Care Standardization Reduces Blood Donor Exposures and Transfusion in Complex Cranial Vault Reconstruction.

BACKGROUND: Complex cranial vault reconstruction (CCVR) often requires a large-volume transfusion of blood products. We implemented a series of improvement interventions to reduce blood donor exposures (BDE) and transfusion requirements in CCVR. METHODS: We implemented interventions over 4 epochs: (E1) reconstituted blood (1:1 ratio of donor-matched red blood cells and fresh-frozen plasma) for intraoperative transfusions, (E2) reconstituted blood plus postoperative transfusion guidelines, (E3) reconstituted blood plus intraoperative antifibrinolytics and postoperative guidelines, and (E4) fresh whole blood for intraoperative transfusion, antifibrinolytics, and postoperative guidelines. Primary outcomes, BDE, and total volume of blood products transfused are presented by using statistical process control charts, with statistical comparisons between each epoch and baseline data. RESULTS: We included 347 patients <72 months old who underwent CCVR between 2008 and 2016 (E1: n = 50; E2: n = 41; E3: n = 87; and E4: n = 169). They were compared with a baseline sample group of 138 patients who were managed between 2001 and 2006. Compared with our baseline group, patients in each epoch had a significant reduction in BDE (P = .02-<.0001). Conversely, compared with the baseline group, we observed an increase the volume of blood products transfused in E1 (P = .004), no difference in E2 (P = .6) or E3 (P = .46), and a reduction in the volume of blood products transfused in E4 (P < .0001). CONCLUSIONS: The implementation of sequential clinical improvement strategies resulted in a sustained reduction in BDE whereas only the use of whole blood resulted in a significant reduction in the total volume of blood products transfused in children undergoing CCVR.

Morphological, functional and neurological outcomes of craniectomy versus cranial vault remodeling for isolated nonsyndromic synostosis of the sagittal suture: a systematic review.

BACKGROUND: Craniosynostosis is a condition characterized by the premature closure of one or more of the cranial vault sutures. It can occur alone or in association with other congenital defects and may be part of a syndrome. The sagittal suture is most commonly affected, comprising 40-60% of cases. Premature fusion of the sagittal suture can cause scaphocephaly due to compensatory anterior-posterior growth of the skull. This is morphologically considered as a narrow elongated skull with a decreased cephalic index, and is diagnosed clinically and/or radiologically. Both the indications for surgery and the techniques used have varied with time and location. Surgical techniques have evolved, from limited craniectomy to calvarial remodeling. In recent times a return to craniectomy methods has occurred with the more recent introduction of endoscopic methods. OBJECTIVES: The objectives of this review were to identify and synthesize the best available evidence on the morphological, functional and neurological outcomes of craniectomy compared to cranial vault remodeling. INCLUSION CRITERIA: This review considered studies of infants with primary isolated sagittal synostosis operated on or before the mean age of 24 months. The intervention of interest was local craniectomy and this was compared to cranial vault remodeling. Morphological (primary), functional and neurological (secondary) outcomes were included. Mortality, complications and aesthetic outcome were included as tertiary outcomes. METHODS: A comprehensive search was undertaken across major databases. The retrieved studies were assessed by two independent reviewers for methodological validity prior to inclusion. Data was then extracted and, where possible, pooled in statistical meta-analysis. For descriptive studies, where statistical pooling was not possible, the findings are presented in narrative form. RESULTS: Search and retrieval: Based on critical appraisal, 27 studies were considered to be suitable for this review. These studies were all descriptive in nature. Meta-analysis was only possible for the primary morphological outcome (post-operative cephalic index).Morphological (cephalic index):At one year follow-up, post-operatively remodeling offers an advantage over craniectomy (Z = 4.16, P<0.0001)Morphological:Improvements of the cephalic index to varying degrees were seen in patients receiving either procedure and there is not enough evidence to suggest that either treatment group had greater improvement over the other.Functional and neurological:Although their global scores may be comparable to an age-matched population, patients with sagittal synostosis who have undergone a surgical repair of any type may have discrepancies in specific domains and may be at risk of developing learning disorders. There is insufficient primary research with inter-procedure comparisons of preoperative and postoperative cognitive and neurological outcomes.Tertiary outcomes:There is not enough evidence to comment on mortality or postoperative infection in either treatment group. Patients undergoing cranial vault remodeling have a higher rate of transfusion compared to those undergoing craniectomy; however, it is likely that this difference relates to elective transfusion based on hospital-specific protocols. It remains unknown whether there is an inherently higher need for transfusion in patients undergoing remodeling procedures. Delaying surgery however may increase the risk of raised intracranial pressure (ICP) and its associated complications. Whilst there is no evidence for raised ICP post-craniectomy, a few studies have shown raised ICP in patients post-remodeling. There is not enough evidence to establish a relationship between both procedures and raised ICP. Aesthetic outcome appears to be "better" in patients who undergo remodeling; however, there is little rigorous evidence to support this hypothesis. CONCLUSIONS: Conclusions were drawn from both the meta-analysis and the narrative results.When comparing the mean change in cephalic index one year after surgery, remodeling was shown to be superior to limited craniectomy in patients with isolated synostosis of the sagittal suture. However both procedures were seen to give improvements at short, medium and longer term time points. Improvements in cephalic index may be sustained, deteriorate or improve over time; based on the current data neither procedure offers a clear long-term advantage over the other. Longer follow-up is required to compare outcomes at different time points.Patients who have surgery (any type) for isolated sagittal synostosis may have deficiencies in different subdomains at later school-age testing, whilst maintaining an age-appropriate global intelligence quotient (IQ) and school performance. There is no evidence to suggest that surgery of either type imparts any benefit in terms of functional or neurological outcomes.There is no evidence to suggest that surgery of either type imparts any benefit in terms of functional or neurological outcomes. While school performance and general IQ may be comparable to age-matched controls, patients with sagittal synostosis who have undergone surgical repair of any type may be at risk of deficiencies in sub-areas of testing and be at risk of learning disorders.There is insufficient evidence regarding mortality, infection, postoperative ICP and aesthetic outcome. While transfusion rates were greater in the remodeling group, this may be due to higher rates of elective transfusion.The inconclusive findings indicate an ongoing need for higher quality primary research comparing the morphological and functional outcomes of craniectomy and cranial vault remodeling in primary sagittal synostosis. Outcomes should be measured in both the short and long term.

Analysis of morbidity and mortality in surgical management of craniosynostosis.

Multidisciplinary care involving plastic surgery and neurosurgery is generally accepted as optimal to manage craniosynostosis to avoid complications and to identify patients at risk. We conducted a retrospective 30-year review of craniosynostosis surgery at a single major craniofacial institute to establish the rate and predictors of complications. Medical records of 796 consecutive patients who underwent primary surgery for craniosynostosis from 1981 to 2010 at our institute were analyzed for complications. Complications were defined as intraoperative and postoperative adverse events requiring changed management. Reoperation was defined as a repeat transcranial procedure. Multivariate logistic regression was used to identify predictors for complications or revision. Across the years, the procedures evolved from technically simple to complex, which increased complications but better outcomes. Complications occurred in 111 patients (14%), and 33 (5.4%) needed major revision. Multivariate analysis identified multisuture and syndromic craniosynostosis, more recent surgeries, younger age (<9 months), spring-assisted cranioplasty, longer surgery, and greater transfusion as predictors of complications. Patients with syndromic and multisutural craniosynostosis and those operated on younger than 9 months had increased risk of major revision surgery for regression to phenotype. Our experience over 30 years indicates that pediatric transcranial craniosynostosis surgery can be safely carried out in our tertiary referral center. There were no deaths from primary surgery, and complication and reoperation rates mirror those of other published studies. Syndromic and complex craniosynostosis predicted both complications and need for major revision. Spring cranioplasty was associated with higher complications. Overall results support a recommended age for craniosynostosis surgery between 9 and 12 months.

Craniosynostosis: early recognition prevents fatal complications.

BACKGROUND: Craniosynostosis is the premature fusion of cranial vault sutures. The overall incidence is 3-5/10,000 live births. With multiple craniosynostoses, brain growth may be impeded by the unyielding skull. Most cases of single suture involvement can be treated with linear excision of suture. Involvement of multiple sutures or skull has usually required combined efforts of neurosurgeons and craniofacial surgeons. METHODS: On the basis of visible skull deformity all patients were admitted in the Department of Neurosurgery, Liaquat University Hospital, Jamshoro, Pakistan. Patients were examined for signs of raised ICP and other congenital deformities. The records of patients were maintained till follow up. RESULTS: Twenty-seven children were included in this study from 2002 to 2009. Age range was 1-6 years, boys were 18 (66.6%), and girls were 9 (33.3%). The common suture affected was coronal 12 (44.4%). Two children with craniostenosis belonged to same family, and all presented with suture involvement. Three (11.1%) deaths occurred due to hypothermia (1), and blood loss (2). CONCLUSION: Early diagnosis, expert surgical techniques and per- and postoperative care for bleeding and temperature regulation prevent mortality and morbidity.

Perioperative factors associated with prolonged intensive care unit and hospital length of stay after pediatric neurosurgery.

OBJECTIVES: This study aims to describe the association between different postoperative complications and the length of hospital stay among children undergoing neurosurgical procedures. METHODS: A retrospective cohort study was carried out between May 2004 and May 2009 in a tertiary community hospital. All postoperative complications following neurosurgical procedures and their association with the main outcomes [length of intensive care unit (ICU) and hospital stay] were investigated in a univariate and multivariate analysis. RESULTS: The medical records of 198 patients treated during the study period were reviewed. The most frequently performed surgeries were ventriculoperitoneal shunting (16.7%), correction of craniosynostosis (30%) and brain tumor resections (28.3%). Of the 198 patients eligible for this analysis, 79 (39.9%) suffered from at least one complication. The most frequent complications were fever (30.3%), hypothermia (16%), postextubation laryngitis (15.1%) and postoperative bleeding (7%). Factors independently associated with a longer pediatric ICU stay were fever (odds ratio 1.39, 95% confidence interval 1.1-3.2; p = 0.001), laryngitis (odds ratio 2.24, 95% confidence interval 1.8-5.2; p = 0.001), postoperative bleeding requiring reoperation (odds ratio 1.8, 95% confidence interval 1.4-3.9; p < 0.001) and infection (odds ratio 3.71, 95% confidence interval 1.8-12.4; p = 0.033). Fever (odds ratio 2.54, 95% confidence interval 2-7.4; p = 0.001) and infection (odds ratio 11.23, 95% confidence interval 4-22.4; p = 0.003) were related to the total length of the patient's hospital stay. CONCLUSIONS: In this study population, most elective neurosurgical procedures were not associated with significant complications, and morbidity and mortality were low. Some complications significantly influenced patients' outcomes and should be monitored for early diagnosis. This study may improve our understanding and identification of postoperative outcomes in pediatric neurosurgery.

Outcome data and analysis in pediatric neurosurgery.

OBJECTIVE: The purpose of this study was to analyze the outcomes of five commonly performed pediatric neurosurgical operations: 1) initial shunt insertion; 2) first shunt revision; 3) craniotomy for brain tumor; 4) correction of sagittal synostosis; and 5) release of tethered cords. A second purpose was to analyze the neurological outcome data after tethered cord releases. METHODS: Morbidity and mortality records, patient charts, and operative records were reviewed to determine length of hospitalization and, for each disorder, the pertinent outcomes such as duration of shunt function and incidence of infection or neurological morbidity. RESULTS: Many outcome data were expected, such as a high long-term shunt function rate after primary shunt insertion (65% at 5 yr), a low mortality rate (1%) and permanent morbidity rate (10%) after craniotomy for brain tumor, and a low frequency of transfusion (20%) for sagittal synostosis operations. The outcomes among the three neurosurgeons varied more than expected, e.g., the duration of hospitalization after sagittal reconstructions ranged from 3.1 to 5.8 days; the frequency of infections of primary shunt revisions ranged from 0 to 15%; and the neurological morbidity after tethered cord releases ranged from 0 to 12%, with all neurological morbidity occurring in patients undergoing their second or third tether release. CONCLUSION: The data may serve as a basis for outcome comparisons for these procedures. Outcome data allow us to analyze factors to improve patient care, but outcome analysis is complex.

Surgical treatment of craniosynostosis: outcome analysis of 250 consecutive patients.

OBJECTIVE: Surgery for craniosynostosis has evolved rapidly over the past two decades, with increased emphasis on early, extensive operations. Older published series may not accurately reflect more recent experience. Our study was designed to analyze outcome in a large series of consecutive patients treated recently at a single center. METHODS: We reviewed 250 consecutive patients who underwent surgical treatment of craniosynostosis between January 1, 1987 and December 31, 1992. They were divided into nine groups by suture involvement: sagittal, unilateral coronal, bilateral coronal, unilateral lambdoid, bilateral lambdoid, metopic, multiple suture, the Klee-blattschädel deformity (cloverleaf skull), and acquired craniosynostosis. Outcome was analyzed in terms of residual deformities and irregularities, complications, mortality, as well as the need for additional surgery. RESULTS: There were 157 males (62. 8%) and 93 females (37.2%), with most of the male preponderance accounted for by the large sagittal synostosis group, which consisted of 82 males and 25 females. Median age at first operation was 147 days. A named syndrome was present in 23 patients (9.2%) and was more common than expected with bilateral and unilateral coronal synostosis, the Kleeblattschädel deformity, and multiple suture synostosis. There were two deaths (0.8%), both with Klee-blattschädel patients, and 17 other complications (6.8%). Morbidity and mortality were significantly associated with secondary vs primary operations and syndromic vs nonsyndromic patients. Outcome analysis revealed the best surgical results with metopic synostosis and significantly less good results with the Kleeblattschädel deformity, multiple suture synostosis, and bilateral coronal synostosis. CONCLUSIONS: Using modern surgical techniques, craniosynostosis can be corrected with good outcomes and relatively low morbidity and mortality, particularly for otherwise healthy, nonsyndromic infants.

Synostectomy versus complex cranioplasty for the treatment of sagittal synostosis.

Although pediatric neurosurgeons traditionally have been trained to perform a sagittal synostectomy for the treatment of sagittal synostosis, numerous articles advocating modifications of this procedure or more complex cranial vault reconstructions point our the inadequacies of strip craniectomy in providing optimal short- and long-term cosmesis. This review addresses the major arguments for and against performing complex cranioplasties for sagittal synostosis, as well as issues regarding timing of surgery, mortality and morbidity, increased need for blood transfusion, and increased expense. The authors support the contention that for the majority of infants with sagittal synostosis, complex calvarial vault reconstruction provides a better outcome with little increase in operative risk.

Surgical management of the cloverleaf skull deformity.

The cloverleaf skull deformity, or Kleeblattschädel, is a rare malformation caused by synostosis of multiple cranial sutures. This anomaly has been reported to carry a dismal prognosis both in terms of neurological outcome as well as cosmetic appearance if treatment is delayed. Due to the paucity of data concerning the results of early operative intervention, it remains uncertain whether aggressive craniofacial decompressive/reconstructive procedures are effective in ameliorating the effects of the malformation on both neurological development and cosmetic appearance. This paper reports the treatment and outcome of 7 children with the cloverleaf skull malformation treated at our institution between 1981 and 1993. All children underwent an initial decompressive craniectomy with the removal of at least 50% of the cranial vault for relief of high intracranial pressure in early infancy. Our first 4 patients underwent near total calvariectomy whereas the 3 children treated subsequently have undergone a staged approach with anterior followed by posterior craniectomies with bone morcellation and replacement. Subsequent reconstructions, intended to further improve the cosmetic appearance, were performed later in infancy or in early childhood. Follow-up ranges from 17 months to 9 years, with a mean of 61 months. Long-term results are reported with regard to neurological outcome as well as normalization of skull shape in terms of both the cephalic indices and general cosmetic appearance. Of the 4 children initially treated with total calvariectomy, only 1 child is neurologically normal and has a good cosmetic appearance. One child is severely impaired neurologically following a sagittal sinus thrombosis suffered during a secondary reconstructive procedure.(ABSTRACT TRUNCATED AT 250 WORDS)

[Premature craniosynostosis--cause of sudden death in children and young adults]. / Prämature Kraniosynostosis--Ursache plötzlicher Todesfälle im Kindes- und jungen Erwachsenenalter.

Primary premature craniosynostosis ordinarily leads to characteristic skull abnormalities, if the ossification of the sutures happens in early childhood and involves all sutures, the elevation of intracranial pressure may not cause severe clinical features and the head morphology may be normal. A disproportion between skull capacity and brain volume will be the result. Three cases are reported, where these preconditions combined with exterior effects like minimal trauma, physical activity or consumption of alcohol or medicaments caused sudden death. Important autoptic findings are ossified cranial sutures with the absence of suture lines and increased scalloping of the internal table of the skull (impressiones digitatae).

Craniofrontonasal dysplasia: clinical and genetic analysis.

We have identified a case of craniofrontonasal dysplasia which demonstrates the potential lethality of this gene. Genetic analysis of this pedigree and nine others reveals that craniofrontonasal dysplasia does not follow a Mendelian mode of inheritance and may be a human mutation analogous to the T-locus of mice.