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Open access

Robert M. Lober, Shobhan Vachhrajani, Salim Mancho, and Kambiz Kamian

The authors describe the use of the Gigli saw for craniectomy in minimal access surgery to address sagittal craniosynostosis. This modification allows for supine positioning and avoidance of potential brain compression with endoscopic instruments, and provides visually clear, safe, and facile removal of the fused suture and surrounding calvaria.

The video can be found here: https://vimeo.com/511568750.

Free access

Christopher M. Bonfield, Chevis N. Shannon, Ron W. Reeder, Samuel Browd, James Drake, Jason S. Hauptman, Abhaya V. Kulkarni, David D. Limbrick Jr., Patrick J. McDonald, Robert Naftel, Ian F. Pollack, Jay Riva-Cambrin, Curtis Rozzelle, Mandeep S. Tamber, William E. Whitehead, John R. W. Kestle, John C. Wellons III, and for the Hydrocephalus Clinical Research Network (HCRN)

OBJECTIVE

Hydrocephalus may be seen in patients with multisuture craniosynostosis and, less commonly, single-suture craniosynostosis. The optimal treatment for hydrocephalus in this population is unknown. In this study, the authors aimed to evaluate the success rate of ventriculoperitoneal shunt (VPS) treatment and endoscopic third ventriculostomy (ETV) both with and without choroid plexus cauterization (CPC) in patients with craniosynostosis.

METHODS

Utilizing the Hydrocephalus Clinical Research Network (HCRN) Core Data Project (Registry), the authors identified all patients who underwent treatment for hydrocephalus associated with craniosynostosis. Descriptive statistics, demographics, and surgical outcomes were evaluated.

RESULTS

In total, 42 patients underwent treatment for hydrocephalus associated with craniosynostosis. The median gestational age at birth was 39.0 weeks (IQR 38.0, 40.0); 55% were female and 60% were White. The median age at first craniosynostosis surgery was 0.6 years (IQR 0.3, 1.7), and at the first permanent hydrocephalus surgery it was 1.2 years (IQR 0.5, 2.5). Thirty-three patients (79%) had multiple different sutures fused, and 9 had a single suture: 3 unicoronal (7%), 3 sagittal (7%), 2 lambdoidal (5%), and 1 unknown (2%). Syndromes were identified in 38 patients (90%), with Crouzon syndrome being the most common (n = 16, 42%). Ten patients (28%) received permanent hydrocephalus surgery before the first craniosynostosis surgery. Twenty-eight patients (67%) underwent VPS treatment, with the remaining 14 (33%) undergoing ETV with or without CPC (ETV ± CPC). Within 12 months after initial hydrocephalus intervention, 14 patients (34%) required revision (8 VPS and 6 ETV ± CPC). At the most recent follow-up, 21 patients (50%) required a revision. The revision rate decreased as age increased. The overall infection rate was 5% (VPS 7%, 0% ETV ± CPC).

CONCLUSIONS

This is the largest prospective study reported on children with craniosynostosis and hydrocephalus. Hydrocephalus in children with craniosynostosis most commonly occurs in syndromic patients and multisuture fusion. It is treated at varying ages; however, most patients undergo surgery for craniosynostosis prior to hydrocephalus treatment. While VPS treatment is performed more frequently, VPS and ETV are both reasonable options, with decreasing revision rates with increasing age, for the treatment of hydrocephalus associated with craniosynostosis.

Free access

Caitlin Hoffman, Alyssa B. Valenti, Eseosa Odigie, Kwanza Warren, Ishani D. Premaratne, and Thomas A. Imahiyerobo

Craniosynostosis is the premature fusion of the skull. There are two forms of treatment: open surgery and minimally invasive endoscope-assisted suturectomy. Candidates for endoscopic treatment are less than 6 months of age. The techniques are equally effective; however, endoscopic surgery is associated with less blood loss, minimal tissue disruption, shorter operative time, and shorter hospitalization. In this study, the authors aimed to evaluate the impact of race/ethnicity and insurance status on age of presentation/surgery in children with craniosynostosis to highlight potential disparities in healthcare access. Charts were reviewed for children with craniosynostosis at two tertiary care hospitals in New York City from January 1, 2014, to August 31, 2020. Clinical and demographic data were collected, including variables pertaining to family socioeconomic status, home address/zip code, insurance status (no insurance, Medicaid, or private), race/ethnicity, age and date of presentation for initial consultation, type of surgery performed, and details of hospitalization. Children with unknown race/ethnicity and those with syndromic craniosynostosis were excluded. The data were analyzed via t-tests and chi-square tests for statistical significance (p < 0.05). A total of 121 children were identified; 62 surgeries were performed open and 59 endoscopically. The mean age at initial presentation of the cohort was 6.68 months, and on the day of surgery it was 8.45 months. Age at presentation for the open surgery cohort compared with the endoscopic cohort achieved statistical significance at 11.33 months (SD 12.41) for the open cohort and 1.86 months (SD 1.1473) for the endoscopic cohort (p < 0.0001). Age on the day of surgery for the open cohort versus the endoscopic cohort demonstrated statistical significance at 14.19 months (SD 15.05) and 2.58 months (SD 1.030), respectively. A statistically significant difference between the two groups was noted with regard to insurance status (p = 0.0044); the open surgical group comprised more patients without insurance and with Medicaid compared with the endoscopic group. The racial composition of the two groups reached statistical significance when comparing proportions of White, Black, Hispanic, Asian, and other (p = 0.000815), with significantly more Black and Hispanic patients treated in the open surgical group. The results demonstrate a relationship between race and lack of insurance or Medicaid status, and type of surgery received; Black and Hispanic children and children with Medicaid were more likely to present later and undergo open surgery.

Free access

Arvid Frostell, Maryam Haghighi, Jiri Bartek Jr., Ulrika Sandvik, Bengt Gustavsson, Adrian Elmi-Terander, and Erik Edström

OBJECTIVE

Isolated nonsyndromic sagittal synostosis (SS) is the most common form of craniosynostosis in children, accounting for approximately 60% of all craniosynostoses. The typical cranial measurement used to define and follow SS is the cephalic index (CI). Several surgical techniques have been suggested, but agreement on type and timing of surgery is lacking. This study aimed to evaluate the authors’ institutional experience of surgically treating SS using a modified subtotal cranial vault remodeling technique in a population-based cohort. Special attention was directed toward the effect of patient age at time of surgery on long-term CI outcome.

METHODS

A retrospective analysis was conducted on all patients with isolated nonsyndromic SS who were surgically treated from 2003 to 2011. Data from electronic medical records were gathered. Eighty-two patients with SS were identified, 77 fulfilled inclusion criteria, and 72 had sufficient follow-up data and were included. CI during follow-up after surgery was investigated with ANOVA and a linear mixed model.

RESULTS

In total, 72 patients were analyzed, consisting of 16 females (22%) and 56 males (78%). The mean ± SD age at surgery was 4.1 ± 3.1 months. Blood transfusions were received by 81% of patients (26% intraoperatively, 64% postoperatively, 9% both). The mean ± SD time in the pediatric ICU was 1.1 ± 0.25 days, and the mean ± SD total hospital length of stay was 4.6 ± 2.0 days. No patient required reoperation. The mean ± SD CI increased from 69 ± 3 to 87 ± 5 for patients who underwent surgery before 45 days of age. Surgery resulted in a larger increase in CI for patients who underwent surgery at a younger age compared with older patients (p < 0.05, Tukey’s HSD test). In the comparison of patients who underwent surgery before 45 days of age with patients who underwent surgery at 45–90, 90–180, and more than 180 days of age, the linear mixed model estimated a long-term loss of CI of 3.0, 5.5, and 7.4 points, respectively.

CONCLUSIONS

The modified subtotal cranial vault remodeling technique used in this study significantly improved CI in patients with SS. The best results were achieved when surgery was performed early in life.

Free access

Concezio Di Rocco, John R. W. Kestle, Richard Hayward, and Jesse A. Taylor

Open access

Craig Birgfeld, Federico Di Rocco, Cormac O. Maher, Mark R. Proctor, and Matthew D. Smyth

Open access

David F. Jimenez

Lambdoid craniosynostosis leads to significant deformational changes of the calvaria and cranial fossae. Surgery used to treat the condition typically consists of a calvarial vault remodeling (CVR) procedure whereby the entire occiput is removed and reshaped along with a bandeau advancement to give the patient a rounded occiput. As an option, this video presents the minimally invasive endoscopic craniectomy used at the author's institution, which was developed there and has been successfully used for 25 years. This procedure is simple and can be done rapidly, with minimal to no blood loss. The video details the key steps necessary to successfully perform the procedure.

The video can be found here: https://vimeo.com/515746378.

Free access

Cordell M. Baker, Vijay M. Ravindra, Barbu Gociman, Faizi A. Siddiqi, Jesse A. Goldstein, Matthew D. Smyth, Amy Lee, Richard C. E. Anderson, Kamlesh B. Patel, Craig Birgfeld, Ian F. Pollack, Thomas Imahiyerobo, John R. W. Kestle, and for the Synostosis Research Group

OBJECTIVE

Sagittal synostosis is the most common form of isolated craniosynostosis. Although some centers have reported extensive experience with this condition, most reports have focused on a single center. In 2017, the Synostosis Research Group (SynRG), a multicenter collaborative network, was formed to study craniosynostosis. Here, the authors report their early experience with treating sagittal synostosis in the network. The goals were to describe practice patterns, identify variations, and generate hypotheses for future research.

METHODS

All patients with a clinical diagnosis of isolated sagittal synostosis who presented to a SynRG center between March 1, 2017, and October 31, 2019, were included. Follow-up information through October 31, 2020, was included. Data extracted from the prospectively maintained SynRG registry included baseline parameters, surgical adjuncts and techniques, complications prior to discharge, and indications for reoperation. Data analysis was descriptive, using frequencies for categorical variables and means and medians for continuous variables.

RESULTS

Two hundred five patients had treatment for sagittal synostosis at 5 different sites. One hundred twenty-six patients were treated with strip craniectomy and 79 patients with total cranial vault remodeling. The most common strip craniectomy was wide craniectomy with parietal wedge osteotomies (44%), and the most common cranial vault remodeling procedure was total vault remodeling without forehead remodeling (63%). Preoperative mean cephalic indices (CIs) were similar between treatment groups: 0.69 for strip craniectomy and 0.68 for cranial vault remodeling. Thirteen percent of patients had other health problems. In the cranial vault cohort, 81% of patients who received tranexamic acid required a transfusion compared with 94% of patients who did not receive tranexamic acid. The rates of complication were low in all treatment groups. Five patients (2%) had an unintended reoperation. The mean change in CI was 0.09 for strip craniectomy and 0.06 for cranial vault remodeling; wide craniectomy resulted in a greater change in CI in the strip craniectomy group.

CONCLUSIONS

The baseline severity of scaphocephaly was similar across procedures and sites. Treatment methods varied, but cranial vault remodeling and strip craniectomy both resulted in satisfactory postoperative CIs. Use of tranexamic acid may reduce the need for transfusion in cranial vault cases. The wide craniectomy technique for strip craniectomy seemed to be associated with change in CI. Both findings seem amenable to testing in a randomized controlled trial.

Open access

Maria Licci, Pierre-Aurelien Beuriat, Alexandru Szathmari, Christian Paulus, Arnaud Gleizal, Carmine Mottolese, and Federico Di Rocco

Premature fusion of the metopic suture results in trigonocephaly with variable degrees of anterior cranial fossa dysmorphia and craniofacial deformity. Different surgical corrective techniques that aim to reshape the forehead and enlarge the cranial volume have been described. Typical variations of the standard fronto-orbitary advancement carry the risk of relevant blood loss during frontal osteotomy, where paired emissary metopic veins are disrupted. The authors present a technical variant that preserves a bony triangle over the glabella to optimize control of these veins, which represent the major source of bleeding, and applies Piezosurgery to perform the osteotomies to minimize bone substance loss.

The video can be found here: https://vimeo.com/511536423.