Posterior Vault Research Articles

Three-Dimensional Animated Videos Improve Caregiver Craniosynostosis Education.

Background: Craniosynostosis management involves various surgical options early in a patient's life. Nevertheless, few three-dimensional (3D) tools exist to help caregivers comprehend craniosynostosis anatomy and surgical options. This study aims to assess the efficacy of 3D animated videos for enhancing craniosynostosis education in caregivers. Methods: We created 3D animated videos describing anatomy and surgical options (eg, fronto-orbital advancement, posterior vault reconstruction) for three craniosynostosis diagnoses: bicoronal, metopic, and sagittal. In a cross-sectional survey, caregivers rated their understanding of craniosynostosis on 10-point Likert-scales, labelled anatomic sutures, and answered true/false general (eg, "The sutures have fused too early") and diagnosis-specific (eg, "The distractors are not removed after surgery") craniosynostosis knowledge questions. Respondents were then shown an animated video and asked the same set of questions after watching the video. Results: A total of 69 craniosynostosis caregivers (mean age 35 years, 73% Caucasian, 64% female) completed the survey. After watching the video, caregivers self-rated their understanding of craniosynostosis as significantly higher (mean score difference: 2.62, P < .01). Caregivers also scored significantly higher on the general and diagnosis-specific knowledge questions (mean score difference: 1.27, P < .01). Conclusions: Our findings indicate that our animated videos improved caregiver craniosynostosis understanding and knowledge. These findings may inform how surgeons approach future caregiver craniosynostosis education.

Spring-assisted posterior vault expansion in children over 2 years of age with craniosynostosis.

This study evaluated spring-assisted posterior vault expansion (SA-PVE) in children aged >2 years with craniosynostosis and signs of high intracranial pressure (ICP). We retrospectively analysed all consecutive patients aged > 2 years and operated with SA-PVEbetween 2018 and 2020 at the Craniofacial Center at Sahlgrenska University Hospital, Sweden. During the procedure, a circumferent occipital bone flap extending below the torcula was created and remained attached to the dura. Intracranial volumes (ICVs) were calculated from computed tomography (CT) images, and demographic data and information regarding symptoms and signs of high ICP were collected. The study included eight patients [Crouzon/Pfeiffer (n = 4), multiple craniosynostosis (n = 3), and secondary synostosis (n = 1)]. Median age at SA-PVE was 3.8 years (range: 2.5-12.8 years), and springs were removed after a median of 5.5 months (range: 2.3-8.3 months). The median operating timewas164min (range: 102-221 min), and estimated blood loss was 4.5 mL/kg body weight (range: 1.4-59.1 mL/kg body weight), with 50% of patients receiving a blood transfusion. The median increase in ICV was 206 cm3 (range: 122-344 cm3) representing an 18.7% increase (range: 7.9-24.1%; p = 0.01). Weobserved no major perioperative complications, and symptoms related to high ICP were improved or absent at clinical follow-up. These results demonstrated that SA-PVE involving creation of a large occipital bone flapincluding the torcula as a safe and effective surgical treatment in children aged >2 years with craniosynostosis and elevated ICP.

Distraction osteogenesis in craniosynosotosis: technical note

Introduction: Craniosynostosis is a group of diseases whose main characteristic is the premature fusion of one or more cranial sutures. They can be subdivided into non-syndromic and syndromic. The posterior vault distraction osteogenesis (PVDO) has become the standard procedure for managing the intracranial hypertension of these patients. The objective of this paper is to report the experience of the Sobrapar Hospital (Campinas, Brazil) in treating and following up on syndromic craniosynostosis patients subjected to PVDO and highlight the lessons learned throughout this process. Methods: The medical records of the patients treated at the Sobrapar Hospital between 2013 and 2023 were reviewed. Results: The total of patients initially candidates to PVDO was 79, and 75 of them proceeded to the surgery. The mean age of surgery was 8,16 months old (ranging from 4 months to 12 years old). The length of stay ranged from 2 to 14 days, with a mean of 3,29 days. The distraction achieved ranged from 13 mm to 30 mm, with a mean distance of 23 mm. The postoperative complications were mostly cutaneous. CSF leak occurred in 3 patients. Conclusion: PVDO provides a progressive formation of a vascularized bone, its intracranial volume expansion is greater than the FOA, increasing the posterior fossa’s size (even when the craniotomy is supratorcular) and it improves Chiari’s symptoms, even when the radiologic abnormalities are not necessarily solved. A specialized and multidisciplinary team is essential to improving the success rates of the surgery.

Changes in Ventricular Volume After Posterior Vault Distraction Osteogenesis in Patients With Syndromic and Nonsyndromic Craniosynostosis.

Little is known about the response of the ventricular system to cranial vault surgery in patients with craniosynostosis. This study aims to evaluate the changes in the cerebral ventricular system in response to posterior vault distraction osteogenesis (PVDO) in patients with syndromic and nonsyndromic craniosynostosis. A single-institution retrospective review of all patients with craniosynostosis undergoing PVDO from 2000 to 2022 was completed. Patients were included for analysis if they had pre and postoperative cranial computed tomography scans. Ventricular volume (VV) and intracranial volume (ICV) were calculated using segmentation software. Both patients with syndromic synostosis and nonsyndromic synostosis (NSS) experienced a significant increase in ICV after PVDO, but only patients with NSS experienced a significant VV change ( P = 0.004). After normalization by ICV, total, lateral, and third VV changes retained significance with percentage increases of 114%, 117%, and 89%, respectively ( P < 0.05 for all). The differing results between cohorts reinforce the concept that the intracranial milieu is different between patients with syndromic synostosis and NSS. The results of the NSS cohort suggest that these patients may exist in a compensated state in which a reduction in cerebral blood flow and VV allows for the maintenance of parenchymal health to prevent the development of intracranial hypertension. Further studies may explore VV as a surrogate marker of ICP elevation, and the utility of cranial vault remodeling on nonsynostotic pathologies with cephalocranial disproportion.

Long-Term Outcomes of Nonsyndromic Isolated Unilateral Lambdoid Craniosynostosis

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.

Genetic Heterogeneity, Craniofacial Surgical Burden, and Surgical Techniques in Patients With Saethre-Chotzen Syndrome.

While genotype correlates with phenotype in patients with many forms of syndromic craniosynostosis, the relationship between molecular diagnosis and craniofacial surgical history in patients with Saethre-Chotzen syndrome (SCS) is more variable. This manuscript characterizes that relationship and evaluates operative trends in these patients over the past 3 decades. Demographic information, molecular diagnosis, and craniofacial surgical history in patients born with SCS between 1989 and 2023 were compared with appropriate statistics, including t tests and analysis of variance. Thirty-five patients with SCS were included, and there was no difference in total craniofacial procedures among those with TWIST1 substitutions (2.1 ± 1.6), duplications (3.0 ± 4.2), insertions (3.5 ± 0.7), or deletions (2.4 ± 1.9; P = 0.97). Cranial expansion rates were also similar across all genetic diagnoses (P>0.05), and surgical incidence was similar across patients with unicoronal, bicoronal, and multisuture involvement (P > 0.05). Those with an initial fronto-orbital advancement had a lower incidence of secondary cranial vault procedures compared with those with an initial posterior vault distraction osteogenesis (29% versus 71%, P < 0.05), though this did not control for phenotypic severity. On average, total cranial vault surgical burden (1.35 ± 0.67 versus 1.75 ± 0.46) and cranial expansion surgical burden (1.40 ± 0.68 versus 1.88 ± 0.64) between the fronto-orbital advancement-first and posterior vault distraction osteogenesis-first cohorts were similar (P = 0.11, P = 0.17, respectively). While SCS is molecularly and phenotypically heterogeneous, genetic diagnosis does not appear associated with rates of craniofacial surgery. Additional prospective study of correlations between genotype, severity of craniofacial manifestations, and treatment algorithms is warranted; but, in the end, it may be that this highly variable form of syndromic craniosynostosis warrants tailored, expectant management.

Vaulting further: cranial vault expansion for craniocerebral disproportion without primary craniosynostosis.

Treatment of subjects with refractory idiopathic intracranial hypertension (IIH) or shunted hydrocephalus with chronic shunt complications is challenging. What is the role for cranial vault expansion, particularly utilizing posterior vault distraction osteogenesis (PVDO), in these cases? This study assesses medium-term efficacy of cranial vault expansion in this unique patient population. A retrospective review was conducted of patients who underwent cranial vault expansion from 2008 to 2023 at the Children's Hospital of Philadelphia. Subjects who did not have a diagnosis of primary craniosynostosis were included in the study. Demographic information, medical history, and perioperative details were collected from medical records. Primary outcomes were the rate of CSF diversion procedures and resolution of presenting signs and symptoms. Secondary outcomes were perioperative and 90-day complications and reoperation requirement. Among 13 included subjects, nine (69.2%) patients had a primary diagnosis of shunted hydrocephalus and 4 (30.8%) patients had IIH. Twelve (92.3%) subjects underwent posterior vault distraction osteogenesis (PVDO) and one (7.7%) underwent posterior vault remodeling (PVR). All 4 patients with IIH demonstrated symptomatic improvement following PVDO, including resolution of headaches, vomiting, and/or papilledema. Among 9 patients with shunted hydrocephalus, CSF diversion requirement decreased from 2.7 ± 1.6 procedures per year preoperatively to 1.2 ± 1.8 per year following cranial vault expansion (p = 0.030). The mean postoperative follow-up was 4.1 ± 2.1years and four (30.8%) patients experienced complications within 90days of surgery, including infection (n = 2), CSF leak (n = 1), and elevated ICP requiring lumbar puncture (n = 1). Four (30.8%) patients underwent repeat cranial vault expansion for recurrence of ICP-related symptoms. At most recent follow-up, 7 of 9 patients with shunted hydrocephalus demonstrated symptomatic improvement. Cranial vault expansion reduced intracranial hypertension-related symptomology as well as the rate of CSF diversion-related procedures in patients with refractory IIH and shunted hydrocephalus without craniosynostosis, and should be considered in those who have significant shunt morbidity.

Early posterior vault distraction osteogenesis changes the syndromic craniosynostosis treatment paradigm: long-term outcomes of a 23-year cohort study

BackgroundEarly surgical management of syndromic craniosynostosis varies widely between centers, with patients typically undergoing multiple intracranial procedures through childhood. This study evaluates the long-term impact of early posterior vault distraction osteogenesis (PVDO) versus conventional treatment paradigms on the number and timing of subsequent craniofacial procedures.MethodsWe retrospectively analyzed the longitudinal operative patterns of patients with syndromic craniosynostosis treated from 2000 to 2023 with greater than five years of follow-up. Outcomes of patients who underwent early PVDO and conventional vault reconstruction were compared.ResultsFifty-five patients met the inclusion criteria (30 PVDO and 25 conventional). Age at initial vault surgery was similar between the PVDO and conventional cohorts (7.6 vs. 8.8 months), as were baseline craniometrics (p > 0.05). Multiple fronto-orbital advancement (FOA) procedures were performed in only 1/30 (3%) PVDO-treated patient versus 12/25 (48%) conventionally-treated patients (p < 0.001). Twelve (40%) PVDO-treated patients did not undergo FOA at all. Among patients with Apert and Crouzon syndromes, fewer PVDO-treated patients required FOA prior to midface surgery (33% vs. 92%, p = 0.004) or repeat FOA (6% vs. 50%, p = 0.005) compared to conventionally-treated patients. Among patients with Muenke and Saethre–Chotzen syndromes, a similar proportion of patients required FOA in the PVDO and conventional cohorts (91% vs. 100%, p = 0.353), though no PVDO-treated patients required repeat FOA (0% vs. 44%, p = 0.018).ConclusionsEarly PVDO is associated with a reduction in the high burden of both FOA and, critically, revisionary frontal procedures which are commonly seen in conventionally-treated patients with syndromic craniosynostosis.

Posterior Vault Distraction in the Acute Setting.

Posterior vault distraction osteogenesis (PVDO) has been shown to resolve acute intracranial hypertension (AIH) while carrying an acceptable perioperative morbidity profile. PVDO has been associated with symptomatic improvement and fewer surgeries in those requiring ventriculoperitoneal shunts. The authors' experience using PVDO as an acute intervention is presented, demonstrating its safety and efficacy for management of AIH. Four cases of children with craniosynostosis that underwent PVDO in the acute setting are outlined. All patients presented with papilledema and symptoms of AIH. One patient with slit ventricle syndrome (SVS) presented with a nonfunctioning shunt following multiple shunt revisions. No intraoperative complications during distractor placement or removal were reported. Distraction protocol for all patients began on postoperative day 1 at 1-2 mm per day, resulting in an average total distraction of 30.25 mm. For the 3 cases with no shunt, the average length of stay was 7 days. As part of the planned treatment course, the patient with SVS required externalization of the shunt during distraction followed by early distractor removal and planned shunt replacement. One case of surgical site infection (in an immunocompromised patient) required premature distractor removal during the consolidation period. Computed tomography (CT) in all patients indicated increased intracranial volume following distraction, and symptomatic improvement was reported. Six-month follow-up showed resolution of papilledema in all patients. The authors' experience using PVDO in the acute setting is reported, alongside a review of current literature, in order to provide supporting evidence for the efficacy of PVDO as a tool for resolving AIH.

Apert syndrome: neurosurgical outcomes and complications following posterior vault distraction osteogenesis.

Posterior vault distraction osteogenesis (PVDO) has been utilized during the past 15 years to treat a variety of clinical features commonly presented by patients with Apert syndrome. The objective of this study is to determine the efficacy of PVDO in addressing both elevated intracranial pressure (ICP) and ectopia of the cerebellar tonsils (ECT) in young Apert patients. In addition, we aimed to determine the prevalence of hydrocephalus in Apert syndrome patients who underwent PVDO. A retrospective study was made with a cohort of 40 consecutive patients with syndromic craniosynostosis (SC), previously diagnosed with Apert syndrome, who underwent PVDO between 2012 and 2022, and thereafter received at least 1 year of follow-up care. Demographic data and diagnosis, along with surgical and outcome data, were verified using medical records, clinical photographs, radiologic examination, and interviews with the parents of all cohort patients. The average patient age when PVDO was performed was 12.91 ± 10 months. The average posterior advancement distance achieved per patient was 22.68 ± 5.26mm. The average hospital stay per patient was 3.56 ± 2.44 days. The average absolute and relative blood transfusion volumes were 98.47ml and 17.63ml/kg, respectively. Although five patients (14%) presented ECT preoperatively, this condition was completely resolved by PVDO in three of these five patients. One of the three patients whose ECT had completely resolved presented syringomyelia postoperatively, requiring subsequent extra dural foramen magnum decompression. All of the remaining four patients were asymptomatic for ECT for at least 1 year of follow-up, and none of these four patients required any additional treatments to address ECT. Two patients presented hydrocephalus requiring ventriculoperitoneal shunt placement. This study demonstrates that PVDO both reduces diagnosed elevated ICP symptoms and is partially effective in treating ECT in Apert syndrome patients. Hydrocephalus in Apert syndrome is an uncommon feature. The effectiveness of PVDO in addressing hydrocephalus is uncertain.

D36. Early Posterior Vault Distraction Osteogenesis is changing the Syndromic Craniosynostosis Treatment Paradigm: Outcomes of A 20-year Cohort Study

D36. Early Posterior Vault Distraction Osteogenesis is changing the Syndromic Craniosynostosis Treatment Paradigm: Outcomes of A 20-year Cohort Study

Impact of Anterior and Posterior Vault Distraction Osteogenesis (A-PVDO) and 3D-Printed Positioning/Shaping Templates in Apert Syndrome: A Case Series Study.

This study seeks to examine the impact of anterior and posterior vault distraction osteogenesis (A-PVDO) in conjunction with 3D-printed positioning and shaping templates for the management of Apert syndrome. From January 2018 to February 2022, a retrospective analysis was conducted on 6 cases of Apert syndrome employing fronto-orbital 3D-printed positioning and molding templates. The cranium underwent surgical modification in accordance with the template's configuration and was affixed with absorbable plates. Subsequently, distraction devices were applied, encompassing both anterior and posterior craniotomies. The evaluation encompassed clinical outcomes, complications (including cerebrospinal fluid leakage and infection), safety, and the feasibility of the distraction osteogenesis procedure. Six patients diagnosed with Apert syndrome underwent treatment involving the integration of fronto-orbital 3D-printed positioning and shaping templates in conjunction with anterior and posterior cranial distraction osteoplasty. Follow-up durations ranged from 18 to 32 months (average: 22mo). No instances of fronto-orbital retraction, cerebrospinal fluid leakage, or intracranial infection were noted during the follow-up period. The sole reported complication entailed an infection at the extension rod site in 1 case. All patients conveyed satisfaction with the treatment outcomes. The application of 3D-printed positioning and shaping templates in tandem with anterior and posterior cranial distraction osteogenesis demonstrates efficacy in addressing Apert syndrome. Notably, significant enhancements in head shape and orbit were observed, and the incidence of postoperative complications such as cerebrospinal fluid leakage and infection remained minimal. Moreover, long-term follow-up affirmed stability.

Metastatic lesions of the uterus, fallopian tubes and ovaries in undifferentiated pleomorphic sarcoma of the left tibia (clinical case)

Undifferentiated pleomorphic osteosarcoma belongs to the group of rarely occurring tumors. Despite the treatment, the disease progresses in 30–40 % of patients with osteosarcomas. The main route of metastasis of bone tissue sarcomas is hematogenous, while lymphogenic spread is observed less frequently. As a rule, secondary metastatic changes occur in the lungs. Less often there is a secondary lesion of the bones of the skeleton and brain. Metastatic lesion of uterus, fallopian tubes and ovaries in malignant undifferentiated pleomorphic sarcoma is extremely rare. Therefore, we found it interesting to describe a clinical case of such a rare metastatic lesion. Patient K. underwent amputation of the left limb at the level of the lower third of the femur for undifferentiated pleomorphic sarcoma of the left tibia in 2019, and 4 courses of adjuvant polychemotherapy were performed. In 20 months after completion of complex treatment of the primary tumor, complaints of lower abdominal pain, increased body temperature up to 37.8 °C in the evenings appeared. According to the results of follow-up examination, a voluminous, multinodular, solid mass of merging character was detected in the pelvis, with total dimensions of up to 11 cm, and a cavitary mass of up to 5 cm was detected in the posterior vault. A trepan-biopsy of the mass in the projection of the right ovary was performed. The morphological picture in the volume of trepan biopsy specimens is characteristic of spindle cell sarcoma. Metastasis of undifferentiated pleomorphic bone sarcoma (malignant fibrous histiocytoma) is most likely. Due to metastatic lesions of the uterus, fallopian tubes, ovaries, omentum, mesentery and serous membrane of the colon loops, peritoneum of the bladder, surgical intervention in the volume of removal of the distal part of the sigmoid colon, rectosigmoid, upper ampullary parts of the rectum, uterus with fallopian tubes and ovaries, appendix was performed. Immunohistochemical study of the postoperative material revealed that the immunophenotype of tumor cells confirmed the morphological picture typical for undifferentiated pleomorphic bone sarcoma. The patient was further prescribed antitumor drug therapy. This clinical case demonstrates a rare, atypical metastasis of undifferentiated pleomorphic osteosarcoma, which allows to expand the knowledge about the flow of malignant diseases of this localization.

The Use of Resorbable Fixation for Posterior Cranial Vault Distraction Osteogenesis

Summary: Posterior vault distraction osteogenesis (PVDO) traditionally relies on the use of cranial distractors affixed with titanium screws to the posterior cranial vault transport segment to allow for expansion of intracranial volume. We describe a novel technique using the Synthes RAPIDSORB IPS resorbable fixation system for stable fixation of the distraction hardware for PVDO. In a retrospective review of our experience with the IPS system, there was no instance of hardware or fixation failure. This off-label use of the IPS system results in a significant reduction in operative time for hardware removal while allowing for a more limited dissection, which can help reduce devascularization of the bony regenerate, bony relapse, and other complications.

Spring-assisted posterior vault expansion: a parametric study to improve the intracranial volume increase prediction

Spring-assisted posterior vault expansion has been adopted at the London Great Ormond Street Hospital for Children to treat raised intracranial pressure in patients affected by syndromic craniosynostosis, a congenital calvarial anomaly causing the premature fusion of skull sutures. This procedure involves elastic distractors used to dynamically reshape the skull and increase the intracranial volume (ICV). In this study, we developed and validated a patient-specific model able to predict the ICV increase and carried out a parametric study to investigate the effect of surgical parameters on that final volume. Pre- and post-operative computed tomography data relative to 18 patients were processed to extract simplified patient-specific skull shape, replicate surgical cuts, and simulate spring expansion. A parametric study was performed to quantify each parameter’s impact on the surgical outcome: for each patient, the osteotomy location was varied in a pre-defined range; local sensitivity of the predicted ICV to each parameter was analysed and compared. Results showed that the finite element model performed well in terms of post-operative ICV prediction and allowed for parametric optimization of surgical cuts. The study indicates how to optimize the ICV increase according to the type of procedure and provides indication on the most robust surgical strategy.

Effects of Posterior Vault Distraction Osteogenesis on Ventricular Morphology and Volume

Effects of Posterior Vault Distraction Osteogenesis on Ventricular Morphology and Volume

Oxycephaly-systematic review, case presentation, and diagnostic clarification.

Oxycephaly is a specific phenotype of multi-suture craniosynostosis that is often misrepresented. This study aims to review the relevant literature, clarify the diagnostic criteria, and present an alternate approach to its management. Published literature regarding oxycephaly was reviewed from 1997, when the largest series was published, until 2022. All cases at a single institution were then retrospectively reviewed. Over the last 25years, four studies met the inclusion criteria, none of which specifically defined oxycephaly. One case, in one study, was potentially consistent with the phenotype. An institutional review yielded two patients who met the original diagnostic criteria set forth by Renier and Marchac. Both patients had unexplained speech delays, mild retinal nerve fiber layer thickening, and diffuse inner table scalloping, along with the characteristic oxycephalic phenotype. One patient also had a direct intracranial pressure (ICP) measurement of 25mmHg, and the other had a Chiari I malformation. Both were treated with posterior vault distraction osteogenesis (PVDO) to alleviate the cephalo-cranial disproportion while simultaneously allowing for turricephaly correction. Oxycephaly presents with late onset multi-suture fusion. Patients have patent sutures at birth. Midface hypoplasia and known syndromic associations are absent. Patients demonstrate supraorbital recession, an obtuse fronto-nasal angle, and turricephaly without substantial brachycephaly. Over 60% of patients have symptomatic ICP elevation, the presentation of which ranges from headaches to rapidly progressive blindness. This rare form of craniosynostosis is particularly virulent and likely often missed due to diagnostic ambiguity and its relatively mild phenotype.

Vertical rotation of phakic intraocular lens to achieve optimal vaulting in a myopic eye

Optimal implantable collamer lens (ICL) sizing and normal postoperative ICL vault are important to avoid adverse events. Spherical phakic intraocular lens (PIOL) implantation was done for a case of both eye myopia. Both eyes had similar ocular biometry, hence the same size PIOL was ordered. The right eye had postoperatively, a high posterior vault of 2000 μ on POD1, which reduced to 1000 μ on POD2. Re-evaluation confirmed similar ocular and PIOL parameters in both eyes. The PIOL was rotated 90° to a vertical rotation after which a reduction in the vault from 1000 to 700 μ was achieved. Satisfactory refractive outcome and optimal vault were maintained during subsequent visits. The left eye course was uneventful.

Frontofacial Features of Unilateral Lambdoid Craniosynostosis: A Multicenter Assessment

Background: Unilateral lambdoid craniosynostosis is differentiated from deformational plagiocephaly primarily by assessing the cranium from posterior and bird’s-eye views. Findings include posterior displacement of the ipsilateral ear, ipsilateral occipitomastoid bossing, ipsilateral occipitoparietal flattening, contralateral parietal bossing, and contralateral frontal bossing. Diagnosis based off facial morphology may be an easier approach because the face is less obstructed by hair and head-coverings, and can easily be assessed when supine. However, frontofacial characteristics of unilateral lambdoid craniosynostosis are not well described. Methods: A retrospective cohort review of patients with isolated, unilateral lambdoid craniosynostosis from the Children’s Hospital of Pittsburgh and the Children’s Hospital of Philadelphia was performed. Preoperative frontal and profile photographs were reviewed for salient characteristics. Results: Nineteen patients met inclusion criteria. Eleven patients had left lambdoid craniosynostosis, and eight had right lambdoid craniosynostosis. All patients were nonsyndromic. Patients demonstrated contralateral parietal bossing and greater visibility of the ipsilateral ear. Contralateral frontal bossing was mild. The orbits were tall and turricephaly was present in varying severity. Facial scoliosis as a C-shaped deformity was present in varying severity. The nasal root and chin pointed to the contralateral side. Conclusions: The combination of greater visibility of the ipsilateral ear, contralateral parietal bossing, and C-shaped convex ipsilateral facial scoliosis are hallmark frontofacial features of unilateral lambdoid craniosynostosis. Although the ipsilateral ear is more posterior, the greater visibility may be attributed to lateral displacement from the mastoid bulge. Evaluation of long-term postoperative results is needed to assess if this pathognomonic facial morphology is corrected following posterior vault reconstruction.

Hyper‐IgE syndrome presenting with early life craniosynostosis in monozygotic twin sisters

<scp>Hyper‐IgE</scp> syndrome presenting with early life craniosynostosis in monozygotic twin sisters