Establishing a colorectal cancer research database from routinely collected health data: the process and potential from a pilot study.

OBJECTIVE: Colorectal cancer is a common cause of death and morbidity. A significant amount of data are routinely collected during patient treatment, but they are not generally available for research. The National Institute for Health Research Health Informatics Collaborative in the UK is developing infrastructure to enable routinely collected data to be used for collaborative, cross-centre research. This paper presents an overview of the process for collating colorectal cancer data and explores the potential of using this data source. METHODS: Clinical data were collected from three pilot Trusts, standardised and collated. Not all data were collected in a readily extractable format for research. Natural language processing (NLP) was used to extract relevant information from pseudonymised imaging and histopathology reports. Combining data from many sources allowed reconstruction of longitudinal histories for each patient that could be presented graphically. RESULTS: Three pilot Trusts submitted data, covering 12 903 patients with a diagnosis of colorectal cancer since 2012, with NLP implemented for 4150 patients. Timelines showing individual patient longitudinal history can be grouped into common treatment patterns, visually presenting clusters and outliers for analysis. Difficulties and gaps in data sources have been identified and addressed. DISCUSSION: Algorithms for analysing routinely collected data from a wide range of sites and sources have been developed and refined to provide a rich data set that will be used to better understand the natural history, treatment variation and optimal management of colorectal cancer. CONCLUSION: The data set has great potential to facilitate research into colorectal cancer.

Prognostic factors for survival and ambulatory status at 8 weeks with metastatic spinal cord compression in the SCORAD randomised trial.

BACKGROUND: Metastatic spinal cord compression (MSCC) carries a poor prognosis and management is based on the likelihood of maintaining mobility and predicted survival. PATIENTS AND METHOD: SCORAD is a randomised trial of 686 patients comparing a single dose of 8 Gy radiotherapy with 20 Gy in 5 fractions. Data was split into a training set (412, 60%) and a validation set (274, 40%). A multivariable Cox regression for overall survival (OS) and a logistic regression for ambulatory status at 8 weeks were performed in the training set using baseline factors and a backward selection regression to identify a parsimonious model with p ≤ 0.10. Receiver Operating Characteristic (ROC) analysis evaluated model prognostic performance in the validation set. Validation of the final survival model was performed in a separate registry dataset (n = 348). RESULTS: The survival Cox model identified male gender, lung, gastrointestinal, and other types of cancer, compression at C1-T12, presence of non-skeletal metastases and poor ambulatory status all significantly associated with worse OS (all p < 0.05). The ROC AUC for the selected model was 75% (95%CI: 69-81) in the SCORAD validation set and 68% (95%CI: 62-74) in the external validation registry data. The logistic model for ambulatory outcome identified primary tumour breast or prostate, ambulatory status grade 1 or 2, bladder function normal and prior chemotherapy all significantly associated with increased odds of ambulation at 8 weeks (all p < 0.05). The ROC AUC for the selected model was 72.3% (95% CI 62.6-82.0) in the validation set. CONCLUSIONS: Primary breast or prostate cancer, and good ambulatory status at presentation, are favourable prognostic factors for both survival and ambulation after treatment.

Developing an Agnostic Risk Prediction Model for Early AKI Detection in Cancer Patients.

Acute kidney injury (AKI) is a common complication among oncology patients associated with lower remission rates and higher mortality. To reduce the impact of this condition, we aimed to predict AKI earlier than existing tools, to allow clinical intervention before occurrence. We trained a random forest model on 597,403 routinely collected blood test results from 48,865 patients undergoing cancer treatment at The Christie NHS Foundation Trust between January 2017 and May 2020, to identify AKI events upcoming in the next 30 days. AKI risk levels were assigned to upcoming AKI events and tested through a prospective analysis between June and August 2020. The trained model gave an AUROC of 0.881 (95% CI 0.878-0.883), when assessing predictions per blood test for AKI occurrences within 30 days. Assigning risk levels and testing the model through prospective validation from the 1st June to the 31st August identified 73.8% of patients with an AKI event before at least one AKI occurrence, 61.2% of AKI occurrences. Our results suggest that around 60% of AKI occurrences experienced by patients undergoing cancer treatment could be identified using routinely collected blood results, allowing clinical remedial action to be taken and disruption to treatment by AKI to be minimised.

Outcomes following front-line chemotherapy in peripheral T-cell lymphoma: 10-year experience at The Royal Marsden and The Christie Hospital.

We evaluated the outcomes for patients with peripheral T-cell lymphoma (PTCL) undergoing front-line chemotherapy at our institutions between 2002 and 2012. One hundred and fifty-six patients were eligible, comprising PTCL not otherwise specified (NOS) (n = 50, 32.0%), angioimmunoblastic T-cell lymphoma (AITL) (n = 44, 28.2%), anaplastic large-cell lymphoma (ALCL) ALK negative (n = 23, 14.7%), ALCL ALK positive (n = 16, 10.3%), and other (n = 23, 14.7%). Most patients received CHOP (66.0%) and 13.0% received an autologous hematopoietic progenitor cell transplant (HPCT). With a median follow-up of 63.4 months, 5-year overall survival (OS) and progression-free survival (PFS) was 38.8% and 19.8% respectively. Independent risk factors for inferior OS were age >60 years, International Prognostic Index (IPI) ≥ 2 and lack of complete response to induction. When responding patients were compared by receipt of an autologous HPCT versus not, HPCT was associated with improved PFS (p = .001) and OS (p = .046) and remained significant for PFS in multivariate analysis suggesting a possible therapeutic benefit.

A combined single high-dose rate brachytherapy boost with hypofractionated external beam radiotherapy results in a high rate of biochemical disease free survival in localised intermediate and high risk prostate cancer patients.

INTRODUCTION: Dose escalation has been shown to improve biochemical outcome in localised prostate cancer. An HDR brachytherapy boost is an effective strategy for dose escalation, since it exploits the low α/ß ratio in prostate cancer, allowing the delivery of a high biological dose to the tumour. We sought to evaluate the biochemical disease free survival in patients with intermediate and high risk localised prostate cancer treated with EBRT plus HDR brachytherapy as a boost, in our institution. PATIENTS AND METHODS: Biochemical outcome was collected prospectively in 95 patients treated from 2008 to 2010, with an HDR boost of 12.5Gy followed by EBRT delivered as 37.5Gy in 15 fractions over 3weeks. The ASTRO definition of biochemical failure (2µg/L above PSA nadir) was used as the outcome measure. 61/95 (64%) were classified as high risk (stage>T2b or PSA>20µg/L or Gleason score>7) while 34/95 (36%) were intermediate risk. 92/95 (97%) patients received neoadjuvant androgen deprivation therapy (ADT). Adjuvant hormone therapy was at the discretion of the treating clinician. RESULTS: The median follow-up for the cohort was 65months (range, 18-88) with a 5-year biochemical DFS of 80.5% (95% Confidence Interval [CI]: 72.8-89.0). The prognostic factors used in the analysis model were: clinical stage, presenting PSA, duration of ADT, Gleason score, risk category, prostate volume, D90 and V100. Only presenting PSA (HR 1.03; CI 1.00-1.05, p=0.03) predicted for a poorer biochemical DFS on multivariate analysis. CONCLUSION: These data confirm that EBRT plus a single-fraction HDR brachytherapy boost achieves good biochemical control in a cohort of predominantly high risk patients.

Survival patterns in teenagers and young adults with cancer in the United Kingdom: Comparisons with younger and older age groups.

AIMS: We aimed to describe and compare survival in teenagers and young adults (TYAs) with cancer to that of younger children and older adults, to identify sub-populations at greater or lesser risk of death. METHODS: We compared survival in cancer patients diagnosed in the United Kingdom aged 13-24 years (TYAs) to those aged 0-12 (children) and 25-49 years (adults) using the National Cancer Data Repository. All cases had a first cancer diagnosis between 1st January 2001 and 31st December 2005 with censor date 31st December 2010 or death if earlier. RESULTS: We found six distinct statistically significant survival patterns. In pattern 1, the younger the age-group the better the 1- and 5-year survival (acute lymphoid leukaemia, carcinoma of ovary and melanoma). In pattern 2, TYAs had a worse 5-year survival than both children and young adults (bone and soft tissues sarcomas). In pattern 3, TYAs had a worse 1-year survival but no difference at 5-years (carcinoma of cervix and female breast). In pattern 4, TYAs had better 1-year survival than adults, but no difference at 5 years (carcinoma of liver and intrahepatic bile ducts, germ cell tumours of extra-gonadal sites). In pattern 5, the younger the age-group the better the 5-year survival, but the difference developed after 1-year (acute myeloid leukaemia, carcinoma of colon and rectum). In pattern 6, there was no difference in 1- and 5-year survival between TYAs and adults (testicular germ cell tumours, ovarian germ cell tumours and carcinoma of thyroid). CONCLUSION: TYAs with specific cancer diagnoses can be grouped according to 1- and 5-year survival patterns compared to children and young adults. To further improve survival for TYAs, age-specific biology, pharmacology, proteomics, genomics, clinician and patient behaviour studies embedded within clinical trials are required.

Incidence of glioma in a northwestern region of England, 2006-2010.

BACKGROUND: Gliomas are important because they affect disproportionately high numbers of people of working age and have a poor prognosis. Neurosurgeons were concerned about a possible recent cluster of glioma cases in a northwestern region in England. METHODS: All patients aged 18-89 years in Lancashire and South Cumbria with a histologically confirmed glioma diagnosed at the Royal Preston Hospital between January 1, 2006, and December 31, 2010, were ascertained. Clinical information was extracted from hospital records. Completeness of case referral to Royal Preston Hospital was checked against the National Cancer Registry and National Brain Tumour Registry records for the same period. For a comprehensive assessment of regional incidence, age-standardized incidence rates of all gliomas diagnosed in adults (aged 15 years and older) in the study area were then compared with those for the North West region and England as a whole. Rates for the North West region in defined small area-units ("Middle Super Output Areas") were also investigated to assess any small-area variation in the region during the decade to 2010. RESULTS: There were 435 glioma patients from Lancashire and South Cumbria diagnosed at the Royal Preston Hospital between 2006 and 2010, with case ascertainment verified to be complete by the National Cancer Registration Service. The age-standardized incidence rate of gliomas in the study area was 7.10 per 100,000 in 2006-2010, which was minimally different from the rate for all cancer networks in England over the 10 years from 2001. Small-area analysis confirmed lack of major variation in glioma rates in the North West region of England. CONCLUSION: Glioma incidence rates in England have remained stable by region and over time during the last decade.

Ovarian cancer among 8,005 women from a breast cancer family history clinic: no increased risk of invasive ovarian cancer in families testing negative for BRCA1 and BRCA2.

BACKGROUND: Mutations in BRCA1/2 genes confer ovarian, alongside breast, cancer risk. We examined the risk of developing ovarian cancer in BRCA1/2-positive families and if this risk is extended to BRCA negative families. PATIENTS AND METHODS: A prospective study involving women seen at a single family history clinic in Manchester, UK. Patients were excluded if they had ovarian cancer or oophorectomy prior to clinic. Follow-up was censored at the latest date of: 31/12/2010; ovarian cancer diagnosis; oophorectomy; or death. We used person-years at risk to assess ovarian cancer rates in the study population, subdivided by genetic status (BRCA1, BRCA2, BRCA negative, BRCA untested) compared with the general population. RESULTS: We studied 8005 women from 895 families. Women from BRCA2 mutation families showed a 17-fold increased risk of invasive ovarian cancer (relative risk (RR) 16.67; 95% CI 5.41 to 38.89). This risk increased to 50-fold in women from families with BRCA1 mutations (RR 50.00; 95% CI 26.62 to 85.50). No association was found for women in families tested negative for BRCA1/2, where there was 1 observed invasive ovarian cancer in 1613 women when 2.74 were expected (RR 0.37; 95% CI 0.01 to 2.03). There was no association with ovarian cancer in families untested for BRCA1/2 (RR 0.99; 95% CI 0.45 to 1.88). DISCUSSION: This study showed no increased risk of ovarian cancer in families that tested negative for BRCA1/2 or were untested. These data help counselling women from BRCA1/2 negative families with breast cancer that their risk of invasive ovarian cancer is not higher than the general population.

Cancer at ages 15-29 years: the contrasting incidence in India and England.

BACKGROUND: There has been a steady increase in published research from Europe and North America on the epidemiology of cancers in young people. There are limited data from the developing world. We contrast the incidence of cancer at ages 15-29 years in India and England. PROCEDURE: Malignant neoplasms in those aged 15-29 years registered during 2001-2003 in five urban population-based cancer registries (PBCRs) of India and in eight PBCRs in England were included. Site-based classification was used. Age-standardized incidence rates were expressed per 100,000 person years. RESULTS: In India, 4,864 (5.8%) of 84,450 cases and in England, 8,137 (1.2%) of 65,6752 cancer cases occurred in those aged 15-29 years. For this age group, the incidence rate for males and females in India were 12.91 and 14.19, and in England were 27.75 and 28.88, respectively. In males aged 15-29 years, the three most common cancers in India were leukemia, lymphoma, and central nervous system tumors and in England were cancers of male genital organs, lymphoma, and leukemia. Cancers of female genital organs, breast, and leukemia were most common in females in India and cancers of female genital organs, lymphoma, and melanoma in England. For cancers of mouth, stomach, and gall bladder, the incidence was higher in India. CONCLUSION: Incidence of cancer at ages 15-29 years in England is higher at most sites than in India. Variation in environmental exposures between the two countries might be an explanation. Under-ascertainment of cases and gender bias in seeking healthcare may also influence reported incidence rates in India.

Mortality in neurofibromatosis 1: in North West England: an assessment of actuarial survival in a region of the UK since 1989.

Neurofibromatosis 1 (NF1) is a comparatively common autosomal dominant disorder. However, relatively few studies have assessed lifetime risk; and information about the effect of NF1 on mortality remains uncertain. NF1 patients were identified using The North West regional family Genetic Register, which covers the 4.1 million people living in North West England, including the regions of Greater Manchester, Cheshire and Cumbria. Data relating to tumours and malignancies were obtained from The North West Cancer Intelligence Service. Death data for the general North West population were obtained from the Office of National Statistics. We identified 1186 individuals with NF1, of whom 1023 lived within the strict regional boundaries (constituting a region of North West England bound by The Pennines to the east and Irish Sea to the west, but excluding the conurbation of Liverpool (Merseyside) and the Wirral peninsula) and 131 had died. MPNST and glioma were found to be the two most common causes of reduced life expectancy among NF1 patients. In Kaplan-Meier analyses the median survival for NF1 patients was shown to be 71.5 years, with women living ∼7.4 years longer than men. On average both men and women lived ∼8 years less than their counterparts in the general population. Reduction in life expectancy for NF1 patients was found to be much lower (8 years) than the previously estimated 15-year decrease. Limitations relating to the underreporting of NF1 on death certificates were once again highlighted and should be considered in future investigations.

Reduced morbidity and improved healing with bone morphogenic protein-2 in older patients with alveolar cleft defects.

BACKGROUND: In older cleft patients, alveolar bone grafting may be associated with poor wound healing, graft exposure, recurrent fistula, and failure of tooth eruption. A new procedure using a resorbable collagen matrix with bone morphogenetic protein (BMP)-2 was compared with traditional iliac crest bone graft to close alveolar defects in older patients. METHODS: Skeletally mature patients with an alveolar cleft defect undergoing alveolar cleft repair were divided into either group 1 (BMP-2, experimental) or group 2 (traditional iliac graft, control) (n = 21). Bone healing was assessed with intraoral examination and NewTom scans (three-dimensional, Panorex, periapical films). Donor-site morbidity was determined with pain surveys. Overall cost and length of hospital stay were used to examine economic differences. RESULTS: Preoperative and follow-up (1 year) intraoral examinations revealed fewer complications (11 percent versus 50 percent) and better estimated bone graft take in group 1 compared with group 2. Panorex and three-dimensional computed tomographic scans showed enhanced mineralization in group 1 compared with group 2. Volumetric analysis showed group 1 had a larger percentage alveolar defect filled with new bone (95 percent) compared with group 2 (63 percent). Donor-site pain intensity and frequency were significant in group 2 but not group 1. The mean length of stay was greater for group 2 compared with group 1. In addition, the mean overall cost of the procedure was greater in group 2 ($21,800) compared with group 1 ($11,100). CONCLUSIONS: For this select group of late-presenting alveolar cleft patients, the BMP-2 procedure resulted in improved bone healing and reduced morbidity compared with traditional iliac bone grafting.

Delayed closure of the hard palate leads to speech problems and deleterious maxillary growth.

BACKGROUND: Hard palate cleft closure has been associated with maxillary hypoplasia. The Schweckendiek procedure offers delayed hard palate closure to avoid early subperiosteal dissection and palatal scarring. This study sought to compare single-stage versus delayed hard palate closure for speech outcome and maxillary growth. METHODS: A retrospective outcome study was performed of unilateral cleft lip and palate patients with either delayed hard palate repair with a pinned-retained speech prosthesis (Schweckendiek repair) (group 1, delayed hard palate repair, 1978 to 1983) or single-stage cleft palate repair (group 2, single-stage repair, 1983 to 1988). Patients with complete records to maturity at the University of Pittsburgh Cleft Palate Craniofacial Center (n = 82, two equal groups of 41 patients) were studied. Comparative data were collected from multidisciplinary evaluations, perceptual speech scores, speech tests, and cephalometric analysis. RESULTS: Single-stage cleft palate repair had a lower fistulization rate (11 percent) compared with delayed hard palate repair (58 percent). It also had better speech outcomes compared with delayed hard palate repair: mean speech score, 3.1 versus 7.8; final speech score, 0.9 versus 2.9; velopharyngeal incompetency, 21 percent versus 66 percent; failed video fluoroscopy or nasoendoscopy, 18 percent versus 52 percent; and need for secondary speech procedure, 20 percent versus 63 percent. Single-stage repair showed less maxillary growth disturbance, with class III malocclusion, 31 percent versus 66 percent; cephalometric SNA, 78.2 versus 74.8; need for Le Fort I advancement, 24 percent versus 42 percent; and amount of maxillary advancement required, 6 mm versus 9 mm. CONCLUSION: The delayed cleft palate repair led to worse speech outcomes; thus, the authors' center abandoned this technique in favor of single-stage repair. In addition, their data showed that the delayed cleft palate repair led to deleterious maxillary growth.

Autologous fat transfer national consensus survey: trends in techniques for harvest, preparation, and application, and perception of short- and long-term results.

BACKGROUND: Despite a perceived interest in autologous fat transfer, there is no consensus as to the best technique or the level of success. The purpose of the present study was to determine the national trends in techniques for harvest, preparation, and application of autologous fat, as well as the success perceived by practitioners. METHODS: Comprehensive surveys were sent to 650 randomly selected members of the American Society for Aesthetic Plastic Surgery. The survey was aimed at determining whether autologous fat transfer is a commonly performed procedure and, if so, the specific methods involved and the subjective perception of short- and long-term results. RESULTS: The results of the national consensus survey from 508 surgeons (78 percent return rate) showed the following: (1) autologous fat transfer is a relatively common procedure (57 percent perform >10 annually), but few perform it in high volume (only 23 percent perform >30 annually); (2) techniques for harvest, preparation, and injection rarely deviate from methods discussed in the literature (microcannula, 54 percent; centrifugation, 75 percent; injection in nasolabial fold > lips > nasojugal folds); (3) most physicians believe that at least some graft survival is clinically evident (93 percent); and (4) patients are pleased with the short-term results (good to excellent, 84 percent), despite a lower rate of long-term patient satisfaction (fair to good, 80 percent). CONCLUSION: Currently, plastic surgeons across the country report a uniformity of autologous fat grafting techniques with acceptable patient satisfaction.

Monobloc advancement by distraction osteogenesis decreases morbidity and relapse.

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.

Genioplasty distraction osteogenesis and hyoid advancement for correction of upper airway obstruction in patients with Treacher Collins and Nager syndromes.

BACKGROUND: Treacher Collins and Nager syndromes may present with mandibular hypoplasia that causes posterior collapse of the tongue base and a decreased oropharyngeal airway. Mandibular distraction and orthognathic advancement are effective treatments to correct the airway, but failure may occur despite achieving class I occlusion. For this select population, the authors propose a novel procedure of genioplasty distraction and hyoid advancement to optimize epiglottal positioning. METHODS: Patients diagnosed with Treacher Collins (n = 5) or Nager syndrome (n = 3) with obstructive sleep apnea or tracheostomy dependency (n = 8) underwent genioplasty distraction and hyoid advancement. Airway outcome was assessed by preoperative and 1-year follow-up comparison of (1) laryngobronchoscopy, (2) sleep studies, and (3) tracheostomy dependency. For genioplasty outcome, three groups were used: group I (distraction genioplasty, syndromic) (n = 8), group II (acute genioplasty, syndromic) (n = 7), and group III (acute genioplasty, nonsyndromic) (n = 10). Lateral cephalogram measurements were used in the preoperative, postoperative, and follow-up periods to assess horizontal and vertical advancement and relapse. RESULTS: Epiglottal position was optimized by the procedure in all patients based on direct endoscopic assessment. All five patients with obstructive sleep apnea had resolution of symptoms, and two of three patients achieved removal of their tracheostomy. Mean advancement for groups I, II, and III was 25, 14, and 8 mm, respectively. Follow-up horizontal advancement for groups I, II, and III were 18, 4, and 6 mm, respectively. Cephalometric measurements showed a horizontal relapse for groups I, II, and III of 10, 62, and 11 percent, respectively. CONCLUSIONS: Data suggest that genioplasty distraction allows for a greater advancement and decreased relapse rate than acute procedures alone; and genioplasty distraction with hyoid advancement is a useful technique for resolution of obstructive sleep apnea or to achieve tracheostomy removal in those syndromic patients who have already undergone mandibular advancement into a class I occlusion.

Improved malar projection with transconjunctival hydroxyapatite granules.

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.

Improved outcomes in cleft patients with severe maxillary deficiency after Le Fort I internal distraction.

BACKGROUND: 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. METHODS: 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 (score > 3). RESULTS: 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). CONCLUSION: 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.

Case report of optic atrophy in pansynostosis: an unusual presentation of scalp edema from hair braiding.

Pansynostosis (fusion of all cranial sutures) and optic atrophy were found as incidental CT scan and ophthalmological findings in an 8-year-old who presented to the emergency room with scalp edema from tight 'cornrow' hair braiding. Cranial vault expansion was successfully performed. Ophthalmological problems have stabilized but have not reversed. Late presentation of craniosynostosis and the pathophysiology of secondary optic atrophy are discussed.