after distraction removal, a value that is not significantly different from the ratio of the control group (0.60). Overall, the data suggest that a certain degree of enophthalmos in the early postoperative period does not necessarily imply a sustained overcorrection.

Respiratory Outcome of Midface Advancement with Distraction

Upper airway stenosis in patients with faciocraniosynostosis is very common and often severe. Midface advancement, either with a Le Fort III or concomitantly to a monobloc frontofacial advancement, may prevent a tracheotomy or result in its ablation. The amelioration of respiratory function appears to be much better if the midface advancement is combined with distraction osteogenesis, although large studies with long-term follow-up are rare. In this study, we reviewed the respiratory outcome between Le Fort III with distraction and monobloc advancement with distraction in 54 faciocraniosynostotic patients. Early respiratory results of both procedures were very good and stable at long-term follow-up. The choice between a Le Fort III and a monobloc procedure is made on the basis of presenting morphology, previous surgery, and age. Both can be expected to give a long-lasting improvement of upper airway obstruction.

Respiratory Outcome of Mid-face Advancement With Distraction

Upper airway stenosis in patients with faciocraniosynostosis is very common and often severe. Mid-face advancement, either with a Le Fort III or concomitantly to a monobloc frontofacial advancement, may prevent a tracheotomy or result in its ablation. The amelioration of respiratory function appears to be much better if the mid-face advancement is combined with distraction osteogenesis, although large studies with long-term follow-up are rare. In this study we reviewed the respiratory outcome between Le Fort III with distraction and monobloc advancement with distraction in 55 faciocraniosynostotic patients. Early respiratory results of both procedures were very good and stable at long-term follow-up. The choice between a Le Fort III and a monobloc procedure is made based on presenting morphology, previous surgery, and age. Both can be expected to give a long-lasting improvement of upper airway obstruction.

How to Make the Blockage between the Nasal Cavity and Intracranial Space Using a Four-Layer Sealing Technique

Craniofacial surgery for facial advancement or correction of severe craniofacial malformations such as orbital hypertelorism, Crouzon's disease, and Apert's syndrome may carry great risk. Postoperative infection after craniofacial surgery is a life-threatening complication. Ascending infection via nasofrontal communication in frontofacial monobloc advancement, intracranial Le Fort III osteotomy, correction of hypertelorism (intracranial approach), and acute trauma of cribriform plate can lead to life-threatening meningitis and meningoencephalitis. A four-layer sealing technique for the closure of nasofrontal communication using Gelfoam, galeopericranial flap, rib bone graft, and Tissel is a very effective method. Until the rib bone graft is taken up, Gelfoam is used to temporarily block bony defects and prevents displacement of the rib bone graft. The authors used galeoperiosteal flap for the sufficient blood supply to the rib bone graft. Tissel is used as a biologic adhesive and for blockage of the surrounding gaps. There were no cases of cerebrospinal fluid rhinorrhea, epidural abscesses due to nasofrontal ascending infection, or meningitis, and no cases underwent débridement due to necrosis of the frontal bone flap. This indicated that the blockage of nasofrontal communication was successful in this series. Moreover, postoperative cosmetic outcomes were satisfactory. This study indicated that the blockage using the Gelfoam, galeopericranial flap, rib bone graft, and Tissel application was effective for the thorough management of nasofrontal fistula and the prevention of recurrent episodes.

Quadruple distraction interne avec avancement fronto-facial précoce pour faciocraniosténose

Frontofacial monobloc advancement (FFMA), known as a high risk procedure, was evaluated in combination with distraction. Twenty patients with faciocraniosynostosis, were treated with FFMA and quadruple internal distraction. Mean age at surgery was 3.2 Years and mean follow-up was 14 Months (3-32 Months). Four distractors were used per patient in combination with complete osteotomy FFMA. Rate of distraction was classical (0.5 mm to 1mm per day) starting on day 7. The rate of short-term complications was evaluated. Correction of exorbitism and the dental occlusion relationship were assessed to evaluate achievement of advancement. Rate of relapse was evaluated by measuring the orbital bony gap on horizontal CT slices, before and 6 Months after removal of the distractor. Respiratory impairment when present was also evaluated. Distraction was completed in 18/20 patients. Fifteen patients completed their distraction uneventfully during the initial period. In three patients with CSF leaks, distraction was delayed at day 14 and 16, but was resumed and completed normally. Second operations were required for two patients during the early phase: the distractor failed in one requiring immediate reoperation to change the device; distraction was completed successfully. Device infection developed in two patients requiring removal of the device and abortion of distraction. One patient died the day after surgery from acute tonsillar herniation before distraction could be initiated. Exorbitism was clinically corrected in all cases in whom distraction was completed (n=18). Class I occlusion was obtained in 16/18 patients. When present, respiratory impairment was corrected in all cases. Septic frontal necrosis occurred in one patient two Months after distraction was completed and necessitated removal of the forehead flap. Distractors were easily removed through a coronal approach at 6 Months, but reossification was limited. Relapse was observed in three patients whose retaining phase was less than five Months. Rate of relapse was much lower when distraction was maintained more than six Months. Internal distraction can provide early correction of respiratory impairment due to faciocraniosynostosis in infancy and can reduce the major risks of frontofacial monobloc advancement. Morbidity is higher in patients with prior surgery before FFMA. Further evaluation is necessary to determine whether the two stage treatment strategy for faciocraniosynostosis (fronto-orbital advancement before one Year of age, and later facial Lefort III type advancement) could be replaced by a routine FFMA procedure.

Double distraction interne avec avancement frontofacial précoce pour faciocraniosténose. À propos de cinq cas cliniques

Frontofacial monobloc advancement is a surgical procedure which corrects at the same time both frontal and facial retrusions in faciocraniosynostosis. The high risk morbidity limited its use to major exorbitism or severe breathing impairment.We report the results of a preliminary experience in five children with a mean age of 3.8 years. All patients presented with a Crouzon or Pfeiffer syndrome. The procedure performed was a frontofacial monobloc osteotomy with peroperative mobilization. Two pairs of distractors (Martin-Medizin ®) were implanted. The upper pair of frontal distractors was located behind the supraorbital bar. The pair of temporo-zygomatic distractors (a modification of the MicroFrance ® prototypes) was implanted behind the zygomatic bone. In one patient a transfacial pin was connected to the anterior part the lower distractors, to avoid facial bipartition. Distraction was performed according to classical protocols with a 1 mm advancement per day, until sufficient advancement was obtained.The correction of exorbitism and facial retrusion was obtained in all cases. An open-bite was created by the advancement in two out of the five patients. Minor infection occured in one patient. This combination of distractors with frontofacial advancement seemed to increase the efficacy and reduce the morbidity. This might lead to a one stage strategy for faciocraniosynostosis treatment, even if minor additive procedures will be necessary when children get older and that insuffiscent facial growth still reccurs.

Complications in craniofacial surgery: the Turkish experience.

The records of 71 patients who underwent 75 consecutive craniofacial procedures involving a transcranial component have been analyzed retrospectively to assess the incidence and type of complications encountered during the 14-year period of 1985 to 1998 at Ege University Hospital, Department of Plastic and Reconstructive Surgery. The results were compared with those of similar reports from other craniofacial centers. Operations were classified into six groups according to their type to evaluate effectively their complication rates. Complications were also graded in terms of their severity. Serious complications were seen in 12 patients (16.8%), whereas life-threatening complications were seen in 6 patients (8, 4). The mortality rate was 2.7%. It is determined that complication incidence was notably higher in the group that underwent monobloc frontofacial advancement. A decrease in the rate of serious complications occurred with increased experience with the surgery. The results of our study indicate that although craniofacial surgery carries an inherent risk for significant complications, the risk can be minimized and the rate of mortality and major complications kept to an acceptable level by a careful and experienced craniofacial team.

The Monobloc Frontofacial Advancement

The results of 32 patients who underwent transcranial monobloc frontofacial advancement by the senior author (Wolfe) are examined. Fifteen of the patients were Crouzon's and 17 were Apert's, 14 of whom also underwent simultaneous facial bipartition. The ages ranged from 7 months to 14 years. Although a transcranial facial advancement carries with it substantial risks, with careful consideration of airway control, the anterior cranial base dura, and the retrofrontal dead space, the procedure is recommended for carefully selected patients. The indications and contraindications for the procedure in various age groups are given.