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  • Author or Editor: John Myseros x
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Benjamin C. Wood, Albert K. Oh, Robert F. Keating, Michael J. Boyajian, John S. Myseros, Suresh N. Magge and Gary F. Rogers

OBJECT

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.

METHODS

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.

RESULTS

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.

CONCLUSIONS

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.

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Jonathan Roth, Robert F. Keating, John S. Myseros, Amanda L. Yaun, Suresh N. Magge and Shlomi Constantini

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.

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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.

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Tina M. Sauerhammer, Albert K. Oh, Michael Boyajian, Suresh N. Magge, John S. Myseros, Robert F. Keating and Gary F. Rogers

Object

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.

Methods

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.

Results

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).

Conclusions

Isolated frontosphenoidal synostosis should be considered in the differential diagnosis of atypical frontal plagiocephaly.

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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

OBJECTIVE

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.

METHODS

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.

RESULTS

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).

CONCLUSIONS

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.

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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

OBJECTIVE

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.

METHODS

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.

RESULTS

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).

CONCLUSIONS

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.

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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

OBJECTIVE

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.

METHODS

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.

RESULTS

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.

CONCLUSIONS

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.

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Matthew F. Sacino, Cheng-Ying Ho, Matthew T. Whitehead, Amy Kao, Dewi Depositario-Cabacar, John S. Myseros, Suresh N. Magge, Robert F. Keating, William D. Gaillard and Chima O. Oluigbo

OBJECTIVE

Focal cortical dysplasia (FCD) is a common cause of medically intractable epilepsy that often may be treated by surgery. Following resection, many patients continue to experience seizures, necessitating a decision for further surgery to achieve the desired seizure outcomes. Few studies exist on the efficacy of reoperation for intractable epilepsy due to FCD in pediatric cohorts, including the definition of prognostic factors correlated with clinical benefit from further resection.

METHODS

The authors retrospectively analyzed the medical records and MR images of 22 consecutive pediatric patients who underwent repeat FCD resection after unsuccessful first surgery at the Children's National Health System between March 2005 and April 2015.

RESULTS

Accounting for all reoperations, 13 (59%) of the 22 patients achieved complete seizure freedom and another 5 patients (23%) achieved significant improvement in seizure control. Univariate analysis demonstrated that concordance in electrocorticography (ECoG) and MRI localization (p = 0.005), and completeness of resection (p = 0.0001), were associated with seizure freedom after the first reoperation. Patients with discordant ECoG and MRI findings ultimately benefited from aggressive multilobe lobectomy or hemispherectomy. Repeat lesionectomies utilizing intraoperative MRI (iMRI; n = 9) achieved complete resection and seizure freedom in all cases.

CONCLUSIONS

Reoperation may be clinically beneficial in patients with intractable epilepsy due to FCD. Patients with concordant intraoperative ECoG and MRI localization may benefit from extended resection of residual dysplasia at the margins of the previous lesional cavity, and iMRI may offer benefits as a quality control mechanism to ensure that a complete resection has been accomplished. Patients with discordant findings may benefit from more aggressive resections at earlier stages to achieve better seizure control and ensure functional plasticity.