Amanda L. Yaun

Long-term outcomes of primary craniofacial reconstruction for craniosynostosis: a 12-year experience.

Background: The purpose of this study was to critically assess long-term outcomes after open reconstruction of craniosynostosis within the recent decade. Methods: The authors performed a retrospective, institutional review board–approved review of open repair for craniosynostosis between 1997 and 2009. Surgical factors, complications, and long-term outcomes were assessed. Pearson chi-square, Fishers exact, and Kaplan-Meier analyses were performed. Results: Of 212 patients, 72 underwent primary extended synostectomy and 140 had traditional open craniofacial repair. Mean follow-up was 36.3 months (range, 0.5 to 138 months). Indications included sagittal (n = 96), metopic (n = 40), unicoronal (n = 33), bicoronal (n = 24), multisutural (n = 15), bilambdoidal (n = 3), and unilambdoidal (n = 1) synostoses; 8.5 percent of patients were syndromic. Surgical reconstruction was performed at a mean age of 11.3 months (range, 0.2 to 117.8 months), including nonsyndromic patients at an average age of 10.6 months and syndromic patients at age 19.3 months. There were no deaths. A 3.3 percent complication rate included two cerebral contusions, two hematomas, one cerebrospinal fluid leak, one infection, and one wound breakdown. Patients were categorized as 89.2 percent Whitaker class I/II and 10.8 percent Whitaker class III/IV. Major and total reoperation rates were 9.0 percent and 10.8 percent, respectively. Higher total reoperation rate and Whitaker class III/IV distribution significantly correlated with syndromic diagnosis, bicoronal synostosis, and surgical age younger than 6 months. Conclusions: In this experience of contemporary open craniosynostosis surgery, rates of morbidity, mortality, and reoperation were low. These results support the merits of surgical delay, targeting an age of 6 months or older, and may serve as a more accurate metric of comparison to current minimally invasive techniques for craniosynostosis repair.

Psychiatric symptoms in children prior to epilepsy surgery differ according to suspected seizure focus

Children and adolescents with epilepsy have an overrepresentation of psychiatric illness. However, few studies in pediatrics have characterized specific psychiatric conditions associated with seizure localization. In addition, degree to which psychiatric illness may be more prominent in children refractory to standard medical treatment for epilepsy is not known. The aim of this study was to assess psychiatric symptoms in children with medically refractory epilepsy and ascertain whether symptoms were associated with specific localization.

Unreliability of intraoperative estimated blood loss in extended sagittal synostectomies

OBJECT Intraoperative blood loss represents a significant concern during open repair of craniosynostosis, and its reliable measurement remains a serious challenge. In this study of extended sagittal synostectomies, the authors analyzed the relationship between estimated blood loss (EBL) and calculated blood loss (CBL), and investigated predictors of hemodynamic outcomes. METHODS The authors reviewed outcomes in infants with sagittal synostosis who underwent primary extended synostectomies (the so-called Pi procedure) between 1997 and 2009. Patient demographic data, operating time, and mean arterial pressures (MAPs) were recorded. Serial MAPs were averaged for a MAP(mean). The EBL was based on anesthesia records, and the CBL on pre- and postoperative hemoglobin values in concert with transfusion volumes. Factors associated with EBL, CBL, red blood cell transfusion (RBCT), and hospital length of stay (LOS) were investigated. Hemodynamic outcomes were reported as percent estimated blood volume (% EBV), and relationships were analyzed using simple and multiple linear and logistic regression models. A p value < 0.05 was considered significant. RESULTS Seventy-one infants with sagittal synostosis underwent primary extended synostectomies at a mean age and weight of 4.9 months and 7.3 kg, respectively. The average operating time was 1.4 hours, and intraoperative MAP was 54.6 mm Hg (21.3% lower than preoperative baseline). There was no association between mean EBL (12.7% EBV) and mean CBL (23.6% EBV) (r = 0.059, p = 0.63). The EBL inversely correlated with the patients age (r = -0.07) and weight (r = -0.11) at surgery (p < 0.05 in both instances). With regard to intraoperative factors, EBL positively trended with operating time (r = 0.26, p = 0.09) and CBL inversely trended with MAP(mean) (r = -0.04, p = 0.10), although these relationships were only borderline significant. Intraoperative RBCT, which was required in 59.1% of patients, positively correlated with EBL (r = 1.55, p < 0.001), yet negatively trended with CBL (r = -0.40, p = 0.01). Undertransfusion was significantly more common than overtransfusion (40.8% vs 22.5%, p = 0.02, respectively). The mean hospital LOS was 2.3 days and was not significantly associated with patient demographic characteristics, intraoperative factors, blood loss, RBCT, or total fluid requirements. CONCLUSIONS In extended synostectomies for sagittal synostosis, EBL and CBL demonstrated a decided lack of correlation with one another. Intraoperative blood transfusion positively correlated with EBL, but inversely correlated with CBL, with a significantly higher proportion of patients undertransfused than overtransfused. These findings highlight the need for reliable, real-time monitoring of intraoperative blood loss to provide improved guidance for blood and fluid resuscitation.

The influence of lesion volume, perilesion resection volume, and completeness of resection on seizure outcome after resective epilepsy surgery for cortical dysplasia in children.

OBJECT Focal cortical dysplasia (FCD) is one of the most common causes of intractable epilepsy leading to surgery in children. The predictors of seizure freedom after surgical management for FCD are still unclear. The objective of this study was to perform a volumetric analysis of factors shown on the preresection and postresection brain MRI scans of patients who had undergone resective epilepsy surgery for cortical dysplasia and to determine the influence of these factors on seizure outcome. METHODS The authors reviewed the medical records and brain images of 43 consecutive patients with focal MRI-documented abnormalities and a pathological diagnosis of FCD who had undergone surgical treatment for refractory epilepsy. Preoperative lesion volume and postoperative resection volume were calculated by manual segmentation using OsiriX PRO software. RESULTS Forty-three patients underwent first-time surgery for resection of an FCD. The age range of these patients at the time of surgery ranged from 2 months to 21.8 years (mean age 7.3 years). The median duration of follow-up was 20 months. The mean age at onset was 31.6 months (range 1 day to 168 months). Complete resection of the area of an FCD, as adjudged from the postoperative brain MR images, was significantly associated with seizure control (p = 0.0005). The odds of having good seizure control among those who underwent complete resection were about 6 times higher than those among the patients who did not undergo complete resection. Seizure control was not significantly associated with lesion volume (p = 0.46) or perilesion resection volume (p = 0.86). CONCLUSIONS The completeness of FCD resection in children is a significant predictor of seizure freedom. Neither lesion volume nor the further resection of perilesional tissue is predictive of seizure freedom.

Blood loss estimation during fronto-orbital advancement: implications for blood transfusion practice and hospital length of stay.

Background Reliable measurement of intraoperative blood loss remains a serious challenge during correction of craniosynostosis. This study analyzed the relationship between estimated blood loss (EBL) and calculated blood loss (CBL) in fronto-orbital advancement and its implications on blood transfusion practice and hospital length of stay (LOS). Methods The authors reviewed infants who underwent primary fronto-orbital advancement for craniosynostosis (1997–2009). Estimated blood loss was based on anesthesia records and CBL by preoperative/postoperative hemoglobin. Perioperative red blood cell transfusion (RCT) and hospital LOS were recorded. Results Ninety infants were included. Mean EBL was 42.2% of estimated blood volume (% EBV), and CBL was 39.3% EBV, without significant difference (P = 0.23). Bland-Altman analysis revealed that EBL was greater than CBL at lower levels of blood loss (⩽47.0% EBV) and less than CBL at higher levels (>47.0% EBV). Mean intraoperative RCT was 45.8% EBV; overtransfusion was more frequent at lower levels of bleeding, and undertransfusion at higher levels. Postoperative RCT occurred more frequently with greater blood loss. Mean LOS was 3.7 days, increasing with CBL (hazard ratio of discharge, HRdischarge = 0.988, P < 0.01), postoperative RCT (HRdischarge = 0.96, P < 0.05), total RCT (HRdischarge = 0.991, P < 0.05), and total intraoperative fluid (HRdischarge = 0.999, P < 0.05). Conclusions Estimated blood loss is a less accurate marker for CBL at the extremes of blood loss during fronto-orbital advancement. The tendency to overestimate blood loss with less intravascular volume loss can result in unnecessary transfusion, whereas underestimation with greater actual blood loss can lead to delay in resuscitation and longer hospitalization.

Pediatric Incidental Brain Tumors: a Growing Treatment Dilemma

OBJECT Rising numbers of MRI studies performed during evaluations for pediatric disorders have contributed to a significant increase in the number of incidentally found brain tumors. Currently, there is very little literature on the nature of and the preferred treatment for these incidental brain tumors. In this paper the authors review their experience diagnosing and treating these lesions in children as well as the current literature on this topic. METHODS Records from 2 centers were reviewed for incidentally found brain tumors, treatment approaches, and outcomes for both surgical and nonsurgical cohorts. RESULTS Forty-seven children (30 males and 17 females) with a mean age of 8.6 years were found to have incidental brain lesions suspected to be neoplasms. Twenty-five underwent surgery and 22 were observed. Two children in the observation group required surgery at a later stage. Tumor pathology in 24 patients was benign. Only 3 patients had high-grade tumors. All nonsurgically treated lesions were presumed to be low-grade tumors and were followed up for 25 ± 20 months. CONCLUSIONS The discovery of incidental brain tumors on MRI in children poses an increasing challenge. Additional studies are needed to determine the significance as well as the optimal management strategies in this situation.

Factors related to blood loss during fronto-orbital advancement.

BackgroundBlood loss during fronto-orbital advancement (FOA) remains a significant potential source of morbidity. This study explored variables that might correlate with calculated blood loss (CBL) during this procedure. MethodsThe authors reviewed infants with craniosynostosis who underwent primary FOA (1997–2009). Patient demographics, operative time, and mean arterial pressure (MAP) were recorded. Serial MAPs were averaged for a MAPmean and subtracted from preoperative baseline to calculate MAP%decrease. This provided indicators of both absolute and relative hypotension, respectively. Calculated blood loss was based on preoperative/postoperative hemoglobin values and transfusion volumes and accounted for hemodilutional effects. ResultsNinety infants underwent FOA at an average age of 10.7 ±12.9 months and mean weight of 9.0 ± 7.0 kg. Average operative time was 4.2 hours, and intraoperative MAP was 56.1 mm Hg, 22.6% lower than baseline. Mean CBL was 259.3 mL, or 39.3% of estimated blood volume, negatively correlating with surgical age (r = −0.033, P < 0.05) and positively trending with operative time (r = 0.55, P < 0.05). Absolute hypotension was associated with greater blood loss, as demonstrated by an inverse relationship between CBL and MAPmean (r = −0.19, P < 0.05). From the perspective of relative hypotension, no association was found between CBL and MAP%decrease. ConclusionsGreater operative efficiency and deferring operative correction to a later age may diminish blood loss during FOA. The study results also raise serious concerns regarding the hemodynamic benefits of controlled systemic hypotension.

Chondromyxoid fibroma of the frontal bone in a teenager

We report a skull chondromyxoid fibroma with symptomatic intracranial extension causing initial misdiagnosis as a psychiatric disorder in a 14-year-old child. CT performed for work-up of the patient’s “stuffy nose” revealed a large calcified frontal bone mass with extensive intracranial growth. We present this child with the diagnosis of intracranial chondromyxoid fibroma with detailed neuroimaging and neuropathology correlations.

Misidentification of Vagus Nerve Stimulator for Intravenous Access and Other Major Adverse Events

The vagus nerve stimulator has become a standard modality for intractable pediatric epilepsy. We reviewed our experience with major adverse events, after accidental puncture of a stimulator wire by an emergency room physician seeking intravenous access to treat status epilepticus. The Childrens National Medical Center database was reviewed for patients undergoing vagus nerve stimulator placement between January 1988 and June 2006. Patient characteristics, duration of therapy, and treatment-limiting adverse events were noted. Of 62 patients implanted over 8 years, 22 (35%) had adverse events which led to a change in therapy. Adverse events included prominent drooling, coughing, throat discomfort, dysphagia, wound infection, difficulty breathing, vomiting, vocal-cord weakness, lead failure, and iatrogenic (piercing of wire; surgical clipping of wire during revision). Eight patients required nonroutine surgical intervention (13%). There were two unusual case presentations. In a 13-year-old boy with status epilepticus at an outlying emergency department, the stimulator line was pierced in search of intravenous access. In a 25-year-old housepainter, neck paresthesias upon right lateral neck turning were attributed to insufficient strain relief. Treatment-limiting adverse events occurred in approximately one-third of patients. Unanticipated adverse events included misidentification of the wire for intravenous access, clipping of the wire during surgical dissection, and cervical dysesthesias associated with head-turning.

Analysis of routine intensive care unit admission following fronto-orbital advancement for craniosynostosis.

Background: Intensive care unit admission following fronto-orbital advancement for craniosynostosis is routine at most institutions. The authors determined the frequency of postoperative events requiring intensive care unit care that justify this practice. Methods: Infants with craniosynostosis who underwent primary fronto-orbital advancement at a single institution from 1997 to 2011 were included. Patient demographics, operative factors, and hemodynamic outcomes were recorded. Adverse postoperative events/interventions were graded as none (group I); minor (group II), easily managed on a surgical floor; or major (group III), requiring intensive care unit care. Results: One hundred seven infants were included. Average length of hospitalization was 3.7 ± 1.6 days, with 1.3 ± 1.0 days in the intensive care unit and 2.4 ± 1.0 days on the floor. Seventy-eight patients (72.9 percent) were categorized into group I, 24 (22.4 percent) into group II, and five (4.7 percent) into group III. Major events/interventions included prolonged intubation (n = 2), reintubation (n = 2), and continuous positive airway pressure support (n = 1). Preexisting end-organ dysfunction was significantly associated with group III patients, who also had significantly higher intraoperative blood loss requiring greater resuscitation. Mean daily charges were