Iatrogenic intradural arachnoid cyst following tethered cord release in a child.

Iatrogenic arachnoid cysts represent uncommon complications of intradural spinal procedures. Here we present the case of a 7-year-old girl who was found to have a symptomatic, pathologically proven, intradural arachnoid cyst 3 years following tethered cord release. The patient originally presented with abnormal urodynamics testing and was found to have fatty infiltration in her filum terminale. She underwent sectioning of the filum terminale without complications. The patient presented 3 years later with pain and neurogenic claudication. The patient was successfully treated with subarachnoid cyst fenestration with resolution of her bilateral lower extremity pain. Spinal intradural arachnoid cysts represent an important, though rare, postoperative complication of dural opening.

Considering the Ectopic Pituitary Gland in Evaluation of the Nasopharyngeal Mass.

IMPORTANCE: Extracranial pituitary ectopia is an uncommon finding characterized by all or a portion of the pituitary gland situated in an aberrant location. Often these lesions come to clinical attention only once they begin to function abnormally or increase in size significantly. OBSERVATIONS: We describe an incidentally noted, asymptomatic pedunculated nasopharyngeal lesion in a 13-month-old girl that may be an extracranial pituitary gland. Consideration of this rare anomaly cautioned against performing a biopsy and may have prevented inadvertent removal of functional pituitary tissue. We describe the embryologic origins of ectopic pituitary tissue, discuss the differential diagnosis for nasopharyngeal lesions in children, and emphasize physical and radiologic findings suggestive of ectopic pituitary tissue to prevent potential inadvertent removal of this rare anomaly. CONCLUSIONS AND RELEVANCE: We report this unusual case to review embryologic origins of ectopic pituitary tissue and to alert otolaryngologists of the need to consider ectopic pituitary tissue in the diagnosis of nasopharyngeal lesions in children.

Acquired cholesteatoma presenting as a pars squamosa temporal bone mass.

Acquired cholesteatomas typically arise in the middle ear and mastoid cavities; they rarely present elsewhere. We describe a case of acquired cholesteatoma that presented as a large mass of the pars squamosa of the temporal bone in a 16-year-old girl. The mass was surgically removed without complication. To the best of our knowledge, this is only the second reported case of an acquired cholesteatoma in the lateral temporal bone.

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

PURPOSE: 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. METHODS: Case records were reviewed for 40 children with medically refractory epilepsy at the time of their referral for presurgical evaluation. Patients received a clinical psychiatric evaluation and parents completed the Child Behavioral Checklist (CBCL). Seizure localization was verified by pediatric epileptologists, and suitability for surgical procedures was verified by neurosurgical specialists. Groups were compared based on localization of seizure foci, either in the temporal lobe or predominantly extratemporal. KEY FINDINGS: The majority of the sample had psychiatric diagnoses and behavior problems, well beyond the level reported in chronic epilepsy populations. In addition, children with temporal lobe seizure foci had more CBCL behavioral problem categories rated in the clinically significant range, and also were more likely to have clinical diagnoses of depression. SIGNIFICANCE: Routine psychiatric evaluation prior to epilepsy surgery may be important for pediatric patients with medically refractory epilepsy. Psychiatric illness, particularly depression, may be especially prominent for those with temporal lobe seizure foci.

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 $7652.33 (10.9 percent of total charges) for intensive care unit care and $2470.62 (6.9 percent of total charges) for floor care. CONCLUSIONS: In this study, 4.7 percent of patients had event/interventions requiring intensive care unit care after fronto-orbital advancement. Predictors included preexisting end-organ dysfunction and higher intraoperative blood loss requiring greater resuscitation. Financial savings from selective postoperative intensive care unit admission may not outweigh the potential cost of an emergent event on the surgical floor.

Age at initial consultation for craniosynostosis: comparison across different patient characteristics.

BACKGROUND: The severity and dysmorphology that results from the premature fusion of one or more cranial sutures is not uniform. Less striking phenotypes may be more easily missed on routine screening, possibly leading to delayed diagnosis and treatment. The purpose of this study was to compare the age at initial presentation for the different forms of craniosynostosis. METHODS: The authors reviewed the records of all patients who underwent open craniofacial repair of craniosynostosis at a single institution from 1996 to 2009. Relationships between type of suture fusion and age at initial consultation were compared. RESULTS: Two hundred eleven patients (136 males, 75 females) were identified. Indications included sagittal (n = 96), metopic (n = 39), unicoronal (n = 33), bicoronal (n = 24), multisutural (n = 15), bilambdoidal (n = 3), and unilambdoidal (n = 1) synostoses. Seventeen patients (8.1%) had a craniosynostosis syndrome and 5 (2.4%) had a syndrome or disorder not typically associated with craniosynostosis [X-linked hypophosphatemic rickets (n = 3), achondroplasia (n = 1), and Beckwith Wiedemann (n = 1)]. Median age at initial consultation was 4.1 months; there was no gender difference. Patients with X-linked hypophosphatemic rickets presented at a significantly older age than nonsyndromic patients or those with a known craniosynostosis syndrome. Those with multisutural synostosis presented at a significantly older age than patients with sagittal or bicoronal synostosis. CONCLUSIONS: Patients with multisutural involvement or X-linked hypophosphatemic rickets had a significant delay in presentation for craniosynostosis. The latter group of patients may especially benefit from routine surveillance for craniosynostosis given their advanced age at diagnosis.

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, HR(discharge) = 0.988, P < 0.01), postoperative RCT (HR(discharge) = 0.96, P < 0.05), total RCT (HR(discharge) = 0.991, P < 0.05), and total intraoperative fluid (HR(discharge) = 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.

Controlled hypotension and blood loss during frontoorbital advancement.

OBJECT: Controlled hypotension is routinely used during open repair of craniosynostosis to decrease blood loss, although this benefit is unproven. In this study the authors analyzed the longitudinal relationships between intraoperative mean arterial pressure (MAP) and calculated blood loss (CBL) during frontoorbital advancement (FOA) for craniosynostosis. METHODS: The authors reviewed the records of infants with craniosynostosis who had undergone primary FOA between 1997 and 2009. Anesthesia records provided preoperative and serial intraoperative MAP. Interval measures of CBL had been determined during the course of the operation. The longitudinal relationships between MAP(mean), MAP(change), and CBL(change) were assessed over the same time interval and compared between adjacent time intervals to determine the directionality of associations. RESULTS: Ninety infants (44 males and 46 females) underwent FOA at a mean age and weight of 10.7 ± 12.9 months and 9.0 ± 7.0 kg, respectively. The average intraoperative MAP was 56.1 ± 4.8 mm Hg, 22.6 ± 12.1% lower than preoperative baseline. A negative correlation was found between CBL(change) and MAP(mean) over the same interval (r = -0.31, p < 0.05), and an inverse relationship was noted between CBL(change) of the previous interval and MAP(change) of the next interval (r = -0.07, p < 0.05). Finally, there was no significant association between MAP(change) of the previous interval and CBL(change) of the next interval. CONCLUSIONS: Calculated blood loss demonstrated a negative correlation with MAP during FOA. Directionality testing indicated that MAP did not affect intraoperative blood loss; instead, blood loss drove changes in MAP. Overall, these findings challenge the benefit of controlled hypotension during open craniofacial repair.

Factors related to blood loss during fronto-orbital advancement.

BACKGROUND: Blood 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. METHODS: The 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. RESULTS: Ninety 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. CONCLUSIONS: Greater 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.

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 patient's 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.

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, Fisher's 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.