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Unilateral lambdoid craniosynostosis case presentation. Patient #5 presented at age 18 months with cosmetic concerns per her parents. A diagnosis of unilateral lambdoid craniosynostosis was confirmed by physical examination and CT imaging. Panel (A) demonstrates patient's preoperative CT head 3D reconstruction which displays a unilateral fused lambdoid suture with resultant deformation and flattening of the posterior fossa. (B) Postoperative X-ray shows distractor placement in the lower third of the suture. (C) Postoperative CT head shows distractor placement within the strip craniectomy with 23 mm of distraction. The osteotomy was carried to the asterion.
Source publication
Object:
Distraction osteogenesis (DO) may allow for maximal volumetric expansion in the posterior vault (PV) by overcoming viscoelastic forces of overlying soft tissues. Little evidence exists regarding surgical planning and anticipated 3D volumetric changes pre- and post-operatively. We aim to study the volumetric changes occurring in PV distract...
Context in source publication
Context 1
... this observation may be due to low power of the study or even surgeon bias towards the syndromic patient, it may also represent a distinct pathophysiologic difference between the 2 entities. Figure 2 through 3 demonstrate radiographic and clinical photos of preoperative and postoperative unilateral lambdoid synostosis treatment. Figure 4 demonstrates pre-and post-operative radiographic results in a bilateral lambdoid synostosis patient. ...
Citations
... The development of distraction osteogenesis and spring-assisted expansion added to the option of conventional vault remodeling procedures. Thatikunta et al., by using the 3D software to investigate the volumetric changes, revealed an average of 13% increase in posterior cranial fossa volume with a mean distraction of 2 cm [21]. Another study from Japan revealed a 20% increased intracranial volume after 3-cm distraction [22]. ...
The cloverleaf skull deformity remains among the most complicated craniofacial conditions to successfully manage. Many cases achieve largely unsatisfactory outcomes due to the requirement for frequent reoperation on the cranial vault and failure to deal with all the elements of the craniofaciostenosis in a timely fashion. Early cranial vault surgery without addressing the cranial base deformity and its attendant cerebrospinal fluid flow changes is invariably challenging and disappointing. A recent focus on the expansion of the posterior cranial vault as a primary procedure with the greater volume change allows a delay in fronto-orbital advancement and reduced need for repeat surgery. We herein describe three cases of complex multisuture craniosynostosis with cloverleaf skull deformity who underwent neonatal posterior cranial vault decompression along with foramen magnum decompression. Our report examines the safety and rationale for this pre-emptive surgical approach to simultaneously deal with the cranial vault and craniocervical junction abnormalities and thus change the early trajectory of these complex cases.
... Thomas et al. 2 have described the role of Posterior cranial vault distraction osteogenesis (PCVDO) in redirecting calvarial growth along PCVDO, pioneered by Nicholas White et al. 5 in 2009, provided a reasonable increase in intracranial volume, avoiding complicated osteotomies of FOA. Thatikunta et al. 6 have highlighted the ability of DO to overcome viscoelastic forces of soft tissue as a vital advantage of the technique. ...
... 5 Zhang et al. 1 from the Philadelphia children's hospital suggested that the distraction vector be tailored to head shape. The vectors dictate the cranial volume expansion achieved, as Meena Thatikunta 6 described, who reported greater expansion when the distractors are placed in the lower third of osteotomy. Intra-operative guidance for placement of distractors in the correct vector has been described by Salokorpi et al., 9 who have used unique distraction vector guides. ...
Purpose
Posterior cranial vault distraction osteogenesis (PCVDO) has become the treatment of choice in specific indications within 12 years since its introduction in 2009. It is used to treat or prevent Intracranial hypertension secondary to craniosynostosis. However, there is still a lack of evidence describing this procedure's safety concerning the osteotomy line being close to the torcula. This systematic review aims to generate evidence regarding the safety of this new technique.
Materials and method
A systematic search of Pubmed and Embase database were done without any time limit until the last search date of July 31, 2021. Articles focused on posterior cranial vault distraction osteogenesis were shortlisted using defined inclusion criteria. In addition, complications, volume gain, and distraction protocols in patients with craniosynostosis were assessed.
Results
The review included 11 articles from 11 different centres. Of the Total 241 patients, 51 were non-syndromic patients, and 190 were syndromic patients. There were 242 procedures done on 241 patients. The proportion of patients with complications was 30%. Wound infection was the most commonly reported complication. PCVDO resulted in 20–25% volume gain and successfully prevented increased intracranial pressure (ICP).
Conclusion
The systematic review has significantly identified a 30.606% incidence of complications in PCVDO. No mortality was linked directly to the Posterior cranial vault distraction osteogenesis procedure (PCVDO). The authors identified the evolution of many unique vectors and osteotomy designs customized for patients and practices for safe surgery. The effects of PCVDO on the anterior cranial fossa remain a topic of interest and require observational studies for better understanding.
... In this case example, the shunt location did not hinder the ideal placement and orientation of the distractors in the caudal third of the suturectomy near the asterion. Utilizing the most horizontal distraction vector possible in the axial plane, in the lower third of the osteotomy, has demonstrated greater volumetric gains and may have an effect on foramen magnum area and Chiari malformation [16]. ...
... This has also been our experience. Our operative time in our published DO cohort was 161± 61 minutes [16]. We have also found that treatment of lambdoid synostosis results in a greater expansion of the foramen magnum. ...
... We have also found that treatment of lambdoid synostosis results in a greater expansion of the foramen magnum. Our morphometric study of 11 lambdoid craniosynostosis patients, produced a significant increase in foramen magnum area with posterior distraction (22% in syndromic, and 26.9% in non-syndromic patients) [16]. The patient described in this report experienced an increase in transverse diameter of 9.9% and an even larger anteroposterior diameter increase of 16.9% resulting in an increased foramen magnum area of 7.85% (not shown). ...
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 ventriculoperitoneal shunt. 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.
Objective:
Posterior cranial distraction (PCD) is a surgical technique to address craniosynostosis, especially in syndromic patients. The technique has the ability to significantly expand the cranium, while requiring minimal dural dissection, compared to cranial remodeling. Our goals were to determine the patient characteristics and surgical outcomes of PCD. The two questions that we sought to answer were: 1) What is the average published complication rate and the most common complications of PCD? and 2) How much intracranial volume expansion can one expect with PCD?
Design:
A PubMed database search of articles on PCD was performed. Case reports and articles with overlapping patients were excluded. A systematic review was performed using the remaining articles.
Main outcome measures:
Patient data were extracted in order to determine the total number of patients, patients with a syndrome, types of syndromes, mean age at surgery, mean distraction distance, mean increase in intracranial volume, and complications.
Results:
18 articles representing 325 patients were analyzed. A syndrome was present in 68.6% of patients. The mean age at time of surgery was 22.1 months. Mean distraction amount was 24.7 mm. Mean increase in intracranial volume was 253.2 cm3. The overall complication rate was 32.2%, with the most common complications being surgical-site infection, hardware-related complications and delayed wound healing.
Conclusions:
PCD is a powerful technique in the management of syndromic craniosynostosis, although complication rates are significantly higher than traditional remodeling techniques. Future studies should compare the effects of supratorcular and infratorcular osteotomies on intracranial volume, cosmesis and complications.
There are multiple treatment options for unilateral lambdoid craniosynostosis (ULS) including open posterior cranial vault remodeling (OCVR) and distraction osteogenesis (DO). There is a paucity of data comparing these techniques in the treatment of ULS. This study compared the perioperative characteristics of these interventions for patients with ULS. An IRB-approved chart review was performed from January 1999 to November 2018 at a single institution. Inclusion criteria included the diagnosis of ULS, treatment with either OCVR or DO using a posterior rotational flap technique, and a minimum 1-year follow-up. Seventeen patients met the inclusion criteria (12 OCVR and 5 DO). Patients in each cohort were found to have a similar distribution in sex, age at the time of surgery, synostosis laterality, weight, and length of follow-up. There was no significant difference in mean estimated blood loss/kg, surgical time, or transfusion requirements between cohorts. Distraction osteogenesis patients had a longer mean hospital length of stay (3.4 +/- 0.6 d versus 2.0 +/- 0.6 d, P = 0.0004). All patients were admitted to the surgical ward postoperatively. In the OCVR cohort, complications included 1 dural tear, 1 surgical site infection, and 2 reoperations. In the DO cohort, 1 patient had a distraction site infection, treated with antibiotics. There was no significant difference in estimated blood loss, volume of blood transfusion, or surgical time between OCVR and DO. Patients who underwent OCVR had a higher incidence of postoperative complications and the need for reoperation. This data provides insight into the perioperative differences between OCVR and DO in patients with ULS.
Study Design
Case report
Objective
Syndromic multi-suture craniosynostosis can result in complex dysmorphology and increased intracranial pressure. We present a case report of a child with craniometaphyseal dysplasia and syndromic multi-suture craniosynostosis who presented with increased intracranial pressure, ventriculomegaly and Chiari deformity Type 1.
Methods
The child underwent a posterior vault distraction to increase the intracranial volume.
Results
The posterior cranial expansion was successful in correcting the craniocerebral disproportion caused by multi-suture synostosis, and resolved the high intracranial pressure and papilloedema. There were no post-operative complications.
Conclusions
Posterior cranial vault distraction was an effective method of addressing increased intracranial pressure by correcting craniocerebral disproportion by increasing intracranial volume and also addressing the Chiari Type I deformity that resulted from syndromic multi-suture craniosynostosis in a child with craniometaphyseal dysplasia.
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.
Summary:
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.
Purpose of review:
Craniosynostosis, a condition of premature cranial suture fusion, can have significantly detrimental effects on development and growth due to sequelae of increased intracranial hypertension (ICP), exophthalmos, and upper airway obstruction. Evolving surgical treatments now include distraction osteogenesis (DO) due to its many benefits relative to standard cranial vault remodeling procedures. This article provides an overview and update of different surgical applications of DO for patients with craniosynostosis.
Recent findings:
DO has been utilized successfully for single and multisuture craniosynostosis with or without midface hypoplasia to increase intracranial volume, decrease ICP and improve aesthetics. It has been applied in single suture synostosis, posterior vault DO, fronto-orbital advancement, monobloc DO and Le Fort III DO. DO has been applied through modification of traditional surgical procedures with success in maintaining goals of surgery while reducing risk.
Summary:
DO is still a relatively new and evolving surgical technique for patients with syndromic and nonsyndromic craniosynostosis. With promising benefits, consideration for each procedure should be weighed until longer-term data is available.
