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OBJECTIVEUnilateral lambdoid synostosis is the premature fusion of a lambdoid suture or sutures and represents the least common form of craniosynostosis, occurring in 1 in 40,000 births. Cranial vault remodeling (CVR) and endoscopic suturectomy with helmet therapy (ES) are surgical approaches that are used to allow for normal brain growth and improved craniofacial symmetry. The authors conducted a comparative outcomes analysis of patients with lambdoid synostosis undergoing either CVR or ES.METHODSThe authors conducted a retrospective consecutive cohort study of patients with nonsyndromic lambdoid synostosis who underwent surgical correction identified from a single-institution database of patients with craniosynostosis seen between 2000 and 2018. Cranial growth was measured in head circumference percentile and z score.RESULTSNineteen patients (8 female and 11 male) with isolated unilateral lambdoid synostosis were identified (8 right and 11 left). Six underwent CVR and 13 underwent ES. No statistically significant differences were noted between surgical groups with respect to suture laterality, the patient’s sex, and length of follow-up. Patients treated with ES presented and underwent surgery at a younger age than those treated with CVR (p = 0.0002 and p = 0.0001, respectively). Operating and anesthesia time, estimated blood loss, and ICU and total hospital days were significantly lower in ES (all p < 0.05). No significant differences were observed in pre- and postoperative head circumference percentiles or z scores between groups up to 36 months postoperatively. No patients required reoperation as of last follow-up.CONCLUSIONSEndoscopic management of lambdoid synostosis is safe, efficient, and efficacious in terms of intraoperative and long-term cranial growth outcomes when compared to CVR. The authors recommend this minimally invasive approach as an option for correction of lambdoid synostosis in patients presenting early in their course.

Moyamoya is a progressive steno-occlusive cerebrovascular pathology of unknown etiology that usually involves the terminal portions of the internal carotid arteries and/or the proximal portions of the anterior and middle cerebral arteries bilaterally. The preoperative Suzuki staging system and postoperative Matsushima grade are nearly universally used markers of natural history and surgical revascularization results, respectively, but their correlation with clinical and radiographic manifestations of moyamoya has not been systematically evaluated in a large cohort. This study evaluated the strength of correlations between pre- and post-operative angiographic parameters and clinical status among pediatric patients with moyamoya. The participants included 58 patients of mean age 11 years at the time of surgery who underwent bilateral indirect revascularization in the same procedure at Boston Children’s Hospital, between January 2010 and December 2015. All included patients had available preoperative and 1-year postoperative digital subtraction angiography. Clinical data included presenting symptoms, degree of functional incapacity, and perioperative and long-term complications. Radiographic data included preoperative Suzuki stage, degree of arterial stenosis, a novel collateral score, the presence of hypovascular territories on digital subtraction angiography, and postoperative Matsushima grade and evolution of stenosis. Chi-squared test and Pearson coefficient were used for correlation studies for categorical variables and Spearman’s rho was used for correlation studies for continuous variables. Results showed that Suzuki stage, collateral score, and degree of stenosis were insufficient to predict clinical presentation, preoperative incapacity, and radiographic presentation, whereas the presence of hypovascular territories was correlated with all of these. At 1-year follow-up, Matsushima grade was insufficient for predicting perioperative or long-term complications, nor did it correlate with postoperative incapacity. The presence of hypovascular territories at 1-year follow-up was correlated with the incidence of postoperative ischemic symptoms.