This study combined qualitative and quantitative approaches to evaluate outcomes of isolated nonsyndromic unilateral lambdoid synostosis at the Children\'s Hospital of Philadelphia. Volumetric, linear, and angular analyses were performed on long-term postoperative and normal control scans. Preoperative and postoperative clinical photographs were evaluated for the presence of dysmorphic frontofacial features. Among 26 included patients, median age of surgery was 10 months and mean postoperative follow-up was 5.9 ± 5.7 years. Two (7.7%) patients underwent secondary cranial vault procedures. At most recent follow-up, 2 (7.7%) subjects reported intermittent headaches. Twenty (90.9%) of 22 patients were assigned Whitaker grade I. Among 9 subjects with long-term imaging at age 11.5 ± 5.3, posterior vault asymmetry, posterior fossa deflection angle, cranial base angle, and ear position asymmetry all remained greater than in the control group. Of subjects included in the frontofacial feature analysis (n = 10), 50% had resolution of all dysmorphic frontofacial features present preoperatively. Overall, most subjects who underwent PVR did not require revisional surgery and had good outcomes both aesthetically and functionally. Despite residual abnormalities in the cranial base and posterior vault, most had resolution of parietal bossing and facial scoliosis. Ear position asymmetry was the most common dysmorphic feature at long-term follow-up.

The resurgence of strip craniectomies began in the mid-1990s with advances in surgical technique and anesthesia coupled with the critical observation that earlier interventions benefitted from an easily molded skull. Jimenez and Barone\'s pioneering introduction of endoscopic approaches to strip craniectomies coupled with postoperative helmeting in newborns and young infants and Claes Lauritzen\'s introduction of spring-mediated cranioplasty began the era of minimally invasive approaches in the surgical correction of craniosynostosis. This article provides technical descriptions of these treatment modalities, a comparative literature review, and our institutional algorithms for the correction of sagittal craniosynostosis and unilambdoid craniosynostosis.

BACKGROUND: Craniosynostosis is a condition characterized by the premature fusion of 2 or more skull bones. Craniosynostosis of the lambdoid suture is one of the rarest forms, accounting for 1-4% of all craniosynostoses. Documented cases are separated into simple (single suture), complex (bilateral), and associated with adjacent synostoses (\"Mercedes Benz\" Pattern) or syndromes (i.e., Crouzon, Sathre-Chotzen, Antley-Bixler). This condition can manifest phenotypic deformities and neurological sequelae that can lead to impaired cognitive function if improperly treated or left undiagnosed. Preferred surgical techniques have varied over time but all maintain the common goals of establishing proper head shape and preventing of complications that could contribute to aforementioned sequelae.
CONCLUSIONS: This comprehensive review highlights demographic distributions, embryological development, pathogenesis, clinical presentation, neurological sequelae, radiologic findings, surgical techniques, surgical outcomes, and postoperative considerations of patients with lambdoid craniosynostosis presentation. In addition, a systematic review was conducted to explore the operative management of lambdoid craniosynostosis using PubMed, Embase, and Scopus databases, with 38 articles included after screening. Key Messages: Due to a low volume of published cases, diagnosis and treatment can vary. Large overlap in presentation can occur in patients that display lambdoid craniosynostosis and posterior plagiocephaly, furthering the need for comprehensive analysis. Possessing the knowledge and tools to properly assess patients with lambdoid craniosynostosis will allow for more precise care and improved outcomes.

Posterior vault distraction osteogenesis (DO) is an emerging treatment option for craniosynostosis. Operative nuances detailing surgical management are being described with increasing use and experience. In this article, we discuss the surgical management of an 8-month-old male with a ventriculoperitoneal shunt (VPS) diagnosed with bilateral lambdoid craniosynostosis and Chiari I malformation. The patient underwent successful bilateral posterior fossa DO without surgical re-implantation of the shunt. Pre- and post-operative imaging confirmed no migration of the VPS. Intracranial volume increased by 20.1% and posterior fossa volume increased by 39.9%. Our experience illustrates that posterior vault DO can be done safely in the setting of a parieto-occipital VPS, in a single operative setting, without the need of additional procedures.

The cause of occipital asymmtery can be either extrinsic or intrinsic. Intrinsic causes include lambdoid craniosynsotosis. This condition is generally treated with cranial vault expansion surgery. Extrinsic causes include deformational plagiocephaly, which became commonplace after the \"Back to Sleep Campaign\" instituted in the 1980s by the American Academy of Pediatrics. The treatment of this condition is non surgical. Dr. Joseph Gruss was instumental in differentiating between these conditions and reducing the number of unnecessary surgeries that were previously being performed.

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Craniosynostosis affecting the lambdoid suture is uncommon. The definition of lambdoid craniosynostosis solely applies to those cases demonstrating true suture obliteration, similar to other forms of craniosynostosis. In patients presenting with posterior plagiocephaly, true lambdoid craniosynostosis must be differentiated from the much more common positional molding. It can occur in a unilateral form, a bilateral form, or as part of a complex craniosynostosis. In children with craniofacial syndromes, synostosis of the lambdoid suture most often is seen within the context of a pansynostotic picture. Chiari malformations are commonly seen in multisutural and syndromic types of craniosynostosis that affect the lambdoid sutures. Posterior cranial vault remodeling is recommended to provide adequate intracranial volume to allow for brain growth and to normalize the skull shape. Although many techniques have been described for the correction of lambdoid synostosis, optimal outcomes may result from those techniques based on the concept of occipital advancement.