Hyponatremia in the postoperative craniofacial pediatric patient population: a connection to cerebral salt wasting syndrome and management of the disorder.

Hyponatremia after cranial vault remodeling has been noted in a pediatric patient population. If left untreated, the patients may develop a clinical hypoosmotic condition that can lead to cerebral edema, increased intracranial pressure, and eventually, to central nervous system and circulatory compromise. The hyponatremia has traditionally been attributed to the syndrome of inappropriate secretion of antidiuretic hormone (SIADH); however, in our patients the treatment has been resuscitation with normal saline as opposed to fluid restriction (the accepted treatment of SIADH), thus placing the diagnosis of SIADH in question. Patients who developed hyponatremia after intracranial injury or surgery were, until recently, grouped together as having SIADH. However, there are diagnosis and treatment differences between SIADH and another distinct but poorly understood disorder that is designated cerebral salt wasting syndrome (CSW). CSW is associated with increased urine output and increased urine sodium concentration and volume contraction, and it is frequently seen after a central nervous system trauma. We therefore developed a prospective study to evaluate the cause of the sodium imbalance.Ten consecutive pediatric patients who underwent intracranial surgery for various craniosynostotic disorders were postoperatively monitored in the pediatric intensive care unit for hemodynamic, respiratory, and fluid management. The first four patients were evaluated for electrolyte changes and overall fluid balance to determine the consistency with which these changes occurred. The remaining six patients had daily (including preoperative) measurement of serum electrolytes, urine electrolytes, urine osmolarity, serum antidiuretic hormone (ADH), aldosterone, and atrial natriuretic hormone (ANH). All patients received normal saline intravenous replacement fluid in the postoperative period. All of the patients developed a transient hyponatremia postoperatively, despite normal saline resuscitation. Serum sodium levels as low as 128 to 133 mEq per liter (normal, 137 to 145 mEq per liter) were documented in the patients. All patients had increased urine outputs through the fourth postoperative day (>1 cc/kg/h). The six patients who were measured had an increased ANH level, with a peak value as high as 277 pg/ml (normal, 25 to 77 pg/ml). ADH levels were low or normal in all but one patient, who had a marked increase in ADH and ANH. Aldosterone levels were variable. On the basis of these results, all but one patient showed evidence of CSW characterized by increased urine output, normal or increased urine sodium, low serum sodium, and increased ANH levels. The other patient had similar clinical findings consistent with CSW but also had an increase in ADH, thus giving a mixed laboratory picture of SIADH and CSW. The association of CSW to cranial vault remodeling has previously been ignored. This study should prompt reevaluation of the broad grouping of SIADH as the cause of all hyponatremic episodes in our postoperative patient population. An etiologic role has been given to ANH and to other, as yet undiscovered, central nervous system natriuretic factors. All of the patients studied required normal saline resuscitation, a treatment approach that is contrary to the usual management of SIADH. These findings should dictate a change in the postoperative care for these patients. After cranial vault remodeling, patients should prophylactically receive normal saline, rather than a more hypotonic solution, to avoid sodium balance problems.

Distraction osteogenesis of the craniofacial skeleton.

Distraction osteogenesis is becoming the treatment of choice for the surgical correction of hypoplasias of the craniofacial skeleton. Its principle is based on the studies of Ilizarov, who showed that osteogenesis can be induced if bone is expanded (distracted) along its long axis at the rate of 1 mm per day. This process induces new bone formation along the vector of pull without requiring the use of a bone graft. The technique also provides the added benefit of expanding the overlying soft tissues, which are frequently deficient in these patients. This article reviews the authors' 11-year clinical and research experience with mandibular distraction osteogenesis. It highlights the indications and contraindications of the technique and emphasizes the critical role that basic science research has played in its evolution.

Development of a device for the delivery of agents to bone during distraction osteogenesis.

Various agents have been theoretically and experimentally implicated as mediators of distraction osteogenesis (DO). The purpose of this study was to develop a vehicle for the potential delivery of these factors to the region of the distraction site in an attempt to manipulate this biologic process. Three adult mongrel dogs (12 months old) had oblique osteotomies performed bilaterally through the gonial regions. In group I, the external distracter was affixed to the right hemimandible of two dogs (n = 2 hemimandibles) with cannulated pins (external diameter = 1.5 mm; lumen diameter = 1.0 mm; length = 60 mm), whereas the distracter on the left was affixed with standard, noncannulated pins of the same dimensions. In group II, cannulated pins were used to affix the external distracter to both hemimandibles (n = 2 hemimandibles) of a dog. The devices were activated after a 5-day latency period and were lengthened at a rate of 1 mm/day for 20 days. During the distraction period, 0.1 ml/d of sterile india ink was injected into the cannulated pins, after which the sterile stylet was replaced. The activation protocol was followed by 28 days of fixation (consolidation period). The hemimandibles from group I underwent removal of soft tissues, acetone fixation, and gross examination/photography, whereas the hemimandibles from group II were prepared for histologic evaluation (whole mount, hematoxylin and eosin staining). All dogs survived to the end of the study and demonstrated successful DO without evidence of complications. Hemimandibles in group I displayed evidence of india ink on both the lingual and buccal cortex around the cannulated pin site, in the regenerate and on the neocortices of the distracted segment. Hemimandibles of group II showed histologic evidence of the india ink being deposited densely around the cannulated pin site and extending in a radial fashion around the pin site into the regenerate. This study demonstrates for the first time a vehicle device for the delivery of an inert dye to the regenerate site during distraction osteogenesis. This vehicle offers the potential of delivery of various factors implicated in distraction osteogenesis (i.e., mitogens) in an attempt to alter this process and also substances (i.e., chemotherapy, antibiotics, etc.) for use in the treatment of various osteopathies.

Imaging the neonatal mandible for accurate distraction osteogenesis.

The position of mandibular teeth is difficult to document in the neonatal patient. Panorex images are difficult to obtain in an uncooperative pediatric patient. The new technique presented by the authors uses computed tomographic data to create a curved, reformatted image of the mandible, and generates an image similar to a panorex image. This curved, reformatted mandibular image provides accurate visualization of the mandible and mandibular teeth. This technique allows for precise pin placement and osteotomy in distraction osteogenesis.

Remodeling of the temporomandibular joint following mandibular distraction osteogenesis in the transverse dimension.

Transverse mandibular distraction osteogenesis involves moving the osteotomized segments of the mandible in either a varus or valgus direction. This maneuver allows for widening of the bigonial distance or for a lateral shift of an asymmetric mandibular midline. During this process, a significant amount of torque is placed on the mandibular condyles, because they act as the pivot point for the mandibular translation. Although standard linear distraction osteogenesis induces transient, reversible changes in the temporomandibular joint, it is not known what effect the varus and valgus stresses of transverse distraction have on the temporomandibular joint. We therefore designed a study to document the temporomandibular joint changes following various degrees of transverse distraction. Bilateral transverse mandibular distraction was performed on 10 adult, female mongrel dogs using an external, multiplanar mandibular distraction device. The distraction protocol was as follows: (1) complete osteotomy at the angle of the mandible, (2) 5-day latency period, (3) distraction rate of 1 mm/day, (4) rhythm of one turn per day, (5) linear activation 16 to 30 mm bilaterally, and (6) 8-week consolidation period. A variety of varus and valgus distraction vectors were applied to the mandible only after 10 mm of initial linear distraction had been achieved. Posteroanterior and lateral cephalograms were performed throughout the entire process. Pre-distraction and post-consolidation computed tomographic scans were also performed. Changes in mandibular conformation, axis of rotation, temporomandibular joint structure, and glenoid fossa changes were directly assessed by evaluating the postmortem craniofacial skeleton. The findings were compared with those of normal, age-matched mongrel dog skulls. Significant remodeling changes were observed in the temporomandibular joints of all animals involved in the study. The mandibular condyles demonstrated varying degrees of flattening and erosion at all contact points with the craniofacial skeleton. In some cases, the condyle became part of the distraction regenerate process and was hypertrophied in all dimensions. The condyles were frequently displaced out of the glenoid fossa, particularly on the side in the direction of varus distraction. When the latter occurred, a new fossa was created on the undersurface of the zygomatic arch. Varying degrees of mandibular rotation in the sagittal plane were also observed, which led to abnormal torquing of the condyles in the coronal plane, depending on whether the axis of rotation occurred primarily around the condyle or around the distraction regenerate zone.In conclusion, transverse mandibular distraction is an effective means of producing a varus or valgus shift in the gonion relative to the midsagittal plane. However, unlike linear or angular mandibular distraction, transverse distraction has a multitude of nontransient effects on the temporomandibular joint. Therefore it must be emphasized that in clinical practice, transverse distraction should be used cautiously. One must also be aware that such a maneuver in distraction can have negative effects on the temporomandibular joint.

ZNF198-FGFR1 transforming activity depends on a novel proline-rich ZNF198 oligomerization domain.

An acquired chromosomal translocation, t(8;13)(p11;q11-12), observed in a distinctive type of stem cell leukemia/lymphoma syndrome, leads to the fusion of the 5' portion of ZNF198 and the 3' portion of FGFR1. ZNF198-FGFR1 fusion transcripts encode 4 to 10 zinc fingers, a proline-rich region, and the intracellular portion of the FGFR1 (fibroblast growth factor receptor 1) receptor tyrosine kinase. We demonstrate that the ZNF198 proline-rich region constitutes a novel self-association domain. When fused to the intracellular domain of FGFR1, the ZNF198 proline-rich region is sufficient to cause oligomerization, FGFR1 tyrosine kinase activation, and transformation of Ba/F3 cells to IL-3 independent growth. (Blood. 2000;96:699-704)

"Pumping the regenerate": an evaluation of oscillating distraction osteogenesis in the rodent mandible.

Mandibular distraction osteogenesis (DO) has become an important technique to lengthen the hypoplastic mandible and to reconstruct osseous defects after ablative surgery. The hallmark of successful DO is the creation of new bone within the distraction gap. Several anecdotal reports have described alternating compressing and lengthening protocols (i.e., "pumping the regenerate") to augment regenerate bone formation. The purpose of this experiment was to analyze formally the effects of an alternating compression/distraction protocol with a traditional distraction protocol. Ten adult male rats underwent unilateral mandibular osteotomy with placement of a custom distractor. After a latency period of 5 days, distraction was initiated at a rate of 0.25 mm twice daily. Animals in the control group (N = 5) were distracted to a length of 5.0 mm for 10 days at a rate of 0.25 mm twice daily. In contrast, animals in the experimental group (N = 5) were distracted to a length of 2.5 mm (at a rate of 0.25 mm twice daily) for 5 days, then compressed 1.0 mm for a 2-day period, and redistracted to a length of 5.0 mm. Regenerate cross-sectional area was evaluated by computed tomography performed after 5 weeks of consolidation. Gross examination and histological analysis were performed by a panel of experienced reviewers. Radiological as well as histological analysis of regenerate cross-sectional area demonstrated no significant differences between experimental (i.e., "pumped") and control groups. Both groups demonstrated excellent regenerate bone formation with no evidence of fibrous union. This study represents the first attempt to investigate the anecdotal technique of pumping the mandibular regenerate. The authors have demonstrated that pumping the regenerate leads to no substantial differences in radiological or histological appearance of regenerate bone formation.

Craniofacial shortening by contraction osteogenesis: an experimental model.

Application of gradual external forces to correct craniofacial deformities challenges many procedures in conventional craniomaxillofacial surgery. Distraction osteogenesis is replacing traditional osteotomies for correction of patients with craniomaxillofacial deficiencies. However, the reverse concept, contraction osteogenesis, has yet to be established for patients with craniomaxillofacial excesses. The purpose of this investigation is to demonstrate the contraction osteogenesis phenomenon applied in a controlled animal model during the craniofacial growth period. Twenty-six 26-day-old rabbits were assigned to one of four groups: 0, control; 1, pin control (pin insertion); 2, no contraction (pins and contraction device application, without active contraction); and 3, contraction (pin insertion, contraction device application, and active contraction). An external fixator was placed across the incisive-maxillary suture, and the effects after 4.5 weeks of contraction at a rate of 0.5 mm twice a week were compared with control groups. The results were assessed by craniometric and cephalometric measurements and by histologic examination. Gross alterations were evident in the contraction group, characterized by midface anteroposterior shortening, maxillary regression, snout deviation, and anterior crossbite. Histologic examination of the contraction group demonstrated a significant increase in osteoblastic activity. Contraction osteogenesis is a new treatment concept in craniofacial development and may offer therapeutic opportunities for shortening skeletal structures without the need of osteotomies, thus taking advantage of the potential of craniofacial growth and remodeling.

Syndromes involving craniosynostosis and midface hypoplasia.

This article reviews a number of well-known syndromes involving craniofacial synostosis and associated midface deficiencies. Syndromes discussed include Apert's, Crouzon's, Saethre-Chotzen, and Carpenter's. Clinical characteristics and genetic defects are discussed. A general approach to surgical management is outlined.

A rat model of gingivoperiosteoplasty.

The ability to avoid a subsequent bone graft makes the use of gingivoperiosteoplasty (GPP) at the time of cleft lip repair an attractive technique. The use of GPP, in combination with presurgical orthodontics, has been shown to result in successful bony union in the majority of patients. However, secondary bone grafting is still necessary in 30% to 40% of patients due to persistent alveolar bony defects. The elucidation of methods to improve the success rates of these procedures has been hampered by the lack of reproducible animal models. The purpose of this study was, therefore, to develop a rodent model of GPP that would facilitate the investigation of methods to improve osteogenesis in alveolar defects. We report a surgically produced rat model (9 x 5 x 3-mm alveolar defect) that is reproducible, inexpensive (relative to large-animal models), and simple technically. In addition, healing in this model occurs in a predictable manner during a 12-week period, thus enabling analysis of methods designed to accelerate or facilitate osseous regeneration.

Controlled multiplanar distraction of the mandible. Part III: Laboratory studies of sagittal (anteroposterior) and horizontal (mediolateral) movements.

Distraction osteogenesis has proven to be an effective technique for the correction of mandibular deficiencies. However, problems have been encountered in achieving a final, idealized form of the mandible when using distraction devices capable of moving the bone segments in only one dimension (uniplanar). Specifically, occlusal irregularities and deficiencies in lower facial contour have been seen following uniplanar distraction. To address these problems, a distraction device capable of independent movements in three planes (multiplanar) was developed. Previously reported studies in a canine model have demonstrated that this device can successfully distract the mandible along both the sagittal axis (anteroposterior or z-axis) and the vertical axis (superoinferior or y-axis). This study examines the ability of the multiplanar device to distract along the sagittal and horizontal axes (mediolateral or x-axis). A total of 12 dogs were included in the study. All animals underwent unilateral or bilateral mandibular distraction using an external multiplanar device. After a latency period of 5 days, primary distraction along the anteroposterior axis at a rate of 1 mm/day for 10 days (10 mm total) was performed. During the following 10 days, along with an additional 11 mm to 20 mm of anteroposterior axis distraction, concomitant secondary distraction was performed along the horizontal (mediolateral) axis at a rate of 5 degrees/day (50 degrees total). Cephalometric radiographs were obtained preoperatively and at the conclusion of both anteroposterior and combined anteroposterior-mediolateral distraction. Computed tomography (CT) scans were obtained preoperatively and at the end of consolidation (28 days), after which all animals were sacrificed and the dry skulls examined. In all animals, distraction along the mediolateral or x-axis was found to change the anteroposterior projection of the mandible. Varus angulation of the device with respect to the midline of the mandible caused compression of the distracted segments and reduced the anteroposterior thrust of the mandible. In contrast, valgus positioning of the device, with respect to the midline of the mandible, created the opposite effect, increasing the distracted length in the anteroposterior direction. The bone (mandibular) segments being distracted assumed the orientation of the device only for valgus positioning of the device (producing a decrease in the bigonial distance). Conversely, there was no effect from the mediolateral angulation on the distracted segments during varus positioning of the device. A possible explanation for this finding may be a greater resistance to an increase in the bigonial distance (varus positioning of the device) posed by obstruction of lateral movement of the condyle. This stands in contrast to a decrease in the bigonial distance observed following valgus positioning of the device. These findings confirm the clinical impression that distraction along the anteroposterior or sagittal axis remains the critical or keystone therapeutic maneuver in distraction of the mandible. Mediolateral or horizontal axis distraction is best used only as a supplementary movement; in essence, it only affects the anteroposterior dimension with little impact on clinically relevant changes to the bigonial distance.

Increased IGF-I and IGF-II mRNA and IGF-I peptide in fusing rat cranial sutures suggest evidence for a paracrine role of insulin-like growth factors in suture fusion.

Premature cranial suture fusion, or craniosynostosis, can result in gross aberrations of craniofacial growth. The biology underlying cranial suture fusion remains poorly understood. Previous studies of the Sprague-Dawley rat posterior frontal suture, which fuses at between 12 and 20 days, have suggested that the regional dura mater beneath the cranial suture directs the overlying suture's fusion. To address the dura-suture paracrine signaling that results in osteogenic differentiation and suture fusion, the authors investigated the possible role of insulin-like growth factors (IGF) I and II. The authors studied the temporal and spatial patterns of the expression of IGF-I and IGF-II mRNA and IGF-I peptide and osteocalcin (bone morphogenetic protein-4) protein in fusing posterior frontal rat sutures, and they compared them with patent coronal (control) sutures. Ten Sprague-Dawley rats were studied at the following time points: 16, 18, and 20 days of gestation and 2, 5, 10, 15, 20, 30, 50, and 80 days after birth (n = 110). Posterior frontal and coronal (patent, control) sutures were analyzed for IGF-I and IGF-II mRNA expression by in situ hybridization by using 35S-labeled IGF-I and IGF-II antisense riboprobes. Levels of IGF-I and IGF-II mRNA were quantified by counting the number of autoradiograph signals per cell. IGF-I and osteocalcin immunoreactivity were identified by avidin-biotin peroxidase immunohistochemistry. IGF-I and IGF-II mRNA were expressed in dural cells beneath fusing sutures, and the relative mRNA abundance increased between 2 and 10 days before initiation of fusion. Subsequently, IGF-I and IGF-II mRNA were detected in the suture connective tissue cells at 15 and 20 days during the time of active fusion. In contrast, within large osteoblasts of the osteogenic front, the expression of IGF-I and IGF-II mRNA was minimal. However, IGF-I peptide and osteocalcin protein were intensely immunoreactive within these osteoblasts at 15 days (during the period of suture fusion). These data suggest that the dura-suture interaction may be signaled in a paracrine fashion by dura-derived growth factors, such as IGF-I and IGF-II. These peptides, in turn, stimulate nearby osteoblasts to produce bone-promoting growth factors, such as osteocalcin.

Cranial vault deformity and intracranial hypertension secondary to cephalic molding at delivery: a case and its management.

Cephalic molding at birth has been traditionally felt to be benign, resulting in only a transient and self-correcting cranial deformity. However, we report a 6-month-old infant who presented with extensive cephalic molding at birth in combination with persistent brachyturricephaly from unilateral coronal synostosis and occipital deformation. Helmet therapy over a 3-month period failed despite patient compliance and numerous adjustments. Intracranial hypertension developed, as documented by multiple occipital bony erosions on computed tomographic scan and by an elevated direct intracranial pressure reading. The cranial vault asymmetry was corrected in two surgical stages: (1) occipital bar advancement, temporoparietal bone remodeling, and midline sagittal strip compression to reduce vertical height, followed in 3 months by (2) fronto-orbital advancement and remodeling.

Moebius syndrome: the new finding of hypertrophy of the coronoid process.

The first detailed description of congenital facial paralysis was reported by Moebius in 1888. It is characterized by either unilateral or bilateral paralysis of the facial muscles and an associated abducens palsy. The present report is of two patients with Moebius syndrome, who were also diagnosed with trismus at birth. Each patient also demonstrated bilateral hypertrophy of the coronoid process of the mandible. In effect, the zygoma obstructed the excursion of the mandible because of a "coronoid block." A three-dimensional computed tomography scan demonstrated normal temporomandibular joints but bilateral hypertrophy of the coronoid processes and micrognathia. Both patients demonstrated less than 10 mm of oral excursion. Bilateral coronoidectomies were performed through an intraoral approach. The oral excursions after surgery increased to at least 20 mm. In each of these patients, the coronoid process was enlarged relative to the zygoma, which was of normal size and configuration. The trismus was associated with blocking of the coronoid by the anterior zygoma, preventing open or full excursion of the hypoplastic mandibles. Moebius syndrome can have a variable presentation at birth. In two patients, the authors describe a new finding of hypertrophy of the coronoid process and trismus secondary to obstruction of the coronoid by the hypertrophic zygomas during oral excursions. Each patient is described, and a review of the literature is discussed.

Distraction osteogenesis of the mandible: a ten-year experience.

Mandibular distraction has been performed at the authors' institution for the past 10 years on a variety of craniofacial anomalies. This article reviews the experience with distraction and outlines the authors' treatment algorithms based on patient age and pathology. The roles of distraction versus conventional orthognathic surgery are reviewed. The need for preoperative surgical planning and postoperative orthodontic therapy is emphasized.

Mandibular growth after distraction in patients under 48 months of age.

Distraction osteogenesis is an effective technique for reconstruction of the congenitally deficient mandible. However, the age at which it is best performed remains under discussion. Distraction performed at an early age, while possibly allowing the face to develop with a more normal functional matrix, may entail a higher rate of complications. Additionally, it is possible that subsequent asymmetric growth of the mandible may necessitate serial distraction. To address this issue, the clinical records and cephalometric radiographs of all patients less than 48 months of age undergoing mandibular distraction at New York University Medical Center between August of 1989 and August of 1997 were examined. There was a total of 14 patients ranging in age from 19 months to 43 months. Nine patients had a diagnosis of unilateral craniofacial microsomia, three had Treacher Collins syndrome, one had Nager syndrome, and one had bilateral developmental micrognathia. The average amount of distraction was 27 mm (range, 23 to 39 mm) in unilateral cases and 24 mm in bilateral cases (range, 15 to 31 mm). The period of clinical follow-up averaged 32.6 months (range, 12 to 92 months). All patients showed significant improvement in craniofacial appearance, and in four patients, long-term tracheostomy tubes were removed. There were two major complications. In one patient with craniofacial microsomia, there was a relapse in the early postretention phase related to the presence of a dentigerous cyst. This required removal of the cyst and repeat distraction. In the patient with Nager syndrome, a coronoid ankylosis developed requiring surgical release. There were no other major complications. The scars required revision in only two of the patients. Cephalometric analysis of the patients in the study revealed a differential in the rate of growth between the affected and the unaffected side in all cases of craniofacial microsomia. The affected side always grew at a slower rate than the contralateral side after the distraction process was complete. This led to a progressive asymmetry of the rami, clinically expressed by some degree of facial asymmetry and an occlusal cant. For this reason, secondary distraction was required in one patient and is planned in a second. Initial overcorrection of the patient would seem to minimize the likelihood that secondary distraction will be necessary. Distraction osteogenesis for reconstruction of the mandible in this subset of young patients was a safe and effective technique for improving the craniofacial skeletal form and appearance, with minimal associated morbidity. Longer follow-up is necessary to assess the full impact of growth in these cases.

Rat mandibular distraction osteogenesis: II. Molecular analysis of transforming growth factor beta-1 and osteocalcin gene expression.

Distraction osteogenesis is a powerful technique capable of generating viable osseous tissue by the gradual separation of osteotomized bone edges. Although the histologic and ultrastructural changes associated with this process have been extensively delineated, the molecular events governing these changes remain essentially unknown. We have devised a rat model of mandibular distraction osteogenesis that facilitates molecular analysis of this process. Such information has significant clinical implications because it may enable targeted therapeutic manipulations designed to accelerate osseous regeneration. In this study, we have evaluated the expression of transforming growth factor beta-1, a major regulator of osteogenesis during endochondral bone formation and development, and osteocalcin, an abundant noncollagenous extracellular matrix protein implicated in the regulation of mineralization and bone turnover. The right hemimandible of 36 adult male rats was osteotomized, and a customized distraction device was applied. Animals were allowed to recover and, after a 3-day latency period, were distracted at a rate of 0.25 mm twice daily for 6 days followed by a 2- or 4-week consolidation period. Distraction regenerate was harvested after the latency period, days 2, 4, or 6 of distraction, and after 2 or 4 weeks of consolidation and processed for Northern analysis (n = 4 at each time point) and immunohistochemical localization of TGF-beta1 (n = 2 at each time point). Six sham-operated animals (i.e., skin incision without osteotomy) were also killed (immediately postoperatively), and the mandibles were harvested and prepared in a similar fashion. Equal loading and transfer of RNA for Northern analysis was ensured by stripping and probing membranes with a probe against GAPDH (a housekeeping gene). Our results demonstrate that the spatial and temporal patterns of TGF-beta1 mRNA expression and protein production coincide with osteoblast migration, differentiation, and extracellular matrix synthesis. In addition, we demonstrate that TGF-beta1 production may be an important regulator of vasculogenesis during mandibular distraction osteogenesis. Finally, we have shown that osteocalcin gene expression coincides temporally with mineralization during rat mandibular distraction osteogenesis.

Early decannulation with bilateral mandibular distraction for tracheostomy-dependent patients.

Obstructive sleep apnea in the neonatal period may originate from a hypoplastic mandibular framework causing retroposition of the base of the tongue and an inadequate hypopharyngeal space. A tracheotomy in childhood is an effective treatment for obstructive sleep apnea, but it is associated with increased morbidity, management problems, and difficulties in social interaction. Tracheostomy-dependent pediatric patients who underwent mandibular distraction were reviewed to determine the effectiveness of this technique in achieving decannulation. A clinical review was completed to determine the status of the tracheostomy after external, unidirectional distraction in tracheostomy-dependent patients. Expansion of the mandibular framework was analyzed using traditional bony landmarks on predistraction and postdistraction lateral cephalograms. The area of the lower face was analyzed, and changes in the position of the hyoid bone were determined. Four patients with tracheostomies underwent an average of 21.3 mm and 20.8 mm of distraction on the left and right hemimandibles, respectively. The average age at the time of distraction was 2.7 years (range, 2.2 to 3.2 years). All patients underwent successful decannulation at an average of 3.8 months (range, 1.5 to 5.5 months) after completion of distraction. The area of the lower face increased 26.9 percent (range, 12.2 to 53.5 percent) after distraction, and the hyoid bone advanced an average of 14.5 mm (range, 8 to 25 mm). Bilateral mandibular distraction is an effective method of expanding the mandibular framework and concomitantly advancing the base of the tongue. The technique provides a tool for early intervention and decannulation in pediatric patients with indwelling tracheostomies secondary to mandibular deficiencies.