A Systematic Review of Idiopathic Secondary Stenosis Following Index Surgery for Craniosynostosis.

BACKGROUND: Primary craniosynostosis is a congenital craniofacial disorder in which cranial sutures prematurely close. Iatrogenic secondary stenosis is abnormal cranial suture closure caused by surgical manipulation of the suture. In contrast, idiopathic secondary stenosis develops in a suture that did not undergo surgical manipulation. The objective of this systematic review was to consolidate and characterize the incidence, classification, and management of idiopathic secondary stenosis in the literature. METHODS: Literature from PubMed, Web Of Science, and EMBASE from 1970 to March 2022 was reviewed. The following information was extracted for individual patients: incidence of idiopathic secondary stenosis, index primary craniosynostosis, primary surgical correction, presenting signs of secondary stenosis, management, and further complications. RESULTS: Seventeen articles detailing 1181 patients were included. Ninety-one developed idiopathic secondary stenosis (7.7%). Only 3 of these patients were syndromic. The most common index craniosynostosis was sagittal synostosis (83.5%). The most common suture undergoing idiopathic secondary stenosis was the coronal suture (91.2%). Patients presented at a median age of 24 months. The most common presenting sign was a radiologic finding (85.7%), although some patients presented with headache or head deformity. Only 2 patients, both syndromic, had complications following surgical correction of secondary stenosis. CONCLUSIONS: Idiopathic secondary stenosis is a rare, long-term complication following index surgical repair of craniosynostosis. It can occur following any surgical technique. It most commonly affects the coronal suture but can affect any of the sutures, including pansynostosis. Surgical correction is curative in nonsyndromic patients.

Comparative Analysis of 2D and 3D Metrics for Evaluation of Postoperative Outcomes Following Endoscopic Suturectomy for Sagittal Craniosynostosis.

To assess whether 3-dimensional (3D) volumetrics can be used to track and evaluate postoperative course of patients treated with endoscopic suturectomy for nonsyndromic sagittal synostosis, we compared changes in 2-dimensional (2D) measurements along with 3D volumetric correlates throughout the period of helmet therapy. Forty-six patients treated at our institution with endoscopic suturectomy for sagittal synostosis were retrospectively reviewed. Head circumference (HC), cephalic index (CI), and total cranial volumes (TCVs) were measured at 3 timepoints following surgery using optical surface scans obtained for helmet orthotics. All measurements showed significant differences between timepoints on the analysis of variance ( P <0.001). There was a significant correlation between CI and TCV (r=0.35, P =0.004) and between HC and TCV (r=0.81, P <0.001). The normalized rate of change over the course of treatment was significantly higher for TCV (36.7%) than for CI (8.8%) and HC (8.4%, P <0.001), with no difference between HC and CI. The authors conclude that 3D metrics were able to reliably follow the course of postoperative 2D metrics. There was a direct and linear correlation between HC and CI with TCV. Total cranial volumes showed the highest rate of sustained change at every timepoint. Although CI and HC plateau after the first measurement, TCV continues to adapt over the course of treatment. These results demonstrate the feasibility and value of volumetrics from 3D imaging to provide a more comprehensive evaluation of postoperative surgical outcomes than traditional 2D metrics without the ionizing radiation traditionally utilized for CT to obtain 3D metrics.

Inferiorly Directed Posterior Cranial Vault Distraction for Treatment of Chiari Malformations.

OBJECTIVE: The authors present a case series of patients with Chiari I malformations treated with distraction osteogenesis of the posterior cranial vault, utilizing a vertical distraction vector for appropriate cranial vault expansion while mitigating the risks of scaphocephaly and cerebellar ptosis. PATIENTS AND METHODS: Patients with syndromic and nonsyndromic Chiari I malformations treated with vertical-vector distraction osteogenesis of the posterior cranial vault were identified from 2008 to 2014. Demographics, preoperative and postoperative clinical symptoms, and perioperative details were assessed. Long-term esthetic outcomes, complications, and symptomatic improvement were evaluated in conjunction with neurosurgery. RESULTS: Nine patients were identified. Five had known syndromes, 2 likely had unidentified syndromes, and 2 were nonsyndromic. Seven had prior Chiari-related surgeries. Most presented with hydrocephalus, motor symptoms, and developmental delay. Operatively, 2 to 3 internal distraction fixators were applied such that the vector of distraction was along a cephalad-caudad axis. Devices were activated on postoperative day 5 and distracted 1 mm per day. Three postoperative complications were found within the first 3 months which included a dislodged distraction arm, a device extrusion, and a local cellulitis. No complications affected the clinical outcome. Radiographic follow-up showed good bone formation, decompression of the posterior fossa, improved cerebrospinal fluid flow, and no cerebellar ptosis. Neurological surveillance showed improvement in intracranial pressure, hydrocephalus, motor symptoms, and behavioral problems. CONCLUSION: The authors have presented 9 patients with Chiari I malformations treated with distraction osteogenesis, along with a novel technique to safely and effectively expand the posterior fossa while minimizing the risk of cerebellar ptosis. LEVEL OF EVIDENCE: Level II (prognostic/risk studies).

The Simultaneous Use of Spring-Mediated Cranioplasty and Distraction Osteogenesis as a Safe Modality for Multi-Suture Craniosynostosis.

ABSTRACT: Craniosynostosis caused by premature fusion of the cranial sutures most commonly involves a single suture. Less commonly, multiple sutures may fuse prematurely resulting in complex craniosynostosis. The authors present 1 case of a patient with unilateral sagittal and unilateral lambdoid craniosynostosis treated safely simultaneous with spring-mediated cranioplasty and distraction osteogenesis.

Characterization of Treatment Modalities for Patients With Syndromic Craniosynostosis in Relation to Degree of Midface Hypoplasia and Patient's Age Using Longitudinal Follow-Up Data.

ABSTRACT: The purpose of this study was to investigate the type and frequency of use of treatment modalities (Tx-Mods) in patients with syndromic craniosynostosis (SC) using longitudinal follow-up data. A total of 28 patients with SC (24 Crouzon, 2 Apert, and 2 Antley-Bixler syndromes), who were treated at the Department of Orthodontics, Seoul National University Dental Hospital, Seoul, South Korea between 1998 and 2020, was included. According to the degree of midface hypoplasia (MH) at the initial visit (T1), the patients were divided into the mild-MH (78°≤SNA < 80°, n = 8), moderate-MH (76≤SNA < 78°, n = 7), and severe-MH (SNA < 76°, n = 13) groups. T1-age and Tx-Mods, including cal-varial surgery (CALS), orthopedic treatment (OPT), fixed orthodontic treatment, and midface advancement surgery in childhood (MAS-child) and adulthood (MAS-adult), were investigated. Complexity of MAS-adult was graded as follows: 0, no surgery; 1, orthognathic surgery; 2, distraction osteogenesis (DOG); 3, combination of distraction osteogenesis and orthognathic surgery. Then, statistical analysis was performed. Percentage distribution of Tx-Mods was 71.4% in CALS, 21.4% in MAS-child, 42.9% in OPT, 100% in fixed orthodontic treatment, and 89.3% in MAS-adult. 92.9% of patients underwent MAS more than once. The number of MAS increased according to the severity of MH ( P < 0.05). The complexity of MAS-adult increased as T1-age and severity of MH increased (all P < 0.05); whereas it decreased when CALS and OPT were performed (all P < 0.05). However, MAS in childhood did not guarantee the avoidance of additional MAS in adulthood ( P > 0.05). These findings may be used as basic guidelines for successful treatment planning and prognosis prediction in patients with SC.

The Dura Split Technique in the Treatment of Craniosynostosis: Is It Still an Option?

BACKGROUND: The aim of this study was to report the outcome and the complications for patients operated on for craniosynostosis using the dura split technique. Specifically, the authors aimed to evaluate the safety of this technique, which is currently not in use, and to determine whether it is still useable. METHODS: The data was collected from the hospital patient records of all children surgically treated for craniosynostosis using the dura split technique in Turku University Hospital during the period 1975 to 2015. The data was analyzed to determine the clinical and radiological outcomes of the surgical procedure, the need for reoperations, and the rate of complications. RESULTS: During the study period, the dura split technique was used in the surgery of 65 patients. The outcome was either good or acceptable in most patients and reoperation was needed in only 2 patients (3.1%). Surgical complications included significant blood loss (26.2%), lesions on the inner layer of the dura (21.5%), leakage of cerebrospinal fluid (13.8%), and persistent bone defects (15.4% on palpation and 63.1% radiologically). CONCLUSIONS: Although the outcome of surgery for craniosynostosis using the dura split technique was mostly acceptable and the need for reoperations rare, the technique cannot, however, be recommended in the future due to high rates of bone defects, frequent problems with lesions on the inner layer of the dura, and consequent perioperative leakage of cerebrospinal fluid.

PLGA-based control release of Noggin blocks the premature fusion of cranial sutures caused by retinoic acid.

Craniosynostosis (CS), the premature and pathological fusion of cranial sutures, is a relatively common developmental disorder. Elucidation of the pathways involved and thus therapeutically targeting it would be promising for the prevention of CS. In the present study, we examined the role of BMP pathway in the all-trans retinoic acid (atRA)-induced CS model and tried to target the pathway in vivo via PLGA-based control release. As expected, the posterior frontal suture was found to fuse prematurely in the atRA subcutaneous injection mouse model. Further mechanism study revealed that atRA could repress the proliferation while promote the osteogenic differentiation of suture-derived mesenchymal cells (SMCs). Moreover, BMP signal pathway was found to be activated by atRA, as seen from increased expression of BMPR-2 and pSMAD1/5/9. Recombinant mouse Noggin blocked the atRA-induced enhancement of osteogenesis of SMCs in vitro. In vivo, PLGA microsphere encapsulated with Noggin significantly prevented the atRA-induced suture fusion. Collectively, these data support the hypothesis that BMP signaling is involved in retinoic acid-induced premature fusion of cranial sutures, while PLGA microsphere-based control release of Noggin emerges as a promising strategy for prevention of atRA-induced suture fusion.

The first Korean case report with scaphocephaly as the initial sign of X-linked hypophosphatemic rickets.

INTRODUCTION: X-linked hypophosphatemic rickets (XLH) can occasionally cause premature fusion of cranial sutures through an increased level of fibroblast growth factor 23 (FGF-23), which leads to the dysregulation of phosphate and vitamin D metabolism. Secondary craniosynostosis has long been considered to present late after XLH has already been diagnosed either clinically or genetically. CASE PRESENTATION: We present observations of a male infant showing sagittal synostosis as the first sign of XLH. Our patient did not show any other skeletal deformities except macrocephaly with a long head shape. There is a family history of genetically unconfirmed hypophosphatemic rickets in his mother. Direct sequencing by genomic polymerase chain reaction revealed that the patient has a large deletion comprising exons 1-3 of the phosphate regulating endopeptidase homolog X-linked (PHEX) gene. CONCLUSION: Our observations suggest that craniosynostosis secondary to rickets can develop in early infancy. Careful monitoring of head shape and growth is therefore critical for early detection of craniosynostosis in XLH.

Characterization of Perinatal Risk Factors and Complications Associated With Nonsyndromic Craniosynostosis.

BACKGROUND: Certain intrauterine risk factors are known to increase the risk of premature cranial suture fusion and may cause complications during birth. Some of these risk factors may be modifiable. Therefore, the authors sought to characterize the institutional patterns of prenatal risk factors and perinatal complications in nonsyndromic craniosynostosis patients compared to normal births from the surrounding area to identify areas for possible intervention or prevention. METHODS: The medical records of all infants with nonsyndromic craniosynostosis and full birth records born at Duke University Health System from 2006 to 2017 were retrospectively reviewed. Maternal comorbidities, prenatal risk factors, and perinatal complications were collected. The North Carolina State Center for Health Statistics was queried for perinatal statistics from Durham county and the Northeastern Perinatal Care Region to represent a control cohort of normal births from the same time period and region. The primary outcome investigated was the incidence of prenatal risk factors and complications at birth associated with premature fusion of cranial sutures. RESULTS: Eighty births with nonsyndromic craniosynostosis were included in this study. The majority of these patients were males (61.7%) and born via cesarean section (55.0%). Intrauterine growth restriction occurred in 10.0% and head trauma during delivery occurred in 2.5%. Twinning (14.8% vs 3.6%, P < 0.0001), cesarean births (55.5% vs 30.0%, P < 0.0001), and breech presentation (17.3% vs 3.2%, P < 0.0001) were significantly more common in craniosynostosis patients. Prenatally, mothers of craniosynostosis infants had higher incidence of gestational diabetes (13.5% vs 5.0%, P < 0.0001) and oligohydramnios (6.1% vs 1.3%, P < 0.0001) compared to regional controls. CONCLUSION: This study demonstrates that premature suture fusion is associated with prenatal risk factors such as gestational diabetes and oligohydramnios. Continued research into potentially modifiable prenatal risk factors and more refined prenatal diagnostic tools has the potential to reduce both the incidence of premature suture fusion and the sequelae of birth complications in this population.

Primary normocephalic pancraniosynostosis detected incidentally after an accidental head injury: a case report and review of the literature.

OBJECTIVE: Majority of multi-suture craniosynostosis are related to single-gene disorders or chromosomal abnormalities. Children with craniosynostosis usually present at an early age due to the presence of an abnormal head shape, with the exception of a unique entity termed primary normocephalic pancraniosynostosis. The objective of this article is to describe an unusual case of primary normocephalic pancraniosynostosis, detected incidentally following an accidental head injury. A comprehensive review of the literature will also be included. To the best of our knowledge, only eight cases of primary normocephalic pancraniosynostosis have been reported thus far. CASE DESCRIPTION: A 3-year 2-month-old child presented to the emergency department after a fall with severe scalp swelling. The child was noted to have mild frontal bossing and bilateral exophthalmos. Head size was normal but bilateral mild papilloedema was noted. CT scan was performed and demonstrated pancraniosynostosis and diffuse subgaleal hematoma. Patient underwent fronto-orbital advancement and total cranial vault reconstruction with favorable outcome. CONCLUSION: Our reported case adds to the current limited knowledge of this rare entity and emphasized the importance of a high index of suspicion in children with apparently normal head size and shape but show subtle evidence of raised intracranial pressure.

The Pantheon Variant of the Classic Cathedral Dome Procedure for Parieto-Occipital Skull Deformities.

OBJECTIVE: To describe a new technique for the reconstruction of parieto-occipital skull deformities. METHODS AND RESULTS: The technique is a variant of the previously described "cathedral dome procedure" used for frontal skull deformities. The authors apply the same principle of remodeling by making meridional slat craniotomies surrounding the depressed dome of the posterior skull deformity, followed by "green-stick fracture-reshaping" of the meridional slats to elevate and support the excised depressed dome. The authors present an illustrative patient with a follow-up of 2 years and an excellent cosmetic result. CONCLUSION: The authors introduce a new operative technique for the reconstruction of parieto-occipital skull deformities with excellent long-term results. The freshly reconstructed occiput resembles the dome of the pantheon in Rome, Italy, whose unique features inspired us to name this procedure the "Pantheon" variant of the cathedral dome operation.

Isolated Post-Shunt Metopic Synostosis and Neural Tube Defects.

BACKGROUND: Craniosynostosis is an uncommon complication after shunting procedures for congenital hydrocephalus. We report a case of a child with myelomeningocele and normocephaly at the time of birth. She underwent ventricular shunting for Chiari malformation and hydrocephalus at 3 days of age. An immediate postoperative CT scan confirmed all sutures were open. Serial CT scans document an open metopic suture at 2 months, closed metopic suture at 5 months, and trigonocephaly at 11 months with concomitant slit ventricle syndrome, and collapsed lateral and third ventricles. METHODS: An Ovid MEDLINE search within the dates of 1948 through 2017, using the keywords "synostosis AND shunt" was carried out. A tabulation of all patients and their respective synostosis patterns were recorded. RESULTS: We identified 8 case series and 2 case reports during 43 years (1966-2017). Seventy-eight patients with 79 suture synostosis patterns were identified (one patient underwent a second cranial reconstruction for identification of a separate, newly formed synostosis). Eighteen (30.5%) cases were associated with a neural tube defect (NTD). Patients with NTD and secondary craniosynostosis had on average earlier age of shunt placement (P = 0.001), craniosynostosis presentation (P = 0.146), and cranioplasty (P = 0.325) than secondary craniosynostosis patients without NTD. CONCLUSIONS: Ventricular shunt drainage in treating hydrocephalus rarely may lead to early synostosis and cranial deformity, especially in patients with NTDs. Early shunt placement poses significant risk in patients with NTD. Close follow-up may be necessary to evaluate overdrainage and cranial deformity after shunting procedures.

Gene/environment interactions in craniosynostosis: A brief review.

It is suggested that craniosynostosis is caused by a heterogeneous set of effects including gene mutations, teratogenic exposure during critical periods of development and gene/environment interactions. Distinguishing between sufficient, additive and interactive effects is important to the study of gene/environment interactions and allows for segregation of environmental exposures effecting susceptible populations. Through the identification of sufficient and interactive effects, efforts in prevention of craniosynostosis may be successful. Here, we provide a brief review focusing on defining these categorized exposures and relevant literature that has interrogated gene/environment interactions for craniosynostosis.

[Shunt-induced craniosynostosis: topicality of the problem, choice of the approach, and features of surgical treatment]. / Shunt-indutsirovannyi kraniosinostoz: aktual'nost' problemy, vybor taktiki, osobennosti khirurgicheskogo lecheniia.

RATIONALE: Shunt-induced craniosynostosis is one of the late complications of CSF shunting surgery, which affects the patient's condition, clinical picture, and treatment approach. OBJECTIVE: to evaluate the prevalence rate and clinical significance of this disease, define the indications for surgery, and choose the optimal surgical approach. MATERIAL AND METHODS: The study included 59 children with shunt system dysfunction, aged 1 to 14 years, who were treated at the Department in the period from 2014 to 2016. The inclusion criteria were as follows: 1) age at the time of examination is older than 1 year; 2) implantation of a shunt system in the first 12 months of life. The state of cranial sutures was assessed using three-dimensional reconstruction of patient's computerized tomography images. Images obtained before or in the first months after primary implantation of a shunt system were used to exclude cases of primary craniosynostosis. RESULTS: Premature synostosis of the cranial sutures was detected in 27 (46%) cases. Of these, 3 (11%) patients with clinical symptoms of increased intracranial pressure and radiographic signs of craniocerebral disproportion underwent cranial vault remodeling surgery: two biparietal craniotomies and one fronto-parieto-occipital reconstruction. In two cases, simultaneous replacement of a valve with a programmable one was performed. There were no complications after reconstructive surgery. CONCLUSION: Shunt-associated craniosynostosis is one of the late complications of CSF shunting surgery. However, its presence is not an indication for surgery and should not be a reason for surgical aggression. Surgery for increasing the intracranial volume is indicated only for secondary craniosynostosis combined with signs of craniocerebral disproportion. In these cases, reconstructive surgery is an effective treatment option for improving the patient's condition.

Craniosynostosis in 10q26 deletion patients: A consequence of brain underdevelopment or altered suture biology?

Approximately a hundred patients with terminal 10q deletions have been described. They present with a wide range of clinical features always accompanied by delayed development, intellectual disability and craniofacial dysmorphisms. Here, we report a girl and a boy with craniosynostosis, developmental delay and other congenital anomalies. Karyotyping and molecular analysis including Multiplex Ligation dependent probe amplification (MLPA) and Array Comparative Genomic Hybridization (aCGH) were performed in both patients. We detected a 13.1 Mb pure deletion at 10q26.12-q26.3 in the girl and a 10.9 Mb pure deletion at 10q26.13-q26.3 in the boy, both encompassing about 100 genes. The clinical and molecular findings in these patients reinforce the importance of the DOCK1 smallest region of overlap I (SRO I), previously suggested to explain the clinical signs, and together with a review of the literature suggest a second 3.5 Mb region important for the phenotype (SRO II). Genotype-phenotype correlations and literature data suggest that the craniosynostosis is not directly related to dysregulated signaling in suture development, but may be secondary to alterations in brain development instead. Further, genes at 10q26 may be involved in the molecular crosstalk between brain and cranial vault.

Maternal occupational exposure to polycyclic aromatic hydrocarbons and craniosynostosis among offspring in the National Birth Defects Prevention Study.

BACKGROUND: Evidence in animal models and humans suggests that exposure to polycyclic aromatic hydrocarbons (PAHs) may lead to birth defects. To our knowledge, this relationship has not been evaluated for craniosynostosis, a birth defect characterized by the premature closure of sutures in the skull. We conducted a case-control study to examine associations between maternal occupational exposure to PAHs and craniosynostosis. METHODS: We used data from craniosynostosis cases and control infants in the National Birth Defects Prevention Study (NBDPS) with estimated delivery dates from 1997 to 2002. Industrial hygienists reviewed occupational data from the computer-assisted telephone interview and assigned a yes/no rating of probable occupational PAH exposure for each job from 1 month before conception through delivery. We used logistic regression to assess the association between occupational exposure to PAHs and craniosynostosis. RESULTS: The prevalence of exposure was 5.3% in case mothers (16/300) and 3.7% in control mothers (107/2,886). We observed a positive association between exposure to PAHs during the 1 month before conception through the third month of pregnancy and craniosynostosis (odds ratio [OR] = 1.75; 95% confidence interval [CI], 1.01-3.05) after adjusting for maternal age and maternal education. The number of cases for each craniosynostosis subtype limited subtype analyses to sagittal craniosynostosis; the odds ratio remained similar (OR = 1.76, 95% CI, 0.82-3.75), but was not significant. CONCLUSION: Our findings support a moderate association between maternal occupational exposure to PAHs and craniosynostosis. Additional work is needed to better characterize susceptibility and the role PAHs may play on specific craniosynostosis subtypes.

Residential agricultural pesticide exposures and risks of selected birth defects among offspring in the San Joaquin Valley of California.

BACKGROUND: We examined associations of birth defects with residential proximity to commercial agricultural pesticide applications in California. Subjects included 367 cases representing five types of birth defects and 785 nonmalformed controls born 1997 to 2006. METHODS: Associations with any versus no exposure to physicochemical groups of pesticides and specific chemicals were assessed using logistic regression adjusted for covariates. Overall, 46% of cases and 38% of controls were classified as exposed to pesticides within a 500 m radius of mother's address during a 3-month periconceptional window. RESULTS: We estimated odds ratios (ORs) for 85 groups and 95 chemicals with five or more exposed cases and control mothers. Ninety-five percent confidence intervals (CI) excluded 1.0 for 11 ORs for groups and 22 ORs for chemicals, ranging from 1.9 to 3.1 for groups and 1.8 to 4.9 for chemicals except for two that were <1 (noted below). CONCLUSION: For groups, these ORs were for anotia/microtia (n = 95 cases) and dichlorophenoxy acids/esters and neonicotinoids; anorectal atresia/stenosis (n = 77) and alcohol/ethers and organophosphates (these ORs were < 1.0); transverse limb deficiencies (n = 59) and dichlorophenoxy acids/esters, petroleum derivatives, and triazines; and craniosynostosis (n = 79) and alcohol/ethers, avermectins, neonicotinoids, and organophosphates. For chemicals, ORs were: anotia/microtia and five pesticides from the groups dichlorophenoxy acids/esters, copper-containing compounds, neonicotinoids, organophosphates, and triazines; transverse limb deficiency and six pesticides - oxyfluorfen and pesticides from the groups copper-containing compounds, 2,6-dinitroanilines, neonicotinoids, petroleum derivatives and polyalkyloxy compounds; craniosynostosis and 10 pesticides - oxyfluorfen and pesticides from the groups alcohol/ethers, avermectins, n-methyl-carbamates, neonicotinoids, ogranophosphates (two chemicals), polyalkyloxy compounds (two chemicals), and pyrethroids; and congenital diaphragmatic hernia (n = 62) and a copper-containing compound.

Perinatal features and rate of cesarean section in newborns with non-syndromic sagittal synostosis.

PURPOSE: The purpose of this study was to evaluate perinatal features and the rate of cesarean section in children with non-syndromic sagittal synostosis and to compare these with the official statistics. METHODS: The birth data of 36 consecutive children (25 boys) operated on using cranial vault remodeling because of primary sagittal synostosis were analyzed retrospectively from hospital records. The children were born between 2007 and 2011, and the surgery was performed before the age of 1 year. The official statistics of all Finnish newborns from the year 2010 (n = 61 371) were used as a reference. Chi-square and Fisher's exact tests were used in statistical analyses. RESULTS: The average gestational age of the newborns with sagittal synostosis was 39.8 weeks (reference 39.7 weeks). The average birth weight was 3565.8 g (3540 g) for boys and 3197.2 g (3427 g) for girls, and the average lengths at birth are 51 cm (50.4 cm) and 49.4 cm (49.6 cm), respectively. The average head circumference was 36 cm for both sexes (35.2 and 34.6 cm for reference boys and girls). The mean age of mothers was 30.5 years (30.1 years). The rate of cesarean section was significantly increased 30.5 % (reference 16.6 %), and the rate of suction cup delivery was increased 13.9 % (9 %). In addition, a prolonged or difficult delivery was reported in three childbirths. CONCLUSION: Newborns with non-syndromic sagittal synostosis appear to be of average birth size and gestational age. The incidences of perinatal complications and cesarean sections were increased with problems occurring in more than half of the childbirths.

Complications in Posterior Cranial Vault Distraction.

BACKGROUND: The use of posterior cranial vault distraction for the treatment of elevated intracranial pressure is gaining popularity and is a standard for first-stage cranial expansion in syndromic craniosynostosis at many institutions. However, although the operation is faster and less complex than other cranial vault remodeling procedures, it is not without its own unique set of complications. METHODS: We surveyed the published literature for case series and case control studies on posterior vault distraction. Complication rates and types for these series were tabulated and grouped by management. When outcomes were unclear, corresponding authors were contacted for clarification and treatment plans. RESULTS: Eleven reports were found from a search of all the literature on posterior cranial vault distraction with a range of 1 to 22 included patients. The average age at surgery was 16.2 ± 11.8 months. Complication rates ranged from 12.5% to 100%, with the average of 30% of patients across all studies. The most common complications reported were cerebrospinal fluid leak or dural injury, followed by wound infections or device exposures, and device failure. There were no reported patient deaths or long-term morbidities. CONCLUSIONS: Posterior cranial vault distraction is a relatively safe and effective therapy for the treatment of elevated intracranial pressure in the setting of syndromic craniosynostosis. The majority of described complications center on the interaction of the device with the dura, device extrusion, and infection. Extreme care must be used with the placement of these distraction devices and with handling of the dura at the osteotomy sites to ensure successful outcomes and avoid complications. LEVELS OF EVIDENCE: III.

Postnatal Pancraniosynostosis in a Patient With Infantile Hypophosphatasia.

Hypophosphatasia is a rare metabolic bone disorder that predisposes patients to craniosynostosis. Typically, patients born with hypophosphatasia will exhibit fused cranial sutures at birth. This is the first reported case of delayed onset of pancraniosynostosis in a patient with infantile hypophosphatasia. The severity of onset and delayed presentation in this patient are of interest and should give pause to those care providers who treat and evaluate patients with hypophosphatasia.