Aneurysmal bone cyst (ABC) is an uncommon benign, tumorlike lesion of bone that is usually located in the long bones and spine. On rare occasions, ABCs are found in the bones of the cranial vault and skull base, occasionally causing mass effect and cranial nerve findings. In this report the authors detail the case of a patient who presented with incidentally discovered hydrocephalus due to a large ABC of the occipital bone that produced mass effect and obstruction of CSF. The diagnosis, imaging findings, and surgical management of this interesting and rare case are discussed.
Brian R. Curtis, Rory J. Petteys, Christopher T. Rossi, Robert F. Keating and Suresh N. Magge
Benjamin C. Wood, Albert K. Oh, Robert F. Keating, Michael J. Boyajian, John S. Myseros, Suresh N. Magge and Gary F. Rogers
Progressive postnatal pansynostosis (PPP) is a rare form of craniosynostosis that is characterized by a normal head shape, insidious decrease in percentile head circumference, and high rates of elevated intracranial pressure (ICP). This investigation describes the clinical, radiographic, and genetic features of this entity.
The authors’ craniofacial database for the period 1997–2013 was retrospectively culled to identify patients who had a normal or near-normal head shape and CT-confirmed multiple-suture synostosis. Patients with kleeblatt-schädel or previous craniofacial surgery were excluded. All demographic information was collected and analyzed.
Seventeen patients fit the inclusion criteria. Nine patients had a syndromic diagnosis: Crouzon syndrome (n = 4), Pfeiffer syndrome (n = 2), Saethre-Chotzen syndrome (n = 1), Apert syndrome (n = 1), and achondroplasia (n = 1). With the exception of 3 patients with mild turricephaly, all patients had a relatively normal head shape. Patients were diagnosed at an average age of 62.9 months. Nearly all patients had some combination of clinical, radiographic, or ophthalmological evidence of increased ICP.
PPP is insidious; diagnosis is typically delayed because the clinical signs are subtle and appear gradually. All normocephalic infants or children with a known or suspected craniosynostotic disorder should be carefully monitored; any decrease in percentile head circumference or signs/symptoms of increased ICP should prompt CT evaluation.
Benjamin C. Wood, Edward S. Ahn, Joanna Y. Wang, Albert K. Oh, Robert F. Keating, Gary F. Rogers and Suresh N. Magge
Endoscopic strip craniectomy (ESC) with postoperative helmet orthosis is a well-established treatment option for sagittal craniosynostosis. There are many technical variations to the surgery ranging from simple strip craniectomy to methods that employ multiple cranial osteotomies. The purpose of this study was to determine whether the addition of lateral barrel-stave osteotomies during ESC improved morphological outcomes.
An IRB-approved retrospective review was conducted on a consecutive series of cases involving ESC for sagittal craniosynostosis at 2 different institutions from March 2008 to August 2014. The patients in Group A underwent ESC and those in Group B had ESC with lateral barrel-stave osteotomies. Demographic and perioperative data were recorded; postoperative morphological outcomes were analyzed using 3D laser scan data acquired from a single orthotic manufacturer who managed patients from both institutions.
A total of 73 patients were included (34 in Group A and 39 in Group B). Compared with Group B patients, Group A patients had a shorter mean anesthetic time (161.7 vs 195 minutes; p < 0.01) and operative time (71.6 vs 111 minutes; p < 0.01). The mean hospital stay was similar for the 2 groups (1.2 days for Group A vs 1.4 days for Group B; p = 0.1). Adequate postoperative data on morphological outcomes were reported by the orthotic manufacturer for 65 patients (29 in Group A and 36 in Group B). The 2 groups had similar improvement in the cephalic index (CI): Group A, mean change 10.5% (mean preoperative CI 72.6, final 80.4) at a mean follow-up of 13.2 months; Group B, mean change 12.2% (mean preoperative CI 71.0, final 79.6) at a mean follow-up of 19.4 months. The difference was not statistically significant (p = 0.15).
Both ESC alone and ESC with barrel staving produced excellent outcomes. However, the addition of barrel staves did not improve the results and, therefore, may not be warranted in the endoscopic treatment of sagittal craniosynostosis.
Jonathan Roth, Robert F. Keating, John S. Myseros, Amanda L. Yaun, Suresh N. Magge and Shlomi Constantini
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.
Records from 2 centers were reviewed for incidentally found brain tumors, treatment approaches, and outcomes for both surgical and nonsurgical cohorts.
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.
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.
Report of 2 cases
Alan Siu, Gary F. Rogers, John S. Myseros, Siri S. Khalsa, Robert F. Keating and Suresh N. Magge
There is no known correlation between Down syndrome and craniosynostosis. The authors report 2 infants with trisomy 21 and right unilateral coronal craniosynostosis. Both patients were clinically asymptomatic but displayed characteristic craniofacial features associated with each disorder. One patient underwent a bilateral fronto-orbital advancement and the other underwent an endoscopically assisted strip craniectomy with postoperative helmet therapy. Both patients demonstrated good cosmesis at follow-up.
Tina M. Sauerhammer, Albert K. Oh, Michael Boyajian, Suresh N. Magge, John S. Myseros, Robert F. Keating and Gary F. Rogers
Unilateral fusion of the frontoparietal suture is the most common cause of synostotic frontal plagiocephaly. Localized fusion of the frontosphenoidal suture is rare but can lead to a similar, but subtly distinct, phenotype.
A retrospective chart review of the authors' craniofacial database was performed. Patients with isolated frontosphenoidal synostosis on CT imaging were included. Demographic data, as well as the clinical and radiographic findings, were recorded.
Three patients were identified. All patients were female and none had an identifiable syndrome. Head circumference was normal in each patient. The mean age at presentation was 4.8 months (range 2.0–9.8 months); 2 fusions were on the right side. Frontal flattening and recession of the supraorbital rim on the fused side were consistent physical findings. No patient had appreciable facial angulation or orbital dystopia, and 2 patients had anterior displacement of the ipsilateral ear. All 3 patients were initially misdiagnosed with unilateral coronal synostosis, and CT imaging at a mean age of 5.4 months (range 2.1–10.8 months) was required to secure the correct diagnosis. Computed tomography findings included patency of the frontoparietal suture, minor to no anterior cranial base angulation, and vertical flattening of the orbit without sphenoid wing elevation on the fused side. One patient underwent CT scanning at 2.1 months of age, which demonstrated a narrow, but patent, frontosphenoidal suture. The patient's condition was assumed to be a deformational process, and she underwent 6 months of unsuccessful helmet therapy. A repeat CT scan obtained at 10.7 months of age demonstrated the synostosis. All 3 patients underwent fronto-orbital correction at mean age of 12.1 months (range 7.8–16.1 months). The mean duration of postoperative follow-up was 11.7 months (range 1.9–23.9 months).
Isolated frontosphenoidal synostosis should be considered in the differential diagnosis of atypical frontal plagiocephaly.
Siri Sahib S. Khalsa, Alan Siu, Tiffani A. DeFreitas, Justin M. Cappuzzo, John S. Myseros, Suresh N. Magge, Chima O. Oluigbo and Robert F. Keating
Previous studies have indicated an association of Chiari malformation Type I (CM-I) and a small posterior fossa. Most of these studies have been limited by 2D quantitative methods, and more recent studies utilizing 3D methodologies are time-intensive with manual segmentation. The authors sought to develop a more automated tool to calculate the 3D posterior fossa volume, and correlate its changes after decompression with surgical outcomes.
A semiautomated segmentation program was developed, and used to compare the pre- and postoperative volumes of the posterior cranial fossa (PCF) and the CSF spaces (cisterna magna, prepontine cistern, and fourth ventricle) in a cohort of pediatric patients with CM-I. Volume changes were correlated with postoperative symptomatic improvements in headache, syrinx, tonsillar descent, cervicomedullary kinking, and overall surgical success.
Forty-two pediatric patients were included in this study. The mean percentage increase in PCF volume was significantly greater in patients who showed clinical improvement versus no improvement in headache (5.89% vs 1.54%, p < 0.05) and tonsillar descent (6.52% vs 2.57%, p < 0.05). Overall clinical success was associated with a larger postoperative PCF volume increase (p < 0.05). These clinical improvements were also significantly associated with a larger increase in the volume of the cisterna magna (p < 0.05). The increase in the caudal portion of the posterior fossa volume was also larger in patients who showed improvement in syrinx (6.63% vs 2.58%, p < 0.05) and cervicomedullary kinking (9.24% vs 3.79%, p < 0.05).
A greater increase in the postoperative PCF volume, and specifically an increase in the cisterna magna volume, was associated with a greater likelihood of clinical improvements in headache and tonsillar descent in patients with CM-I. Larger increases in the caudal portion of the posterior fossa volume were also associated with a greater likelihood of improvement in syrinx and cervicomedullary kinking.
Suresh N. Magge, Arthur R. Bartolozzi IV, Neil D. Almeida, Deki Tsering, John S. Myseros, Chima O. Oluigbo, Gary F. Rogers and Robert F. Keating
Sagittal craniosynostosis is managed with a wide variety of operative strategies. The current investigation compares the clinical outcomes of two widely performed techniques: pi craniectomy and minimally invasive endoscopic strip craniectomy (ESC) followed by helmet therapy.
This IRB-approved retrospective study examined patients diagnosed with nonsyndromic, single-suture sagittal craniosynostosis treated with either pi craniectomy or ESC. Included patients had a minimum postoperative follow-up of 5 months.
Fifty-one patients met the inclusion criteria (pi 21 patients, ESC 30 patients). Compared to patients who underwent ESC, the pi patients were older at the time of surgery (mean age 5.06 vs 3.11 months). The mean follow-up time was 23.2 months for ESC patients and 31.4 months for pi patients. Initial cranial index (CI) was similar between the groups, but postoperatively the ESC patients experienced a 12.3% mean increase in CI (from 0.685 to 0.767) compared to a 5.34% increase for the pi patients (from 0.684 to 0.719), and this difference was statistically significant (p < 0.001). Median hospital length of stay (1 vs 2 days) and operative duration (69.5 vs 93.3 minutes) were significantly less for ESC (p < 0.001 for both). The ESC patients showed a trend toward better results when surgery was done at younger ages. Craniectomy width in ESC cases was positively associated with CI improvement (slope of linear regression = 0.69, p = 0.026).
While both techniques effectively treated sagittal craniosynostosis, ESC showed superior results compared to pi craniectomy. ESC showed a trend for better outcomes when done at younger ages, although the trend did not reach statistical significance. A wider craniectomy width (up to 2 cm) was associated with better outcomes than smaller craniectomy widths among the ESC patients.
Orgest Lajthia, Jerry W. Chao, Max Mandelbaum, John S. Myseros, Chima Oluigbo, Suresh N. Magge, Christopher S. Zarella, Albert K. Oh, Gary F. Rogers and Robert F. Keating
Intracranial empyema is a life-threatening condition associated with a high mortality rate and residual deleterious neurological effects if not diagnosed and managed promptly. The authors present their institutional experience with immediate reimplantation of the craniotomy flap and clarify the success of this method in terms of cranial integrity, risk of recurrent infection, and need for secondary procedures.
A retrospective analysis of patients admitted for management of intracranial empyema during a 19-year period (1997–2016) identified 33 patients who underwent emergency drainage and decompression with a follow-up duration longer than 6 months, 23 of whom received immediate bone replacement. Medical records were analyzed for demographic information, extent and location of the infection, bone flap size, fixation method, need for further operative intervention, and duration of intravenous antibiotics.
The mean patient age at surgery was 8.7 ± 5.7 years and the infections were largely secondary to sinusitis (52.8%), with the most common location being the frontal/temporal region (61.3%). Operative intervention involved removal of a total of 31 bone flaps with a mean surface area of 22.8 ± 26.9 cm2. Nearly all (96.8%) of the bone flaps replaced at the time of the initial surgery were viable over the long term. Eighteen patients (78.3%) required a single craniotomy in conjunction with antibiotic therapy to address the infection, whereas the remaining 21.7% required more than 1 surgery. Partial bone flap resorption was noted in only 1 (3.2%) of the 31 successfully replaced bone flaps. This patient eventually had his bone flap removed and received a split-calvaria bone graft. Twenty-one patients (91.3%) received postoperative CT scans to evaluate bone integrity. The mean follow-up duration of the cohort was 43.9 ± 54.0 months.
The results of our investigation suggest that immediate replacement and stabilization of the bone flap after craniectomy for drainage of intracranial empyemas has a low risk of recurrent infection and is a safe and effective way to restore bone integrity in most patients.
Matthew F. Sacino, Cheng-Ying Ho, Jonathan Murnick, Tammy Tsuchida, Suresh N. Magge, Robert F. Keating, William D. Gaillard and Chima O. Oluigbo
Previous meta-analysis has demonstrated that the most important factor in seizure freedom following surgery for focal cortical dysplasia (FCD) is completeness of resection. However, intraoperative detection of epileptogenic dysplastic cortical tissue remains a challenge, potentially leading to a partial resection and the need for reoperation. The objective of this study was to determine the role of intraoperative MRI (iMRI) in the intraoperative detection and localization of FCD as well as its impact on surgical decision making, completeness of resection, and seizure control outcomes.
The authors retrospectively reviewed the medical records of pediatric patients who underwent iMRI-assisted resection of FCD at the Children's National Health System between January 2014 and April 2015. Data reviewed included demographics, length of surgery, details of iMRI acquisition, postoperative seizure freedom, and complications. Postsurgical seizure outcome was assessed utilizing the Engel Epilepsy Surgery Outcome Scale.
Twelve consecutive pediatric patients (8 females and 4 males) underwent iMRI-guided resection of FCD lesions. The mean age at the time of surgery was 8.8 years ± 1.6 years (range 0.7 to 18.8 years), and the mean duration of follow up was 3.5 months ± 1.0 month. The mean age at seizure onset was 2.8 years ± 1.0 year (range birth to 9.0 years). Two patients had Type 1 FCD, 5 patients had Type 2A FCD, 2 patients had Type 2B FCD, and 3 patients had FCD of undetermined classification. iMRI findings impacted intraoperative surgical decision making in 5 (42%) of the 12 patients, who then underwent further exploration of the resection cavity. At the time of the last postoperative follow-up, 11 (92%) of the 12 patients were seizure free (Engel Class I). No patients underwent reoperation following iMRI-guided surgery.
iMRI-guided resection of FCD in pediatric patients precluded the need for repeat surgery. Furthermore, it resulted in the achievement of complete resection in all the patients, leading to a high rate of postoperative seizure freedom.