Impact of surgery timing for craniosynostosis on neurodevelopmental outcomes: a systematic review.

OBJECTIVE: There are currently no guidelines for the optimum age for surgical treatment of craniosynostosis. This systematic review summarizes and assesses evidence on whether there is an optimal age for surgery in terms of neurodevelopmental outcomes. METHODS: The databases MEDLINE, PsycINFO, CINAHL, Embase + Embase Classic, and Web of Science were searched between October and November 2016 and searches were repeated in July 2017. According to PICO (participants, intervention, comparison, outcome) criteria, studies were included that focused on: children diagnosed with nonsyndromic craniosynostosis, aged ≤ 5 years at time of surgery; corrective surgery for nonsyndromic craniosynostosis; comparison of age-at-surgery groups; and tests of cognitive and neurodevelopmental postoperative outcomes. Studies that did not compare age-at-surgery groups (e.g., those employing a correlational design alone) were excluded. Data were double-extracted by 2 authors using a modified version of the Cochrane data extraction form. RESULTS: Ten studies met the specified criteria; 5 found a beneficial effect of earlier surgery, and 5 did not. No study found a beneficial effect of later surgery. No study collected data on length of anesthetic exposure and only 1 study collected data on sociodemographic factors. CONCLUSIONS: It was difficult to draw firm conclusions from the results due to multiple confounding factors. There is some inconclusive evidence that earlier surgery is beneficial for patients with sagittal synostosis. The picture is even more mixed for other subtypes. There is no evidence that later surgery is beneficial. The authors recommend that future research use agreed-upon parameters for: age-at-surgery cut-offs, follow-up times, and outcome measures.

Impact of age at surgery on neurodevelopmental outcomes in sagittal synostosis.

OBJECTIVE: The aim of this study was to ascertain whether age at surgery has an impact on later neurodevelopmental outcomes for children with sagittal synostosis (SS). METHODS: The developmental outcome data from patients who had surgery for SS and who attended their routine preoperative, 6-7 months postoperative, and 5-year-old developmental assessments (yielding general quotients [GQs]) (n = 50), 10-year-old IQ assessment (n = 54), and 15-year-old IQ assessment (n = 23) were examined, comparing whether they had surgery at < 7 months, 7 to < 12 months, or ≥ 12 months). RESULTS: There was no significant effect for age at surgery for GQ at 5 years of age, but there was a significant effect (p = 0.0001) for those undergoing surgery at < 7 months in terms of preoperative gross locomotor deficit that resolved by 6-7 months postoperatively (increase of 22.1 points), and had further improved by 5 years of age (total increase of 29.4 points). This effect was lessened when surgery was performed later (total increase of 7.3 points when surgery was performed at ≥ 12 months). At 10 years of age, 1-way ANOVA showed a significant difference in Full Scale IQ (FSIQ) score (p = 0.013), with the highest mean FSIQ being obtained when surgery was performed at < 7 months of age (score 107.0), followed by surgery at 7 to < 12 months (score 94.4), and the lowest when surgery was performed at ≥ 12 months (score 93.6). One-way ANOVA for the Performance IQ (PIQ) was very similar (p = 0.012), with PIQ scores of 101.4, 91.4, and 87.3, respectively. One-way ANOVA for Verbal IQ (VIQ) was again significant (p = 0.05), with VIQ scores of 111.3, 98.9, and 100.4, respectively. At 15 years, 1-way ANOVA showed a significant difference in PIQ (p = 0.006), with the highest mean PIQ being obtained when surgery was performed at < 7 months (score 104.8), followed by surgery at 7 to < 12 months (score 90.0), and the lowest when surgery was at performed at ≥ 12 months of age (score 85.3). There were no significant results for FSIQ and VIQ, although there was a similar trend for better outcomes with early surgery. CONCLUSIONS: The findings of this study add to the literature that suggests that early surgery for SS may result in improved neurodevelopmental outcomes, with surgery optimally undertaken when patients are < 7 months of age, and with those undergoing surgery at ≥ 12 months performing the least well. These results also have potential implications for ensuring early diagnosis and referral and for the type of surgery offered. Further research is needed to control for confounding factors and to identify the mechanism by which late surgery may be associated with poorer neurodevelopmental outcomes.

A proposal for a new classification of complications in craniosynostosis surgery.

OBJECTIVE Complications have been used extensively to facilitate evaluation of craniosynostosis practice. However, description of complications tends to be nonstandardized, making comparison difficult. The authors propose a new pragmatic classification of complications that relies on prospective data collection, is geared to capture significant morbidity as well as any "near misses" in a systematic fashion, and can be used as a quality improvement tool. METHODS Data on complications for all patients undergoing surgery for nonsyndromic craniosynostosis between 2010 and 2015 were collected from a prospective craniofacial audit database maintained at the authors' institution. Information on comorbidities, details of surgery, and follow-up was extracted from medical records, anesthetic and operation charts, and electronic databases. Complications were defined as any unexpected event that resulted or could have resulted in a temporary or permanent damage to the child. RESULTS A total of 108 operations for the treatment of nonsyndromic craniosynostosis were performed in 103 patients during the 5-year study period. Complications were divided into 6 types: 0) perioperative occurrences; 1) inpatient complications; 2) outpatient complications not requiring readmission; 3) complications requiring readmission; 4) unexpected long-term deficit; and 5) mortality. These types were further subdivided according to the length of stay and time after discharge. The overall complication rate was found to be 35.9%. CONCLUSIONS The proportion of children with some sort of complication using the proposed definition was much higher than commonly reported, predominantly due to the inclusion of problems often dismissed as minor. The authors believe that these complications should be included in determining complication rates, as they will cause distress to families and may point to potential areas for improving a surgical service.

Long-term developmental follow-up in children with nonsyndromic craniosynostosis.

OBJECT: The aim of this study was to determine the distribution of Full Scale IQ (FSIQ) by type of craniosynostosis and to verify the finding that at long-term follow-up, verbal IQ (VIQ) is significantly higher than performance IQ (PIQ) in patients with single-suture sagittal synostosis (SS) despite falling within the "average" range for intelligence. Whether this also occurs in other types of craniosynostosis and whether surgery and sex are relevant were also determined. The relationship between age at time of surgery and later IQ was ascertained. METHODS: The data for 91 children with craniosynostosis (47 sagittal, 15 unicoronal, 13 metopic, 9 multisuture, and 7 bicoronal) were collected at their routine, 10 years of age IQ assessment (mean age 123.8 months). The patients included 61 males and 30 females; 62 patients had undergone surgery and 29 had not. RESULTS: The mean FSIQ for all types of craniosynostosis combined (96.2) fell within the average range for the general population. Some variation was evident across the different types of craniosynostosis: the SS group showed the highest FSIQs and a "normal" distribution of bandings; the other types had a higher proportion of FSIQs in the lower bandings. The data confirmed the finding that VIQ is greater than PIQ despite falling within the average range for intelligence, with a difference of 5.0 for all types of craniosynostosis combined (p = 0.001), 7.6 for the SS group (p = 0.001), and 6.9 for the unicoronal group (p = 0.029). This VIQ > PIQ effect was not found with multisuture craniosynostosis. The VIQ > PIQ discrepancy occurred regardless of whether the patient had undergone surgery and occurred more often in males than females. In the SS group and the bicoronal group, FSIQ (p = 0.036 and p = 0.046, respectively) and PIQ (p = 0.012 and p = 0.017, respectively), though not VIQ, were higher when surgery had been performed early. CONCLUSIONS: The study confirms that at long-term follow-up, although children with nonsyndromic craniosynostosis fall within the normal range for intelligence, there is a VIQ > PIQ discrepancy above what would be expected in the normal population, which may be indicative of more subtle difficulties in achievement. This discrepancy is affected by type of craniosynostosis, sex, and age at time of surgery.

Does patient ethnicity affect site of craniosynostosis?

OBJECT: There are no published papers examining the role of ethnicity on suture involvement in nonsyndromic craniosynostosis. The authors sought to examine whether there is a significant difference in the epidemiological pattern of suture(s) affected between different ethnic groups attending a regional craniofacial clinic with a diagnosis of nonsyndromic craniosynostosis. METHODS: A 5-year retrospective case-notes analysis of all cases involving patients attending a regional craniofacial clinic was undertaken. Cases were coded for the patients' declared ethnicity, suture(s) affected by synostosis, and the decision whether to have surgical correction of synostosis. The chi-square test was used to determine whether there were any differences in site of suture affected between ethnic groups. RESULTS: A total of 312 cases were identified. Of these 312 cases, ethnicity data were available for 296 cases (95%). The patient population was dominated by 2 ethnic groups: white patients (222 cases) and Asian patients (56 cases). There were both more cases of complex synostosis and fewer cases of sagittal synostosis than expected in the Asian patient cohort (χ(2) = 9.217, p = 0.027). CONCLUSIONS: There is a statistically significant difference in the prevalence of the various sutures affected within the nonsyndromic craniosynostosis patient cohort when Asian patients are compared with white patients. The data from this study also suggest that nonsyndromic craniosynostosis is more prevalent in the Asian community than in the white community, although there may be inaccuracies in the estimates of the background population data. A larger-scale, multinational analysis is needed to further evaluate the relationship between ethnicity and nonsyndromic craniosynostosis.

Preoperative and postoperative developmental attainment in patients with sagittal synostosis: 5-year follow-up.

OBJECT: The object of this study was to clarify whether improved developmental attainment following surgical correction of sagittal synostosis (SS), previously identified at initial postoperative assessment, is maintained at longer-term follow-up at 5 years of age. METHODS: The study involved 32 children with SS who underwent corrective surgery at a mean (± SD) age of 8.5 ± 7.25 months (range 2.8-39.9 months). All the children were assessed preoperatively, at 7 months postoperatively, and at 5 years of age, using the Griffiths Mental Development Scales. A control group consisted of 23 children with SS who had received developmental assessment on 2 or more occasions without surgical intervention (8 of these children had had follow-up at 5 years of age). RESULTS: The data indicated that, prior to surgical correction, children with SS had poorer Gross Locomotor function than other areas of development and that, following surgical intervention, the deficit resolved (even where there was severe developmental delay). The results further showed that improvement in Gross Locomotor function observed at 7 months postoperatively was further improved upon by 5 years of age. The same was true for their overall General Quotient, even in those children exhibiting severe developmental delay. Lesser improvements across time were shown for other skill areas. The children with SS who did not undergo surgery did not show any improvement in development, and in fact a deterioration in fine locomotor control was identified in these patients. CONCLUSIONS: The results of this study suggest that corrective surgery for SS has a positive early impact on development, which is maintained and improved upon by 5 years of age, and that this surgery therefore offers more than simply a cosmetic improvement. Furthermore, the results suggest that not operating on children with SS means not only that this opportunity for developmental gain is missed, but that it may also cause an actual deterioration in developmental attainment.

The impact of head and neck cancer and facial disfigurement on the quality of life of patients and their partners.

BACKGROUND: Psychological and physical stresses from head and neck cancer can be substantial for patients and partners. There is minimal research exploring treatment impact, particularly facial disfigurement after surgery. MATERIALS AND METHODS: Twenty-eight surgery and radiotherapy/brachytherapy/chemoradiation patients and 25 of their partners were compared with 23 radiotherapy/brachytherapy patients and 19 partners. Participants completed the Hospital Anxiety and Depression Scale, Psychosocial Adaptation to Illness Scale, Dyadic Adjustment Scale, and European Organisation for Research and Treatment of Cancer Quality Of Life Scale, including the Head and Neck Cancer module. The Dropkin Disfigurement and Dysfunction scale classified surgical impairment. RESULTS: Partners reported greater distress than patients on some scales. Patients did not have a lower quality of life compared with normal populations and other cancer patients. However, on the EORTC they did and were comparable to a normed sample of head and neck cancer patients. Treatment modality was not predictive of psychological vulnerability. DISCUSSION: Head and neck cancer patients do not necessarily experience poor quality of life. The disease can have a significant impact on partners.