Matthew D. Smyth, Marissa J. Tenenbaum, Christian B. Kaufman and Alex A. Kane
Although most patients with sagittal craniosynostosis are recognized and treated in infancy, some children are not referred to craniofacial centers until later in childhood. In this paper the authors describe a novel operative technique for calvarial reconstruction in older children with previously untreated sagittal craniosynostosis.
The authors report a clinical series of eight patients who were treated using novel single-stage calvarial reconstruction, and they assess the complications and outcomes. The patient is placed supine for the procedure, which consists of a coronal incision, bifrontal craniotomy without orbital osteotomy, and multiple interlocking midline parietooccipital osteotomies and recontouring. Fixation is achieved using a bioabsorbable plate system. Cranial indices were calculated from measurements obtained before and after the reconstructive procedures. Preoperative, intraoperative, and postoperative photographs and three-dimensional computed tomography scans are presented for review.
Between November 2003 and April 2005, the authors treated seven boys (age range ~ 1–10 years, mean age 4.2 years) with uncorrected sagittal craniosynostosis and one with bicoronal and sagittal synostosis. The mean operating time was 5.13 hours (range 4.3–8 hours), with a mean blood loss of 425 ml (range 200–800 ml). As a percentage of the estimated circulating blood volume, the mean operative blood loss was 33.5% (range 17–57%). The mean hospital stay was 4.9 days. The cranial index significantly improved from a mean of 65.6 to 71.3% (p = 0.001). No acute or delayed complications have been noted. Follow-up examinations performed at an average of 12 months (range 1–17 months) have confirmed early patient and family satisfaction.
An approach of aggressive calvarial reconstruction with multiple interleaving osteotomies crossing the midline achieves improvements in biparietal narrowing. Combined with a bifrontal reconstruction, early outcomes are excellent, with an acceptable amount of intraoperative blood loss and no significant complications.
Case report and review of the literature
James M. Johnston, Nilesh A. Vyas, Alex A. Kane, David W. Molter and Matthew D. Smyth
✓Epignathus, a congenital oropharyngeal teratoma, is a rare clinical entity with variable clinical outcomes described in the literature. Even fewer cases of epignathus with intracranial extension have been reported, all with poor outcomes. In this manuscript, the authors present a case of epignathus with intracranial extension, emphasizing clinical presentation, imaging findings, a staged surgical approach, multidisciplinary management, and outcome.
Timothy W. Vogel, Albert S. Woo, Alex A. Kane, Kamlesh B. Patel, Sybill D. Naidoo and Matthew D. Smyth
The surgical management of infants with sagittal synostosis has traditionally relied on open cranial vault remodeling (CVR) techniques; however, minimally invasive technologies, including endoscope-assisted craniectomy (EAC) repair followed by helmet therapy (HT, EAC+HT), is increasingly used to treat various forms of craniosynostosis during the 1st year of life. In this study the authors determined the costs associated with EAC+HT in comparison with those for CVR.
The authors performed a retrospective case-control analysis of 21 children who had undergone CVR and 21 who had undergone EAC+HT. Eligibility criteria included an age less than 1 year and at least 1 year of clinical follow-up data. Financial and clinical records were reviewed for data related to length of hospital stay and transfusion rates as well as costs associated with physician, hospital, and outpatient clinic visits.
The average age of patients who underwent CVR was 6.8 months compared with 3.1 months for those who underwent EAC+HT. Patients who underwent EAC+HT most often required the use of 2 helmets (76.5%), infrequently required a third helmet (13.3%), and averaged 1.8 clinic visits in the first 90 days after surgery. Endoscope-assisted craniectomy plus HT was associated with shorter hospital stays (mean 1.10 vs 4.67 days for CVR, p < 0.0001), a decreased rate of blood transfusions (9.5% vs 100% for CVR, p < 0.0001), and a decreased operative time (81.1 vs 165.8 minutes for CVR, p < 0.0001). The overall cost of EAC+HT, accounting for hospital charges, professional and helmet fees, and clinic visits, was also lower than that of CVR ($37,255.99 vs $56,990.46, respectively, p < 0.0001).
Endoscope-assisted craniectomy plus HT is a less costly surgical option for patients than CVR. In addition, EAC+HT was associated with a lower utilization of perioperative resources. Theses findings suggest that EAC+HT for infants with sagittal synostosis may be a cost-effective first-line surgical option.
Manish N. Shah, Alex A. Kane, J. Dayne Petersen, Albert S. Woo, Sybill D. Naidoo and Matthew D. Smyth
This study investigated the differences in effectiveness and morbidity between endoscopically assisted wide-vertex strip craniectomy with barrel-stave osteotomies and postoperative helmet therapy versus open calvarial vault reconstruction without helmet therapy for sagittal craniosynostosis.
Between 2003 and 2010, the authors prospectively observed 89 children less than 12 months old who were surgically treated for a diagnosis of isolated sagittal synostosis. The endoscopic procedure was offered starting in 2006. The data associated with length of stay, blood loss, transfusion rates, operating times, and cephalic indices were reviewed.
There were 47 endoscopically treated patients with a mean age at surgery of 3.6 months and 42 patients with open-vault reconstruction whose mean age at surgery was 6.8 months. The mean follow-up time was 13 months for endoscopic versus 25 months for open procedures. The mean operating time for the endoscopic procedure was 88 minutes, versus 179 minutes for the open surgery. The mean blood loss was 29 ml for endoscopic versus 218 ml for open procedures. Three endoscopically treated cases (6.4%) underwent transfusion, whereas all patients with open procedures underwent transfusion, with a mean of 1.6 transfusions per patient. The mean length of stay was 1.2 days for endoscopic and 3.9 days for open procedures. Of endoscopically treated patients completing helmet therapy, the mean duration for helmet therapy was 8.7 months. The mean pre- and postoperative cephalic indices for endoscopic procedures were 68% and 76% at 13 months postoperatively, versus 68% and 77% at 25 months postoperatively for open surgery.
Endoscopically assisted strip craniectomy offers a safe and effective treatment for sagittal craniosynostosis that is comparable in outcome to calvarial vault reconstruction, with no increase in morbidity and a shorter length of stay.
Amy Lee, Andrea E. Van Pelt, Alex A. Kane, Thomas K. Pilgram, Daniel P. Govier, Albert S. Woo and Matthew D. Smyth
Deformational plagiocephaly (DP) is the leading cause of head shape abnormalities in infants. Treatment options include conservative measures and cranial molding. Pediatric neurosurgeons and craniofacial plastic surgeons have yet to agree on an ideal therapy, and no definable standards exist for initiating treatment with helmets. Furthermore, there may be differences between specialties in their perceptions of DP severity and need for helmet therapy.
Requests to participate in a web-based questionnaire were sent to diplomates of the American Board of Pediatric Neurological Surgery and US and Canadian members of the Pediatric Joint Section of the American Association of Neurological Surgeons and the Congress of Neurological Surgeons and the American Cleft Palate–Craniofacial Association. Questions focused on educational background; practice setting; volume of DP patients; preferences for evaluation, treatment, follow-up; and incentives or deterrents to treat with helmet therapy. Six examples of varying degrees of DP were presented to delineate treatment preferences.
Requests were sent to 302 neurosurgeons and 470 plastic surgeons, and responses were received from 71 neurosurgeons (24%) and 64 plastic surgeons (14%). The following responses represented the greatest variations between specialties: 1) 8% of neurosurgeons and 26% of plastic surgeons strongly agreed with the statement that helmet therapy is more beneficial than conservative therapy (p < 0.01); and 2) 25% of neurosurgeons and 58% of plastic surgeons would treat moderate to severe DP with helmets (p < 0.01).
Survey responses suggest that neurosurgeons are less likely to prescribe helmet therapy for DP than plastic surgeons. Parents of children with DP are faced with a costly treatment decision that may be influenced more strongly by referral and physician bias than medical evidence.
Afshin Salehi, Katherine Ott, Gary B. Skolnick, Dennis C. Nguyen, Sybill D. Naidoo, Alex A. Kane, Albert S. Woo, Kamlesh B. Patel and Matthew D. Smyth
The goal of this study was to identify the rate of neosuture formation in patients with craniosynostosis treated with endoscope-assisted strip craniectomy and investigate whether neosuture formation in sagittal craniosynostosis has an effect on postoperative calvarial shape.
The authors retrospectively reviewed 166 cases of nonsyndromic craniosynostosis that underwent endoscope-assisted repair between 2006 and 2014. Preoperative and 1-year postoperative head CT scans were evaluated, and the rate of neosuture formation was calculated. Three-dimensional reconstructions of the CT data were used to measure cephalic index (CI) (ratio of head width and length) of patients with sagittal synostosis. Regression analysis was used to calculate significant differences between patients with and without neosuture accounting for age at surgery and preoperative CI.
Review of 96 patients revealed that some degree of neosuture development occurred in 23 patients (23.9%): 16 sagittal, 2 bilateral coronal, 4 unilateral coronal, and 1 lambdoid synostosis. Complete neosuture formation was seen in 14 of those 23 patients (9 of 16 sagittal, 1 of 2 bilateral coronal, 3 of 4 unilateral coronal, and 1 of 1 lambdoid). Mean pre- and postoperative CI in the complete sagittal neosuture group was 67.4% and 75.5%, respectively, and in the non-neosuture group was 69.8% and 74.4%, respectively. There was no statistically significant difference in the CI between the neosuture and fused suture groups preoperatively or 17 months postoperatively in patients with sagittal synostosis.
Neosuture development can occur after endoscope-assisted strip craniectomy and molding helmet therapy for patients with craniosynostosis. Although the authors did not detect a significant difference in calvarial shape postoperatively in the group with sagittal synostosis, the relevance of neosuture formation remains to be determined. Further studies are required to discover long-term outcomes comparing patients with and without neosuture formation.
Dennis C. Nguyen, Scott J. Farber, Gary B. Skolnick, Sybill D. Naidoo, Matthew D. Smyth, Alex A. Kane, Kamlesh B. Patel and Albert S. Woo
Endoscope-assisted repair of sagittal craniosynostosis was adopted at St. Louis Children’s Hospital in 2006. This study examines the first 100 cases and reviews the outcomes and evolution of patient care protocols at our institution.
The authors performed a retrospective chart review of the first 100 consecutive endoscopic repairs of sagittal craniosynostosis between 2006 and 2014. The data associated with length of hospital stay, blood loss, transfusion rates, operative times, cephalic indices (CIs), complications, and cranial remolding orthosis were reviewed. Measurements were taken from available preoperative and 1-year postoperative 3D reconstructed CT scans.
The patients’ mean age at surgery was 3.3 ± 1.1 months. Of the 100 patients, 30 were female and 70 were male. The following perioperative data were noted. The mean operative time (± SD) was 77.1 ± 22.2 minutes, the mean estimated blood loss was 34.0 ± 34.8 ml, and the mean length of stay was 1.1 ± 0.4 days; 9% of patients required transfusions; and the mean pre- and postoperative CI values were 69.1 ± 3.8 and 77.7 ± 4.2, respectively. Conversion to open technique was required in 1 case due to presence of a large emissary vein that was difficult to control endoscopically. The mean duration of helmet therapy was 8.0 ± 2.9 months. Parietal osteotomies were eventually excluded from the procedure.
The clinical outcomes and improvements in CI seen in our population are similar to those seen at other high-volume centers. Since the inception of endoscope-assisted repair at our institution, the patient care protocol has undergone several significant changes. We have been able to remove less cranium using our “narrow-vertex” suturectomy technique without affecting patient safety or outcome. Patient compliance with helmet therapy and collaborative care with the orthotists remain the most essential aspects of a successful outcome.
Rowland H. Han, Dennis C. Nguyen, Brent S. Bruck, Gary B. Skolnick, Chester K. Yarbrough, Sybill D. Naidoo, Kamlesh B. Patel, Alex A. Kane, Albert S. Woo and Matthew D. Smyth
The authors present a retrospective cohort study examining complications in patients undergoing surgery for craniosynostosis using both minimally invasive endoscopic and open approaches.
Over the past 10 years, 295 nonsyndromic patients (140 undergoing endoscopic procedures and 155 undergoing open procedures) and 33 syndromic patients (endoscopic procedures in 10 and open procedures in 23) met the authors’ criteria. Variables analyzed included age at surgery, presence of a preexisting CSF shunt, skin incision method, estimated blood loss, transfusions of packed red blood cells, use of intravenous steroids or tranexamic acid, intraoperative durotomies, procedure length, and length of hospital stay. Complications were classified as either surgically or medically related.
In the nonsyndromic endoscopic group, the authors experienced 3 (2.1%) surgical and 5 (3.6%) medical complications. In the nonsyndromic open group, there were 2 (1.3%) surgical and 7 (4.5%) medical complications. Intraoperative durotomies occurred in 5 (3.6%) endoscopic and 12 (7.8%) open cases, were repaired primarily, and did not result in reoperations for CSF leakage. Similar complication rates were seen in syndromic cases. There was no death or permanent morbidity. Additionally, endoscopic procedures were associated with significantly decreased estimated blood loss, transfusions, procedure length, and length of hospital stay compared with open procedures.
Rates of intraoperative durotomies and surgical and medical complications were comparable between endoscopic and open techniques. This is the largest direct comparison to date between endoscopic and open interventions for synostosis, and the results are in agreement with previous series that endoscopic surgery confers distinct advantages over open surgery in appropriate patient populations.