Endoscopic Treatment of Combined Metopic-Sagittal Craniosynostosis
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CLINICAL ARTICLE J Neurosurg Pediatr 26:113–121, 2020 Endoscopic treatment of combined metopic-sagittal craniosynostosis Ema Zubovic, MD,1 Gary B. Skolnick, BS,1 Sybill D. Naidoo, PhD, RN, CPNP,1 Mark Bellanger, CPed, COA, ATC,3 Matthew D. Smyth, MD,2 and Kamlesh B. Patel, MD, MSc1 1Division of Plastic & Reconstructive Surgery, Department of Surgery, and 2Department of Neurosurgery, Washington University School of Medicine in St. Louis; and 3Orthotic and Prosthetic Lab, St. Louis, Missouri OBJECTIVE Combined metopic-sagittal craniosynostosis is traditionally treated with open cranial vault remodeling and fronto-orbital advancement, sometimes in multiple operations. Endoscopic treatment of this multisuture synostosis pres- ents a complex challenge for the surgeon and orthotist. METHODS The authors retrospectively analyzed the preoperative and 1-year postoperative CT scans of 3 patients with combined metopic-sagittal synostosis, all of whom were treated with simultaneous endoscope-assisted craniectomy of the metopic and sagittal sutures followed by helmet therapy. Established anthropometric measurements were applied to assess pre- and postoperative morphology, including cranial index and interfrontal divergence angle (IFDA). Patients’ measurements were compared to those obtained in 18 normal controls. RESULTS Two boys and one girl underwent endoscope-assisted craniectomy at a mean age of 81 days. The mean preoperative cranial index was 0.70 (vs control mean of 0.82, p = 0.009), corrected postoperatively to a mean of 0.82 (vs control mean of 0.80, p = 0.606). The mean preoperative IFDA was 110.4° (vs control mean of 152.6°, p = 0.017), cor- rected postoperatively to a mean of 139.1° (vs control mean of 140.3°, p = 0.348). The mean blood loss was 100 mL and the mean length of stay was 1.7 days. No patient underwent reoperation. The mean clinical follow-up was 3.4 years. CONCLUSIONS Endoscope-assisted craniectomy with helmet therapy is a viable single-stage treatment option for combined metopic-sagittal synostosis, providing correction of the stigmata of trigonoscaphocephaly, with normalization of the cranial index and IFDA. https://thejns.org/doi/abs/10.3171/2020.2.PEDS2029 KEYWORDS metopic-sagittal synostosis; endoscopic; craniosynostosis; multisuture; craniofacial OMBINED synostosis of the metopic and sagittal su- The risk of potential surgical and anesthetic morbidity tures is exceedingly rare, representing just 0.5%– is increased with multiple open transcranial operations in 2% of all craniosynostosis cases.1,2 Trigonoscapho- the 1st year of life. For single-suture craniosynostosis, en- Ccephaly, the result of simultaneous frontal, temporal, and doscopic procedures have been shown to decrease blood biparietal growth restriction, presents a more complex loss, operating time, length of hospital stay, and cost rela- reconstructive challenge than either metopic or sagittal tive to open approaches.4–8 In patients with isolated metopic synostosis alone. Traditional practice has been a 2-stage or sagittal synostosis, equivalent morphological outcomes open surgical repair to address each synostosis separately. have been achieved with endoscope-assisted craniectomy Czerwinski et al. suggested an initial procedure to repair followed by postoperative molding helmet therapy.9–14 To the sagittal synostosis when patients are 6–8 months of our knowledge, there has been no report in the literature of age, followed by correction of the metopic synostosis by single-stage endoscope-assisted craniectomy with molding fronto-orbital advancement at 10–12 months of age, allow- helmet therapy for combined metopic-sagittal synostosis. ing for a more stable fronto-orbital bandeau reconstruc- In this report, our aim was as follows: to 1) present our tion.3 More recently, Dobbs et al. published the first report experience in endoscopic treatment of combined metopic- of single-stage open repair for combined metopic-sagittal sagittal synostosis, demonstrating our operative technique synostosis.1 and postoperative helmet therapy protocol; 2) objectively ABBREVIATIONS IFDA = interfrontal divergence angle; ZF-G-ZF angle = glabella and bilateral zygomaticofrontal sutures; ZF-ZF = interzygomaticofrontal. SUBMITTED January 10, 2020. ACCEPTED February 18, 2020. INCLUDE WHEN CITING Published online April 17, 2020; DOI: 10.3171/2020.2.PEDS2029. ©AANS 2020, except where prohibited by US copyright law J Neurosurg Pediatr Volume 26 • August 2020 113 Unauthenticated | Downloaded 10/06/21 05:19 AM UTC Zubovic et al. evaluate the postoperative morphological outcomes of is taken to remove the bone in segments after controlling patients treated endoscopically; and 3) compare these pa- any emissary veins with bipolar electrocautery. The an- tients to normal controls as an evaluation of success in terior remainder of the fused metopic suture is resected, normalization of cranial and facial morphology. tapering to the level of the nasofrontal suture. The ma- jority of the 1-cm strip craniectomy is done under direct Methods visualization. Completion of suturectomy is confirmed by endoscopic visualization of the nasal upper lateral carti- After obtaining institutional review board approval, we lages (Fig. 1), the tips of which are seen at the nasofrontal identified 3 patients treated endoscopically for combined junction under the nasal bones. The nasal bones are not metopic-sagittal craniosynostosis at our institution be- resected. Floseal (Baxter International) is placed in the tween 2012 and 2017. All 3 underwent endoscope-assisted craniectomy defect. The skin incision is closed in layered craniectomy performed by the same craniofacial surgeon fashion with resorbable suture and skin glue. (K.B.P.) and neurosurgeon (M.D.S.), followed by custom molding helmet therapy until 1 year of age. Sagittal Suturectomy Preoperative Evaluation After completion of the metopic suturectomy and skin closure, the patient is repositioned in the sphinx position After preoperative evaluation by both treating surgeons, and sterile preparation and draping is repeated. Hair is patients underwent preoperative low-dose CT imaging to clipped, and 2 new 2.5-cm transverse incisions are made confirm the clinical diagnosis of metopic-sagittal cranio- just posterior to the anterior fontanelle and just anterior synostosis. Our practice is to offer both open and endo- to lambda. Subgaleal dissection is again extended across scopic treatment options to eligible patients younger than the fused sagittal suture, connecting the 2 access incisions. 5 months of age, and only open treatment to older patients. Burr holes are made to the side of the fused sagittal suture We prefer patients to be 2–3 months of age for endoscopic through each access incision, then widened with curettes treatment to allow the orthotist to take advantage of the 14,15 and Kerrison punches, and osteotomies are extended rapid skull growth of early infancy. Endoscopic treat- across midline. After mobilization of the dura, the wedge ment of multisuture synostosis places more complex de- of cranial bone between the anterior osteotomy and the mands on postoperative molding helmet therapy, which are posterior aspect of the anterior fontanelle is excised under best met by early initiation of helmet therapy. Patients meet direct visualization with Tessier bone scissors. An endo- the orthotist and undergo scanning for a custom molding scope is introduced into the epidural space posteriorly, and helmet in the week before surgery, in order to ensure hel- the dura is dissected from the inner table. A 2-cm-wide met availability in the immediate postoperative period. segment of the fused sagittal suture is excised with para- median cuts by using Tessier bone scissors and extracted Operative Technique through the anterior incision. Finally, the remaining bone We initially performed this procedure with the patient anterior to lambda is freed and excised. Bone edges are entirely in the prone sphinx position, but our preferred again coagulated with suction electrocautery, under visu- technique is now to complete the metopic suturectomy alization by 0° endoscope. Floseal is again placed in the with the patient supine, repositioning to the sphinx po- craniectomy defect and access incisions are closed in the sition for the sagittal suturectomy. This allows for more same fashion as above. complete visualization and more facile instrumentation Patients without significant comorbidities are typically for each approach. We have previously published exam- admitted overnight to a regular surgical ward and hemato- ples of patient positioning for endoscopic repairs of me- crit is measured 4 hours postoperatively. Discharge home topic14 and sagittal13 synostosis. on postoperative day 1 is standard. Patients using a local orthotist are typically fitted with their helmet prior to hos- Metopic Suturectomy pital discharge; those using a more distant orthotic com- The patient is positioned supine, with the head elevat- pany have a helmet fitting appointment within the first 3 ed in a Mayfield headrest above the level of the heart to postoperative days. Edema control with head elevation and minimize blood loss. Intravenous dexamethasone and 24 hours of intravenous dexamethasone (beginning with tranexamic acid are administered. After sterile prepara- the intraoperative dose) are essential to allow early helmet tion, draping, and infiltration of local anesthesia, a 2.5-cm fitting. transverse incision is made in the frontal hairline, and sub- galeal dissection is extended