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Introduction Saethre-Chotzen syndrome is a genetic condition characterized by craniofacial and limb anomalies, with craniosynostosis (mainly coronal) being the most frequent craniofacial finding. Cranial and facial deformities can be extremely variable requiring individualization of treatment strategies. We present our case series to highlight clinical findings, treatment philosophy, and challenges facing Saethre-Chotzen patients. Methods A retrospective review was performed on records of patients given a diagnosis of Saethre-Chotzen syndrome at the University of California Los Angeles (UCLA) Craniofacial Clinic (n = 7) between 1980 and 2010. Patients with complete records were included in this study, and review of demographic data, clinical findings, surgical interventions and postoperative follow-up, and stability were performed. Results Seven patients (1 male and 6 female) were included in this study. The average age at which the patients were first seen was 6.5 years. Suture involvement was bicoronal (n = 6) and unicoronal (n = 1). There was 1 patient having superimposed metopic synostosis, and there was another patient having Kleeblattschadel deformity. Previous procedures performed for patients before establishing care at UCLA were strip craniectomy (n = 2) and fronto-orbital advancement (n = 2). All patients (n = 7) had fronto-orbital advancements at UCLA. Other skeletal operations included the following: redo forehead advancement and contouring (n = 3), monobloc advancement (n = 1), and LeFort III distraction (n = 1). Five patients reached skeletal maturity, and 2 patients received LeFort I advancement for class III malocclusion, one of which also required a bilateral sagittal split osteotomy of the mandible. Conclusion Clinical presentation and severity of deformity in Saethre-Chotzen syndrome are variable. Our current report reviews our treatment strategies and illustrates the predominance of cranial and upper face deformities and frequent need for redo surgeries to address forehead asymmetry in this group of syndromic craniosynostosis patients.

BACKGROUND Both internal and external distraction devices have been used successfully in correcting midface hypoplasia. Although the indication for surgery and the osteotomy techniques may be similar, deciding when to use internal versus external devices has not been studied. The authors studied patient-reported outcomes with FACE-Q and functional surveys for internal and external devices for midface distraction patients. METHODS Patients who underwent distraction advancement after Le Fort I and Le Fort III were surveyed using the FACE-Q survey and a functional survey. Equal groups of internal and external device patients were compared (n = 64). Data recorded included: sex, age, follow-up, diagnosis, operating room time, expected blood loss, length of stay, distraction length, consolidation time, and complications. RESULTS Internal and external device groups were similar with regards to patient diagnosis, operative time, expected blood loss, distraction length but consolidation times differed (internal = 3.6 versus external = 1.1 months). For FACE-Q appearance appraisal, there were similarities in domain and scale. For the functional survey (airway/breathing, ocular/vision, occlusion/eating, speech/articulation), there was also similar scoring. However, internal device patients had superior FACE-Q scores for Quality of Life: Social Function (80.9 versus 68.9), Early Life Impact (92.9 versus 62.4), Dental Anxiety (70.2 versus 48.3), Psychological Well-being (87.8 versus 68.6); and Decision Satisfaction (81.2 versus 56.9) and Outcome Satisfaction (91.0 versus 84.7). CONCLUSIONS Internal and external midface distraction patients had similar patient-reported outcomes for appearance and functional improvement; however, internal device patients were more satisfied with their quality of life and their decision to undergo the procedure.

Transabdominal preperitoneal (TAPP) repair is the most widely used laparoscopic technique for the treatment of inguinal hernia in Japan. Many studies have shown that in comparison with open hernia repair, laparoscopic repair results in less pain and a shorter convalescence. However, postoperative pain remains a concern. One possible cause of postoperative pain in the early postoperative phase is strain or cough on removal of the endotracheal tube. Use of a supraglottic airway (SGA) device helps to avoid such complaints. We evaluated postoperative pain after TAPP repair using the SGA for general anesthesia. We evaluated the postoperative pain in 146 patients with inguinal hernia repaired by TAPP in our hospital between May 2013 and May 2016. A total of 144 adult patients of American Society of Anesthesiologists physical status I and II who underwent needlescopic TAPP surgery were randomly allocated to one of two groups of 72 patients: group A (SGA), in which the patient’s airway was secured with an appropriately sized I-gel, and group B (endotracheal tube), in which the airway was secured under laryngoscopy. There was no significant difference between the groups regarding patient background, postoperative hospital stay, and operation time, and TAPP was performed safely in all cases. In the analysis of postoperative pain, the mean Numerical Rating Scale score of peak pain in group A was significantly less than that of group B (2.10 ± 2.05 vs 2.90 ± 2.65; p = 0.043). In group A, the percentage of patients who had an NRS score of 0 was 51.4% 30 min after surgery, 62.5% after 6 h and 68.1% at POD1, and compared to group B, the NRS scores were significantly higher at POD1 (p = 0.003), and the level of postoperative pain in group A tended to decrease earlier than that in group B. The results of this study are the first to show that an SGA device can reduce postoperative pain after laparoscopic surgery.

In the absence of randomized controlled data and even propensity-matched data, indications for, and outcomes of, laparoscopic repeat liver resection for hepatocellular carcinoma (HCC) remain uncertain. This study aimed to clarify the current indications for laparoscopic repeat liver resection for HCC, and to evaluate outcomes.Forty-two liver surgery centres around the world registered patients who underwent repeat liver resection for HCC. Patient characteristics, preoperative liver function, tumour characteristics, surgical method, and short- and long-term outcomes were recorded.Analyses showed that the laparoscopic procedure was generally used in patients with relatively poor performance status and liver function, but favourable tumour characteristics. Intraoperative blood loss (mean(s.d.) 254(551) versus 748(1128) ml; P 0·001), duration of operation (248(156) versus 285(167) min; P 0·001), morbidity (12·7 versus 18·1 per cent; P = 0·006) and duration of postoperative hospital stay (10·1(14·3) versus 11·8(11·8) days; P = 0·013) were significantly reduced for laparoscopic compared with open procedures, whereas survival time was comparable (median 10·04 versus 8·94 years; P = 0·297). Propensity score matching showed that laparoscopic repeat liver resection for HCC resulted in less intraoperative blood loss (268(730) versus 497(784) ml; P = 0·001) and a longer operation time (272(187) versus 232(129); P = 0·007) than the open approach, and similar survival time (12·55 versus 8·94 years; P = 0·086).Laparoscopic repeat liver resection is feasible in selected patients with recurrent HCC.Dado que no existen ensayos clínicos controlados ni estudios de datos emparejados por puntaje de propensión, todavía hay dudas sobre las indicaciones y los resultados de la resección iterativa laparocópica de un carcinoma hepatocelular (hepatocellular carcinoma, HCC). Este estudio tuvo como objetivo esclarecer las indicaciones actuales y los resultados de la resección hepática laparoscópica iterativa del HCC. MÉTODOS: Se incluyeron los pacientes de 42 centros de cirugía hepática a nivel mundial en los que se había realizado una resección hepática iterativa por HCC. Se analizaron las características del paciente, la función hepática preoperatoria, las características del tumor, el abordaje quirúrgico y los resultados a corto y largo plazo.El análisis demostró que la vía laparoscópica generalmente se utilizaba en pacientes con carácteristicas tumorales favorables, pero con estado funcional y función hepatica relativamente peores. La pérdida de sangre intraoperatoria (254,3 ± 551,2 versus 748,0 ± 1127,7 mL, P 0,001), la duración de la intervención (247,6 ± 155,8 versus 285,1 ± 167,0 minutos, P 0,001), la morbilidad (12,7 versus 18,1%, P = 0,005) y la estancia hospitalaria postoperatoria (10,07 ± 14,29 versus 11,80 ± 11,79 días, P = 0,010) fueron significativamente menores para los pacientes tratados por via laparoscópica en comparacion con la vía abierta, mientra que el tiempo de supervivencia fue comparable (mediana 10,04 versus 8,94 años, P = 0,297). El estudio de emparejamiento por puntaje de propensión mostró que la resección hepática iterativa por vía laparoscópica de un HCC (frente a la vía abierta) conllevaba una menor pérdida sanguínea intraoperatoria (268,0 ± 730,2 versus 496,5 ± 784,2 mL, P = 0,01), una mayor duración de la intervención (272,1 ± 187,2 versus 231,8 ± 129,1 minutos , P = 0,07) y un tiempo de supervivencia similar (mediana 12,55 versus 8,94 años, P = 0,0855). CONCLUSIÓN: La resección hepática iterativa por vía laparoscópica es factible en pacientes seleccionados con HCC recidivado.

Summary Background To determine the need for latency period in membranous bone distraction, we performed 1) in vitro comparison of preosteoblasts suspended in a 3D microdistraction model and 2) a clinical study comparing mandibular distraction cases with/without latency. Methods In the In Vitro study, Preosteoblasts polymerized in 3D-collagen gel were placed in a microdistractor and separated into three groups: 1) distraction with latency, 2) distraction without latency, and 3) static. After 2, 4, 6, and 8 days, cell proliferation, total protein levels, alkaline phosphatase activity, and osteogenic gene expression were assessed through RT-PCR. In the clinical study, patients underwent mandibular distraction in two groups: 1) latency and 2) no latency (n = 45). The rest of the distraction protocol was identical. Outcome was based on clinical examination, radiographs at six months, and 3D CT scans. Results In the In Vitro study, The distraction without latency group compared to the latency group had delays in: proliferation, total protein count, alkaline phosphatase activity, osteogenic gene expression in CBFA-1 (fourfold vs. eighteenfold), and in osteocalcin (twofold vs. sixfold). The distraction without latency group had higher apoptotic levels during the first four days compared to the latency group (68% vs. 14%). For the clinical study, similar perioperative complications (5% vs. 6%), X-ray mineralization (93% vs. 94%), bone volume, (8.6 vs. 9.1 cc) and bone density of central distraction zone (78% vs. 81%) were observed with or without latency. Conclusions In vitro studies showed poorer results in cell survival, proliferation and osteogenic activity compared to distraction with latency; yet, clinically, there were no differences in distraction with latency versus without.

The Tessier number 3 cleft is one of the most intricate and destructive of all facial clefts, presenting surgeons with a difficult task for reconstruction. We present a series of 10 patients with this rare cleft all treated by a single surgeon over 30 years. All patients with Tessier number 3 clefts treated between 1978 and 2008 by the senior surgeon were reviewed. Demographic data and all associated clinical findings including cranial and extracranial anomalies were recorded. Methods used to reconstruct each patient were also noted. Seven males and three females were identified and age at initial treatment ranged from 12 months to 12 years. Mean follow-up was 6.3 years. Multiple craniofacial anomalies were appreciated including other rare facial clefts, hypertelorbitism, lacrimal obstruction, anophthalmia, choanal atresia, and hemifacial microsomia. Amniotic banding was the most prominent extracranial finding noted in these patients. Tessier number 3 clefts can be associated with multiple other craniofacial anomalies making reconstruction challenging. Soft tissue and bony reconstruction must be considered separately, and a variety of tools may be employed to accomplish each goal. As the presentation can be highly variable, an individualized treatment plan must be made to meet each patient's specific needs.

BACKGROUND The philtrum and the Cupid's bow are the most prominent features of the upper lip and are crucial for normal appearance. Unfortunately, projection of the philtral column is a common deficiency of the repaired cleft lip. Although a multitude of methods for constructing the philtral dimple and ridge have been described, no single procedure has achieved complete satisfactory results. The authors describe their technique for construction of the philtral column using palmaris longus tendon. METHODS Between 1998 and 2009, a retrospective study was performed for identifying patients with depression of the philtral scar following cleft lip repair. Pre- and postoperative photographs were taken with at least 6 months follow-up. Postoperative patient satisfaction and occurrence of complications are reported. RESULTS In all, 17 patients who developed depression of the philtral ridge following cleft lip repair underwent philtral reconstruction utilizing palmaris longus tendon grafts by the senior author (H.K.K.). At the time of surgery, average age for 11 females and 6 males was 18.5 years. The follow-up period ranged from 6 to 126 months, with an average of 42.3 months. In the early postoperative period, 2 complications in the form of graft exposure occurred. However, both cases were successfully managed with preservation of the graft. All patients were satisfied with their long-term results, and none required further surgery. CONCLUSIONS The use of palmaris longus tendon graft is an effective, reproducible, and safe method by which to construct the philtral column in the secondary cleft lip deformity.

Introduction Pneumosinus dilatans is a rare condition and different techniques have been proposed for its management and correction. The abnormally expanded, aerated frontal sinus has been described in the literature as: frontal sinus hypertrophy, pneumosinus dilatans, pneumosinus frontalis, aerocele, pneumocele, sinus ectasia, hyperpneumatization and others. The precise aetiology and pathogenesis of the condition is unknown, although several basic hypotheses have been proposed Material and methods The authors report two cases of frontal bossing and supraorbital ridge deformity correction using craniofacial surgical principles. Discussion Functional and morphological results are discussed and compared with other open procedures. Conclusion A variety of surgical procedures have been proposed for the correction of the pneumosinus dilatans frontalis. The craniofacial approach is advocated to reproduce the normal anatomy of the forehead in the upper part, the supraorbital rim and glabellar area.

Antley-Bixler syndrome is a rare form of craniosynostosis characterized by unique clinical features that present particular challenges to long-term treatment. Typical craniofacial features include midface hypoplasia, trapezoidocephaly, frontal bossing, ocular proptosis, low-set protrusive ears, and choanal atresia. A staged surgical approach including fronto-orbital and midface advancements is required for intracranial pressure reduction, globe protection, airway patency, and malocclusion. In this case report, the combined, longitudinal surgical and orthodontic treatment strategies employed to correct severe facial deformity in a long-term 21-year survivor of Antley-Bixler syndrome are reviewed.

The optimal timing and treatment of Treacher Collins syndrome with regard to zygomatico-orbital osseous reconstruction has not been fully established. Osseous reconstruction performed at an early age may result in bone graft resorption; however, delays in surgical improvement may result in adverse psychosocial effects on the patient. To study the optimal age for reconstruction clinically, we examined 3 age groups based on timing of malar and eyelid reconstruction using a three-dimensional computed tomographic scan and three-dimensional photometric volume assessment. In addition, we collected outcome assessments from parents/patients using satisfaction surveys. Of 73 patients with Treacher Collins at the University of California Los Angeles Craniofacial Clinic, 45 had malar reconstruction, complete records, and were available for study. The patients were separated into 3 groups: (1) very young = 0 to 5 years, (2) mid-childhood = 6 to 12 years, and (3) adolescent/adult 13 years and older. As hypothesized, the 0 to 5 age group experienced the greatest percentage of complete bony resorption (77%) compared with the 6 to 12 age group (only 4%) and the 13 and older age group (0%). Interestingly, the 0 to 5 age group experienced the highest parent/patient satisfaction, possibly owing to better psychosocial experience. When dealing with treatment plans for patients with Treacher Collins, patients, parents, and physicians must balance the benefits of early surgical intervention, such as improved psychologic well-being, with the disadvantages of having to undergo a greater number of procedures, such as fat grafting after malar bone graft resorption.

The etiology of Angle class III malocclusion with facial asymmetry has not been fully elucidated. To investigate the etiology, patients with asymmetric prognathism (n = 30) from a single institution were assessed for previously undiagnosed torticollis and cranial base asymmetry. Presence of torticollis was determined by measuring restricted head movement when turning the head against a wall and cranial base tilt with upward gaze. Cranial base asymmetry was evaluated by preoperative three-dimensional computed tomography scans. Thirty-one percent of patients with prognathism presented with concurrent facial asymmetry. In patients with asymmetric prognathism, cranial base tilt was present on upward gaze in all patients; mean angle between head and wall was 31 degrees greater than that in control patients, and a 22% to 36% difference in the angle was present when comparing one side with the other. Based on these findings, all patients with asymmetric prognathism were found to be affected by torticollis. By computed tomography scan, 85% of these torticollis patients showed slight anteromedial displacement of the glenoid fossa ipsilateral to torticollis, and 73% demonstrated temporal fossa shift of 4 mm or greater. The current study demonstrates a strong association between asymmetric class III malocclusion, torticollis, and cranial base asymmetry. We conclude that undiagnosed torticollis is a likely etiology for otherwise idiopathic cranial base asymmetry and that cranial base asymmetry in turn causes facial asymmetry and malocclusion. This study highlights the importance of evaluating cranial base asymmetry and torticollis in patients with class III malocclusion to allow for earlier treatment and improved outcomes.

Patients with congenital craniofacial malformations present with complex challenges for reconstruction. Successful management requires individualized treatment often involving rebuilding the facial skeleton de novo, as well as correcting the overlying soft-tissue deficiencies in the final stages. At the University of California, Los Angeles (UCLA) Craniofacial Clinic, serial autologous fat transplantation performed during staged reconstruction is the preferred method.A total of 27 patients with a diagnosis of either craniofacial microsomia/Goldenhar (CM) (n = 19) or Treacher Collins syndrome (TC) (n = 8) were treated at the UCLA Craniofacial Clinic by autologous fat transfer between July 1999 and February 2009. Two-dimensional analysis was conducted on standardized preoperative and postoperative photographs to determine facial symmetry for every patient. Results was grouped based on pathology and analyzed by comparing adipocyte donor site (abdomen vs other).The mean ages at the time of first fat transfer were 17 years for the CM group and 15 years for the TC group. The average numbers of fat transfers per patient were 2.05 (CM) and 2.12 (TC). A mean of 3.74 procedures other than fat grafting was performed in each CM patient, whereas 4.38 other procedures were performed in each TC patient. In CM patients, less than 10.5 months between procedures resulted in improved symmetry. There was an average 6.63% improvement in facial symmetry in the CM group, and a 7.67% improvement in the TC group.Based on the UCLA experience, the durability of facial asymmetry and contour correction with fat transplantation is attainable in the craniofacial patient but may also require concomitant skeletal correction in the most severe cases.

BACKGROUND The specialty of plastic surgery has evolved to encompass a wide breadth of both reconstructive and aesthetic surgery. Practitioners in other specialties have increasingly advanced on procedures and areas that have traditionally been served by plastic surgeons. To date, no evaluation and comparison of the operative experience of graduating residents from various specialties has been performed. METHODS The authors review the case log statistical reports of the Accreditation Council for Graduate Medical Education. For each specialty, this annual report highlights the average number of cases performed for all graduating residents. The national case log reports were reviewed for dermatology, ophthalmology, otolaryngology, and plastic surgery. Four procedures (i.e., blepharoplasty, face lift, liposuction, and rhinoplasty) were compared for residents graduating in the 2006 to 2010 academic years. The hypothesis that no difference exists between the average numbers of aesthetic procedures performed by various specialty residency training was tested using a two-sample t statistic. RESULTS For blepharoplasty, face lift, and liposuction, the higher number of cases performed by graduating plastic surgery residents was statistically significant (p < 0.00001) for all years examined. Although plastic surgery trainees graduating from 2006 to 2010 had a higher number of recorded rhinoplasties, this difference in case logs was statistically significant only for plastic surgery residents graduating in 2008. CONCLUSIONS The quantitative operative experience of graduating plastic surgery residents for selected aesthetic surgery cases exceeds that of other surgical subspecialties. Given the exposure and strength of plastic surgery training, plastic surgeons should remain at the forefront of aesthetic surgery.

BACKGROUND: Practitioners in other surgical specialties have increasingly advanced their volume of reconstructive procedures traditionally served by plastic surgeons. Because there has not been a previous specialty training comparison, the average operative reconstructive volume of graduating plastic surgery residents was formally compared with that of other specialties. METHODS: The authors review the case log statistical reports of the Accreditation Council for Graduate Medical Education. For each specialty, this annual report highlights the average number of cases performed for all graduating residents. The national case log reports were reviewed for orthopedic surgery, otolaryngology, and plastic surgery. Six procedures were compared for residents graduating in the 2006 to 2010 academic years and are reviewed. A two-sample Welch-Satterthwaite t test for independent samples with heterogeneous variance was conducted to compare the average number of procedures performed per graduating resident. RESULTS: Graduates of plastic surgery residencies compared with graduates of other specialties performed more cleft lip and palate repairs, hand amputation, hand fracture, and nasal fracture procedures. This difference showed statistical significance for all years examined (2006 to 2010). For repair of mandible fractures, plastic surgery trainees had significantly more cases for 2006 to 2009 but not 2010. CONCLUSIONS: The quantitative operative experience of graduating plastic surgery residents for selected reconstructive cases is above that of the average graduating trainee outside of plastic surgery. Given the exposure and strength of plastic surgery training, plastic surgeons should remain at the forefront of reconstructive surgery.

Despite increasing specialization of craniofacial surgery, certain craniofacial techniques are widely applicable. The authors identified five such craniofacial techniques and queried American Society of Plastic Surgeons members and plastic surgery program directors regarding their comfort level with the procedures and their opinion on resident training for these selected procedures.First, a select group of senior craniofacial surgeons discussed and agreed on the top five procedures. Second, active American Society of Plastic Surgeons were surveyed regarding their opinion on training and their comfort level with each procedure. Third, plastic surgery residency program directors were studied to see which of the top five procedures are taught as part of the plastic surgery residency curriculum.The top five widely applicable craniofacial procedures are technically described and include the following: (1) cranial or iliac bone graft for nasal reconstruction, (2) perialar rim bone graft, (3) lateral canthopexy, (4) osseous genioplasty, and (5) bone graft harvest for orbital floor defects. For practicing plastic surgeons, comfort level in all procedures increased with advancing years in practice (except those with5 years). A majority of plastic surgeons (75 percent), especially those with craniofacial fellowship training, felt competent in all procedures except osseous genioplasty (53 percent). Plastic surgery program directors agreed that all top five procedures should be mastered by graduation.Although program directors felt that all five selected craniofacial procedures should be taught and mastered during residency training, plastic surgeons without craniofacial fellowship training were less comfortable with the techniques. Residency training goals should include competence in core craniofacial techniques.

Summary Purpose Lower eyelid scaring and malposition following violation of all three lamellae pose a significant ophthalmologic reconstructive challenge. The purpose of our study was to document a staged approach for this problem using: 1) transconjunctival scar release followed by palatal graft below the tarsal plate and subciliary scar release followed by full-thickness skin graft superficial to the tarsal plate and 2) subsequent autologous fat grafting to the lower eyelid. Methods Cadaveric anatomic dissections were performed. Post-traumatic and post-surgical lower eyelid deformities requiring reconstruction were reviewed and outcome assessment was based on symptomatic improvement, perioperative complications, reoperations and long-term follow-up (> 1 year). Results Cadaver dissections demonstrated consistent lower eyelid tarsal plate and lamellar anatomy for the use of palatal graft and skin grafting. Clinically, 75% cases resulted from full thickness traumatic laceration of the lower eyelid or malar region and 25% of cases occurred after transconjunctival incisions were made for zygomatic maxillary repositioning following partial lower eyelid laceration. Preoperative symptoms of : epiphora, tearing, redness, blurry vision and dryness improved in all patients and complete resolution was seen in 63% of patients. Thirty-seven percent of patients had complications: Redundancy of palatal graft, Partial FTSG loss, cellulitis after fat transfer. Conclusions We describe an approach for the scarred and displaced lower eyelid following injury to all three lamellae that provided symptomatic improvement after lower lid scar tissue release, lengthening of the contracted septum, support of the posterior lamellae with a palatal graft and a replacement of anterior lamella with full thickness skin graft.

In craniofacial microsomia, patients with severely hypoplastic mandibles (Pruzansky type III) require replacement of the ramus and condyle unit. Autogenous costocartilaginous rib graft and distraction osteogenesis are the most important techniques used, but long-term results need to be looked at to determine optimal management.Of the 485 patients with craniofacial microsomia and mandibular abnormality identified by the authors' craniofacial multidisciplinary clinic, 31 patients were identified with Pruzansky type III mandibles who underwent treatment and were available for study. Patients primarily had either costocartilaginous grafts or mandibular distraction after molar extraction. Outcomes assessed rib failure, undergrowth, or overgrowth. Reoperation included regrafting for graft failure, rib distraction for undergrowth, and mandibular setback for overgrowth. Details surrounding complications for each modality including osteotomy site were recorded.For primary mandibular reconstruction, 27 patients underwent costocartilaginous rib graft surgery (30 grafts, three bilateral) at 9.9 ± 4.1 years; four patients underwent mandibular distraction at 7.4 ± 2.3 years. Rib graft failure in seven of 30 cases (23 percent) required regrafting. Undergrowth in 17 cases (57 percent) required rib distraction. Overgrowth in three cases (10 percent) required correction at the time of orthognathic correction. For rib graft distraction, osteotomy site locations included native mandible (25 percent), rib-mandible junction (19 percent), and rib graft (56 percent). The rib-mandible junction site had graft-related complications (100 percent) that the other sites did not.For the severely hypoplastic mandibles (Pruzansky type III), costocartilaginous grafts are an accepted modality. However, when rib graft growth is insufficient, secondary distraction should be performed within the native mandible or rib graft and not at the rib graft-mandible junction site.

The authors investigated the use of serial autologous fat grafting to restore soft-tissue contour in craniofacial microsomia patients.Patients with moderate to severe craniofacial microsomia were divided into two groups. Microvascular free flap patients had reconstruction with inframammary extended circumflex scapular flaps at skeletal maturity (n = 10). Alternatively, patients had fat grafting during multiple staged operations for mandible and ear reconstruction (n = 21). Sex, age, severity of deformity [determined by OMENS (orbital deformity, mandibular hypoplasia, ear deformity, nerve involvement, and soft-tissue deficiency) classification], number of procedures, operative times, and augmentation volumes were recorded. A digital three-dimensional photogrammetry system was used to determine "final fat take" and symmetry (affected side versus unaffected side). Physician and patient satisfaction were elicited.Microvascular free flap and fat grafting groups had similar OMENS scores, 2.4 and 2.3, and similar mean prereconstruction symmetry scores, 74 percent and 75 percent, respectively. Although the mean number of procedures was less for the microvascular free flap group versus the fat grafting group (2.2 versus 4.3), the combined surgical time was greater for the microvascular free flap group. The complication rate for the microvascular free flap group was 12 percent and that for the fat grafting group was 5 percent. The mean microvascular free flap volume implanted was 131 cc, with a final measured volume of 106 cc. Mean fat grafting volume injected per case was 33 cc, with total fat injections of 146 cc and a final measured volume of 121 cc. There was a mean fat loss of 25 cc and 83 percent fat take. Symmetry score was 121 percent for the microvascular free flap group and 99 percent for the fat grafting group. No statistically significant difference in patient or physician satisfaction was noted.Serial fat grafting provided a useful alternative to microvascular free tissue transfer after skeletal reconstruction.

Given the multiple permutations in craniofacial malformations, classification of median craniofacial dysplasia or midline Tessier no. 0 to 14 clefts has been difficult and disjointed. In this review, the authors present a summary of normal embryology, prior terminology, and their proposed new classification system. Median craniofacial dysplasia has tissue agenesis and holoprosencephaly at one end (the hypoplasias), frontonasal hyperplasia and excessive tissue (the hyperplasias) at the other end, and abnormal splitting or clefting and normal tissue volume (dysraphia) occupying the middle portion of the spectrum. These three distinct subclassifications have different forms of anomalies within their groups.

Background Transsphenoidal encephaloceles are rare cystic herniations of meninges, cerebrospinal fluid, and/or brain matter resulting from incomplete closure of the cranial base and may be associated with midfacial, central nervous system, and endocrine anomalies. Although some centers choose not to operate because of risks, the authors document their staged operative approach to avoid recurrent meningitis, progressive neurologic decline, and other symptoms. Methods Patients with symptomatic transsphenoidal encephaloceles who underwent staged treatment with intracranial and transpalatal cyst correction, facial bipartition, and cleft palate repair were studied (n = 4). Outcome measures included perioperative complications, recurrence, interdacyron distance comparison, and speech and developmental assessments. Results The authors' staged correction of transsphenoidal encephaloceles as detailed in this article proved successful in all four patients, with no recurrence of meningitis, no cerebrospinal fluid leakage, alleviation of headaches, aesthetic improvement, and no encephalocele relapse. Skeletal correction by computed tomographic scan showed correction of interdacyron distance with a mean 22-mm reduction (56 percent). After the initial procedure of encephalocele correction, speech scores fell from 2.2 (borderline incompetent) to 7.9 (incompetent) but improved after the cleft palate repair and speech therapy to 1.4 (borderline competent). Follow-up developmental tests showed normal global evaluations in memory and attention skills in all but one patient (who had persistent deficiencies consistent with preoperative evaluations). Conclusion A staged operative treatment for symptomatic transsphenoidal encephaloceles offers functional and morphologic correction.

The Kapetansky flap was described to repair a whistle deformity or central vermilion defect following a primary bilateral cleft lip repair. The authors studied a modification of this technique, called the Whistle (wide-hinged island swing transposition labial enhancement) flap, to correct a wide array of secondary cleft lip deformities.Patients with secondary cleft lip deformity who underwent the Whistle flap correction were studied (n = 21). Vertical lip height, lateral lip projections, and three-dimensional volumetric measurements were recorded along with physician and parent-patient satisfaction surveys.From 1994 to 2006, 23 Whistle flap procedures were performed on 21 cleft lip patients (bilateral, n = 13; unilateral, n = 8). The average age at surgery was 15.4 years (range, 13 to 21 years). The average follow-up was 27.6 months. The vertical height of the cleft side Cupid's bow to vermilion bottom had a mean increase of 164 percent. Lateral projection of the upper lip showed a mean increase of 117 percent. Preoperatively, patients exhibited mean volumetric asymmetry of 29.4 percent, and this was reduced following treatment to approximately 3.1 percent. Physician satisfaction using a modified Whitaker classification (categories I through IV) demonstrated that 56 percent of patients were category I (no refinements necessary) and 44 percent were category II (minor revisions are advisable). Parent-patient satisfaction in the follow-up period was a mean of 3.5 as measured using a five-point scale ranging from 0 to 4. There were minimal perioperative complications.The Whistle flap procedure provided a versatile and reliable option for the correction of vermilion defects from secondary cleft lip deformities.

With a microform (forme fruste) cleft lip, obtaining an optimal functional and esthetic result is a challenge to a reconstructive surgeon. We describe modifications to existing techniques by Mulliken, Furlow, and Cutting that provides an optimal functional result with correction of the cleft furrow, vermillion notch, and cleft nose deformity.Sequential unilateral microform cleft lip patients underwent our microform cleft lip repair modified from the following 3 techniques: the Mulliken microform cleft lip repair with no cutaneous scar, the Furlow complete cleft lip repair with interdigitating muscle, and the Cutting cleft nose repair (n = 12). From follow-up (1 year) examinations, photographs (and 3-dimensional images), and video, 3 reviewers scored esthetic and functional outcomes using a 0 to 4-point scale. In addition, parental surveys were obtained.For our microform cleft lip repair, there were no infections, bleeding, or other complications. Esthetic outcome score, evaluating the cutaneous scar, symmetry of philtral columns, alignment of lip line and vermillion border, and nasal tip symmetry was a mean of 3.7 (+/-0.3). Thus, the reviewers' scored the cleft lip and nose repair as "Very Good" (minor cleft lip/nose deformity but no need for revision) to "Excellent" (Imperceptible cleft lip or nose deformity). The functional outcome score, based on the ability to smile, pucker their lips, and whistle, was a mean of 3.8 (+/-0.2). The parental survey, including postoperative course, functional, and cosmetic outcome, demonstrated a high level of satisfaction with a score of 3.9 (+/-0.2).The modified microform cleft lip and nose technique provided very good to excellent esthetic and functional results in a series of patients with this rare deformity.

Cell-mediated immunity, especially of human CD8+ cytotoxic T lymphocytes (CTLs) is believed to have an important role in the long-term survival of pig islet xenografts. Protection against human CD8+ CTL cytotoxicity may reduce the direct damage to pig islets and enable long-term xenograft survival in pig-to-human islet xenotransplantation. We have previously reported that c-FLIP S/L genes, which are potent inhibitors of death receptor–mediated proapoptotic signals through binding competition with caspase-8 for recruitment to the Fas-associated via death domain (FADD), markedly suppress human CD8+ CTL-mediated xenocytotoxicity. In addition, the cytoprotective effects of c-FLIP L seem to be significantly stronger than those of c-FLIP S . Accordingly, in the present study, expression of c-FLIP L was induced in intact pig islets by adenoviral transduction. Consequently, the cytoprotective capacity of the transgene in pig islets was examined in in vitro and in vivo exposure to human CD8+ CTLs. Cells from untransduced islets or mock islets were sensitive to CD8+ CTL-mediated lysis (59.3% ± 15.9% and 64.0% ± 8.9% cytotoxicity, respectively). In contrast, cells from pig islets transduced with the c-FLIP L gene were markedly protected from lysis (30.5% ± 3.5%). Furthermore, prolonged xenograft survival was elicited from pig islets transduced with this molecule as assessed using an islet transplant model using the rat kidney capsule. Thus, these data indicate that intact pig islets can be transduced to express c-FLIP L with adenovirus. Pig islets expressing c-FLIP L are significantly resistant to human CTL killing and further exhibit beneficial effects to prolong xenograft survival.

Patients with cloverleaf skull deformity are known to have high morbidity and poor outcome. Physical anomalies include a misshapen, trilobar skull, with a high "bossed" forehead, a bulging temporal region, and a flat posterior skull from multiple cranial suture fusion.Patients with cloverleaf skull deformity treated at the University of California, Los Angeles from 1990 to 2006 (n = 14) underwent early cranial vault remodeling (group 1) or staged correction with ventriculoperitoneal shunt (neonate), fronto-orbital advancement (3 to 6 months), and posterior vault remodeling (1 year) (group 2). Morbidity, necessary revisions, and neurologic (developmental testing) and aesthetic (Whitaker score) outcomes were assessed at follow-up.Diagnoses included Apert syndrome, Crouzon syndrome, Pfeiffer syndrome, Saethre-Chotzen syndrome, and nonsyndromic. Early cranial vault remodeling patients had more complications than staged correction patients [pneumonia, meningitis, and excessive bleeding (each 66 percent versus 9 percent); wound infection (66 percent versus 18 percent); and seizure (100 percent versus 0 percent)]. Early cranial vault remodeling patients had prolonged intensive care unit and hospital stays compared with staged correction patients (13 versus 2 days and 27 versus 5 days, respectively). The Whitaker score showed acceptable results at 18 months in group 2 (1.4, no revisions necessary) but not in group 1 (2.8, minor to major bony recontouring). Developmental tests showed that all early cranial vault remodeling patients had lower scores in both preschool tests and global evaluations compared with normative data (mean preschool receptive scores, 95 versus 85; mean preschool expressive scores, 94 versus 87).Staged correction of cloverleaf skull provided acceptable neurologic and aesthetic outcomes in the authors' series of patients.

Human CD8(+) cytotoxic T lymphocyte (CTL)-mediated cytotoxicity, which participates in xenograft rejection, is mediated mainly by the Fas/FasL apoptotic pathway. We previously developed methods to inhibit human CTL xenocytotoxicity by extracellular remodeling using overexpression of membrane-bound human FasL on pig xenograft cells, and by intracellular blockade of death receptor-mediated apoptotic signals, such as the Fas/FasL pathway using the pig c-FLIP(L) molecule. To investigate the cooperative effects of both membrane-bound FasL and pig c-FLIP(L), we cotransfected both genes into pig endothelial cells (PEC). The double remodeling with these molecules effectively prevented CD8(+) CTL killing. Although double transfectants and single high transfectants of either membrane-bound FasL or c-FLIP(L) gene displayed similar inhibition of CTL cytotoxicity, the expression levels of these 2 molecules in double transfectants were almost half the expression levels of single transfectants. Furthermore, to show in vivo prolongation of xenograft survival, we transplanted PEC transfectants under the rat kidney capsule. Prolonged survival was displayed by PEC double transfectant xenografts whereas those from either parental PEC or MOCK (vehicle control) were completely rejected by day 5 posttransplantation. These data suggested that intracellular and extracellular remodeling by coexpression of membrane-bound FasL and pig c-FLIP(L) in xenograft cells may prevent an innate cellular response to xenografts. The gene compatibility of these molecules to generate transgenic pigs may be sufficient to create a window of opportunity to facilitate long-term xenograft survival.

Background: Patients with craniofacial dysostosis may require correction for hypertelorbitism and midface hypoplasia. Traditionally, these procedures were sequential or staged, and used acute advancement with bone grafting and rigid fixation. The authors reviewed a series of combined, one-stage facial bipartition and monobloc distraction procedures using internal devices. They describe the Roman arch, keystone fixation modification to maximize the stability of the bipartition segments and support the vertical load of the distraction forces. Methods: Patients diagnosed at the University of California, Los Angeles as having craniofacial dysostosis with hypertelorbitism and midface hypoplasia who underwent the Roman arch, keystone fixation procedure were included in the study (n = 13). Reduction of interdacryon and intercanthal distances and the lateral cephalometric horizontal change of the forehead, midface, and maxilla were studied postoperatively and at 1-year follow-up. Results: There were no serious complications (e.g., cerebrospinal fluid leak, meningitis, frontal bone loss); there was a 10 percent rate of total complications (wound infection). Facial bipartition successfully narrowed the interdacryon distance by a mean of 55 percent (21 mm), with only a 3-mm relapse. The mean distraction advancement/relapse was as follows: forehead, +16 mm/-2 mm; midface, 14 mm/-1 mm; and maxilla, 13 mm/-1 mm. Only one of 13 patients required a repeated monobloc procedure. Of the seven patients who reached skeletal maturity, 86 percent underwent a subsequent Le Fort I and/or III procedure. Conclusion: The Roman arch, keystone fixation modification of a combined facial bipartition with monobloc distraction using internal devices provided a stable construct for advancement, with minimal relapse.

A unique situation of twins with similar sagittal synostosis pathology who underwent different surgical corrective procedures allowed us an opportunity to compare an endoscopic technique to the more traditional technique of a modified cranial vault remodeling (CVR). At 4 months of age, 1 twin underwent an endoscopic-assisted extended strip craniectomy with postoperative helmet therapy for 12 months, and the other underwent a reverse pi CVR procedure. Cephalic index, the Whitaker Aesthetic score, and developmental tests were used for comparison during a 6-year follow-up. The modified CVR had a superior outcome compared to the endoscopic procedure with regard to cephalic index measurements (CVR = 77; endoscopic = 63; P < 0.05) and the Whitaker score (CVR = 1 or no revisions necessary; endoscopic = 2.3 or minor to major bony work required for correction). Patients had similar intelligence quotient test scores; however, the endoscopic patient had lower scores in both preschool tests and global evaluations compared with the normal scores of the CVR patient. The modified CVR procedure was superior to the endoscopic procedure, at least in these twins with sagittal synostosis.

Background: Numerous techniques exist to address poor nasal tip projection in the cleft nose deformity. The authors describe a secondary closed rhinoplasty technique using diced cartilage nasal tip grafting. Methods: Cleft patients who previously underwent lower lateral cartilage repositioning with residual poor nasal tip projection underwent the “stuffy nose” rhinoplasty technique in which diced septal cartilage grafts were placed in a pocket made from a unilateral marginal incision over the lower lateral cartilages. Preoperative and follow-up (1 year) comparative measurements included (1) columellar length, (2) alar base–nasal tip–columellar base angle, and (3) lateral tip projection. Results: Twenty cleft patients had improvement in nasal form and tip projection from the stuffy nose rhinoplasty. Mean change from preoperatively to follow-up was as follows: columellar length, 11.3 mm to 13.3 mm (17.7 percent); alar base–nasal tip–columellar base angle, 42.0 to 33.5 degrees (8.5 degrees, or 20.2 percent decrease); and lateral tip projection, 7.7 mm preoperatively to 9.0 mm postoperatively (16.9 percent increase). Two patients developed complications (one graft exposure and one infection) but healed with conservative treatment. Two different patients underwent revisionary nasal surgery but for other concerns (alar base asymmetry and internal nasal valve collapse). Conclusions: The stuffy nose rhinoplasty was shown to objectively improve nasal tip projection in cleft patients with secondary nasal deformities, with minimal complications and decreased need for revisions.

Craniofrontonasal dysplasia is a rare, familial X-linked syndrome with coronal synostosis (brachycephaly or plagiocephaly), hypertelorbitism (frequently asymmetric), and extracranial anomalies. Details of the timing and technique of the craniofacial correction have not been well described. The largest series of patients with craniofrontonasal dysplasia treated at a single institution was used for review.A review of patients at the University of California, Los Angeles Craniofacial Clinic with the diagnosis of craniofrontonasal dysplasia was performed (n = 21). Data included office, hospital, and operative records; photographs; lateral cephalograms; and three-dimensional computed tomographic scans. Based on surgical outcomes, a treatment algorithm was created.Fourteen patients were female, seven were male, and five had a family history of craniofrontonasal dysplasia (24 percent). Eight patients had unilateral coronal synostosis (plagiocephaly) and 13 had bilateral coronal synostosis (brachycephaly). Eleven patients had asymmetric hypertelorbitism and 10 had symmetric hypertelorbitism. Patients also had cleft lip-cleft palate (10 percent), ear deformities (19 percent), strabismus or esotropia (81 percent), dry frizzy hair (100 percent), syndactyly (14 percent), and nail (100 percent) or other anomalies. After fronto-orbital advancement, no patients had increased intracranial pressure problems or difficulty related to resynostosis. After hypertelorbitism correction, three patients relapsed. Because of this, correction in later patients was delayed until after eruption of permanent maxillary incisors. The mean anterior interorbital distance was reduced in patients from 184 percent to 98 percent of sex-matched controls.The phenotypic expression of craniofrontonasal dysplasia is described to recognize patients early. A treatment algorithm for craniofrontonasal dysplasia based on timing and technique is offered to decrease the need for revision and improve outcomes.

Harvest of the autogenous iliac crest bone graft for an alveolar cleft defect (the gold standard) may cause short- and long-term pain and sensory disturbances. To determine if a tissue engineering technique with similar bone healing results offered decreased morbidity, we compared techniques for postoperative donor site pain. Traditional iliac crest bone graft had more donor site complications compared with both tissue engineering and minimally invasive iliac crest bone graft. With donor site pain, traditional had the most patients with pain and tissue engineering had the least patients with pain at all time points. The mean pain score, including both intensity and pain frequency, was greatest at all time points in traditional and least at all time points in tissue engineering. Closure of alveolar cleft defects with a resorbable collagen sponge and bone marrow stem cells resulted in reduced donor site morbidity and decreased donor site pain intensity and frequency.

症例は53歳の男性で, 便秘を主訴に来院. 大腸内視鏡検査で直腸に2型の腫瘍を認め生検にて低分化腺癌と診断され, 低位前方切除術 (D2郭清) を施行された. 組織学的に悪性度の高いカルチノイド腫瘍で, INFb, ss, ly2, v0, n1 (+), stage IIIaであった. 2年5カ月後に局所再発のため, 腫瘤切除術を施行された. 再発組織には核分裂像が多数認められ, Ki-67陽性細胞率68%であり, 内分泌細胞癌と診断された. 化学療法 (CDDP+UFT) を施行するも腫瘍は骨盤内にて増大し, 再手術1年5カ月後に, 再々手術が施行されたが, その5カ月後, 局所再発, リンパ節転移, 脳転移にて原癌死した. 内分泌細胞腫瘍は, 腫瘍細胞の異型度により臨床像に違いが見られるが, 脳転移をきたした内分泌細胞癌を経験したので考察を加え報告する.

Background For repair of cranial vault (skull) defects, alloplastic (methylmethacrylate or titanium mesh) techniques may result in nonhealing or infectious complications and autogenous (split rib or calvarial) techniques may result in excessive blood loss or donor-site morbidity. Osteogenic factors such as bone morphogenetic protein-2 (BMP-2) provide promising alternatives for repairing bone defects. As a new option for this reconstructive challenge, we investigated by using a bilaminar resorbable construct made from computer-aided design and computer-aided manufacturing computed tomographic scan technology with BMP-2 in hopes of providing initial structural support for the skull, followed by bone healing without permanent foreign body problems. Methods Outcomes of consecutive adult patients with long-standing critical-sized cranial vault defects who underwent cranial vault reconstruction between 1999 and 2010 were studied (n = 69). Reconstructive options were titanium mesh, cryopreserved bone, patient-specific implants, autogenous (split calvarial), and a new bilaminar resorbable mesh construct sandwiched with BMP-2. These reconstructive options were compared for operating room time, blood loss, complications, reoperations, and bone healing (3-dimensional computed tomographic scans). Results Defects in the group were similar, 92 cm (60-176 cm). The autogenous bone group had the longest operating room time (1.1 times longer than the BMP-2 construct group) and the greatest blood loss (1.5 times more than the BMP-2 construct group). Perioperative complications and reoperation rates were lower in autogenous bone (5%) and BMP-2 construct groups (14%) when compared with alloplastic groups (22%-36%). Bone healing was superior with BMP-2 construct and autogenous bone (85%; 90%) versus cryopreserved bone (18%) and minimal healing for alloplastic. Conclusions In select cases, a customized bilaminar resorbable construct with BMP-2 resulted in improved bone healing with fewer complications than alloplastic options and without the morbidity and blood loss associated with autogenous bone grafting. This allowed critically sized calvarial defects in adults to be successfully corrected with no residual foreign body.

Background: Treatment of midface hypoplasia and forehead retrusion with monobloc advancement is associated with significant complications, including meningitis, prolonged intubation, and frontal bone flap necrosis. To see whether distraction of the monobloc segment offered decreased morbidity, the authors compared clinical outcomes of patients who underwent conventional monobloc advancement with those of patients who underwent monobloc distraction. Methods: Group 1 (conventional monobloc; n = 12) underwent traditional monobloc advancement with bone grafting. Group 2 (modified monobloc; n = 11) did not receive ventriculoperitoneal shunts and underwent the above procedures with placement of a pericranial flap and fibrin glue over the midline defect. Group 3 (monobloc distraction; n = 24) underwent advancement of the monobloc segment by distraction osteogenesis using internal distraction devices. Complications included meningitis, cerebrospinal fluid leak, frontal bone flap loss, and wound infection. Preoperative, postoperative, and follow-up lateral cephalograms were used to assess horizontal changes of the forehead, midface, and maxilla. Results: Group 3 (distraction monobloc) had the lowest complication rate (8 percent), followed by groups 2 (modified monobloc; 43 percent) and 1 (conventional monobloc; 61 percent) (p < 0.05). Group 3 achieved greater advancement (12.6 mm) than did group 2 (9.4 mm) or group 1 (9.1 mm) (p < 0.05). Relapse was least in group 3 (8 percent) compared with groups 2 (67 percent) and 1 (45 percent). Conclusions: Monobloc advancement by distraction osteogenesis had less morbidity and achieved greater advancement with less relapse compared with conventional methods of acute monobloc advancement with bone grafting. Monobloc distraction is superior to conventional methods of acute monobloc advancement and is an alternative to staged fronto-orbital advancement followed by Le Fort III advancement.

A basal encephalocele is a rare congenital malformation involving a cranial bone defect and cystic-like herniation through the defect. Moyamoya is a rare cerebrovascular disease of unclear etiology involving occlusion of the distal internal carotids and formation of collateral vascular networks. Both diseases have been correlated with optic disc anomalies, hypopituitarism, and midfacial defects. We present a case of a 2-year-old boy with a midline facial cleft who underwent surgical correction of a basal encephalocele. His moyamoya disease may have contributed to a vascular complication. There is growing evidence indicating an overlap in disease profiles for these two rare diseases. In addition, molecular evidence indicates elevated levels of fibroblast growth factor and transforming growth factor in both diseases, suggesting common molecular pathways.

Background: Augmentation of the zygomatic body enhances appearance and provides a more youthful look. Porous hydroxyapatite granules offer an alternative to alloplastic implants. Methods: Hydroxyapatite granules were placed by means of a transconjunctival approach into subperiosteal malar pockets (n = 8). From the preoperative, postoperative, and 1-year follow-up lateral views, malar projection was measured as the right angle distance from the point of malar prominence to the nasale-subnasale line. Results: Patients were either very satisfied (six of eight) or satisfied (two of eight). Malar projection was significantly improved postoperatively and was maintained after 1 year. Conclusion: This technique, performed for cosmetic and reconstructive indications, resulted in measurable improvement in malar projection, minimal complications, and optimal patient satisfaction.

Correction of severe maxillary deficiency in cleft lip-cleft palate patients often results in undercorrection, relapse, and need for secondary corrective procedures. Le Fort I internal distraction osteogenesis offers an alternative to one-step orthognathic advancement, with advantages of gradual lengthening through scar and earlier treatment in growing patients.Patients with cleft lip-cleft palate deformities and maxillary deficiency were divided into three groups treated by Le Fort I advancement: group 1, mild to moderate deficiency (10 mm) with conventional orthognathic procedure; group 2, severe deficiency (or = 10 mm) with conventional orthognathic procedure; and group 3, distraction procedure for severe deficiency (or = 10 mm) (n = 51). Preoperative, postoperative, and follow-up (1 year) lateral cephalogram measurements were compared including angular (SNA and SNB) and linear (Deltax = horizontal and Deltay = vertical) changes. The Pittsburgh Speech Score was used to assess for velopharyngeal insufficiency (score3).Results demonstrated that group 1 patients had a mean SNA change from preoperatively (78.7) to postoperatively (83.8), and a horizontal change of 5.0 mm, with no relapse. Group 2 patients had a mean SNA change from preoperatively (76.3) to postoperatively (82.0) and a horizontal change of 7.2 mm, with 63 percent relapse. Group 3 patients had a mean SNA change from preoperatively (74.1) to postoperatively (84.9) and a horizontal change of 16.5 mm, with 15 percent relapse. Thus, for severe maxillary deficiency, the distraction group had 48 percent less relapse than the conventional Le Fort I group. Postoperative speech evaluation showed velopharyngeal insufficiency in the following: group 1, four of 20 patients (20 percent); group 2, nine of 11 patients (82 percent); and group 3, nine of 20 patients (45 percent).These data suggest that Le Fort I internal distraction for severe cleft maxillary deficiency leads to better dental occlusion, less relapse, and better speech results.

Alloplastic bone substitutes can be used to alter facial contour. In contrast, autogenous bone grafts have a successful 80-year history of restoring facial contour as well as the basic functional support of the craniofacial skeleton. The traditional procedures for harvesting and using autogenous bone grafts are not obsolete. During the past 30 years, the techniques have been refined and new sources have been found, such as calvarial grafts. New tools were required and have been designed to make harvesting of grafts easier and faster for the surgeon and safer and less expensive for the patient. Four short articles under the heading of "Techniques and Tools" are presented addressing the harvesting of (1) iliac, (2) costal, (3) tibial, and (4) calvarial grafts. These articles are based on the experience of six surgeons using the same technique and instruments in more than 20,000 autogenous bone grafting procedures. (These figures represent the group experience as of 2001. Since then, one of the junior coauthors has retired, but the remaining five continue to harvest autogenous bone grafts on a regular basis. So, the group experience as of 2004 is in the range of 23,000 procedures). The-senior surgeon's experience of 9500 procedures spans a period of 50 years (from 1946 to 1996). For the other surgeons (10,500 procedures combined), the collection period was 25 years (from 1975 to 2000).

The particularities of the surgical procedure for the separation of craniopagi twins performed at UCLA in 2002 are presented. The main difficulty for the neurosurgeons was that the twins shared a common sagittal sinus and that one of the sisters had a poorly developed collateral venous system, thus anticipating a set of postoperative clinical problems. During surgery, the response of the brain to the progressive occlusion of the venous bridges was monitored with EEG and Doppler ultrasound. One of the sisters had a large intracerebral hematoma that was evacuated and later developed hydrocephalus, which was treated with a ventriculoperitoneal shunt. One of the sisters had a basically uneventful recovery while the other contracted E. coli meningitis 8 months after surgery, thus impairing her motor and cognitive development. The details and intricacies of the anesthesia and plastic surgery are also outlined.