Updated Guideline on Treatment and Management of Craniosynostosis

Mathijssen, Irene M.J.

doi:10.1097/scs.0000000000007035

Cited by 71 publications

(95 citation statements)

References 10 publications

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“…Additionally, this may result in exorbitism, globe subluxation, malocclusion, and aesthetic and/or psychosocial problems. 3,30 Up to 40% of patients may require some intervention for OSA. 23 Craniofacial anomaly is the second most common reason for infant tracheostomy, and the incidence of tracheostomy in children with craniofacial anomalies is approximately 20%.…”

Section: Discussionmentioning

confidence: 99%

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Syndromic craniofacial dysostosis involves the fusion of the cranial sutures and skull base sutures. [1][2][3][4] Clinical features of raised intracranial pressure (ICP) are a constant and early issue in children with this condition. 5 Additionally, due to midface hypoplasia, these patients have important morphological (e.g., facial retrusion, short nose, ocular dystopia, and strabismus) and functional (breathing difficulties, exorbitism, and recurrent globe dislocation) issues.

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confidence: 99%

“…face distraction is frequently postponed until the patient is older to avoid relapse. 3,22 Up to 40% of patients may require some intervention for OSA. 23,24 In the presence of severe airway and ocular issues, midface distraction can be performed early.…”

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confidence: 99%

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Robot-assisted frontofacial correction in very young children with craniofacial dysostosis syndromes: a technical note and early functional outcome

Udayakumaran

Krishnadas

Subash

2022

Neurosurgical Focus

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OBJECTIVE In this study, the authors aimed to 1) retrospectively analyze the early functional outcomes in a cohort of very young children with craniofacial dysostoses who underwent robot-assisted frontofacial advancement (RAFFA) or robot-assisted midface distraction (RAMD), and 2) analyze the utility of robotic assistance in improving the accuracy and safety of performing transfacial pin insertion for RAFFA or RAMD. METHODS A retrospective analysis of a cohort of 18 children (age range 1–42 months at presentation), who underwent RAFFA or RAMD from February 2015 to February 2021 in the craniofacial unit at Amrita Institute of Medical Sciences and Research Centre in Kochi, India, was performed. Inclusion criteria were patients who had undergone RAFFA in a single stage or RAMD where the cranial vault had been addressed earlier, had been addressed on follow-up, or had not been addressed and had follow-up of at least 6 months. RESULTS Overall, 18 children with syndromic craniosynostosis underwent LeFort level III midface distraction, with or without RAFFA, from February 2015 to February 2021 at a single center in India. The patients’ ages ranged from 6 to 47 months at the time of the procedure. All patients had significant obstructive sleep apnea (OSA), significant ocular issues, and disturbed sleep as determined by the authors’ preoperative protocol. Clinically significant intracranial pressure issues were present in 17 patients. None of the patients had injury due to the transfacial pin trajectory such as globe injury, damage to the tooth buds, or the loss of purchase during the active distraction phase. The mean distraction achieved was 23 mm (range 18–30 mm) (n = 16/18). Of the 18 patients, 10 (56%) had an excellent outcome and 6 (33%) had a satisfactory outcome. In all cases, the degree of OSA had significantly reduced after surgery. Eye closure improved in all patients, and complete closure was seen in 11 patients. On follow-up, the functional gain remained in 14 of 16 patients at the final follow-up visit. The distraction results were stable during the follow-up period (mean 36 months [range 6–72 months]). CONCLUSIONS The early RAFFA and RAMD protocols investigated in this study gave a significant functional advantage in very young patients with craniofacial dysostoses. The results have demonstrated the accuracy and safety of robotic assistance in performing transfacial pin insertion for RAFFA or RAMD.

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Section: Discussionmentioning

confidence: 99%

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Robot-assisted frontofacial correction in very young children with craniofacial dysostosis syndromes: a technical note and early functional outcome

Udayakumaran

Krishnadas

Subash

2022

Neurosurgical Focus

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“…Socioeconomic factors predispose toward a delayed primary surgery, which again causes an increase of the 30-day readmission rate and the risk for a surgical site infection ( 11 ). PAP is known to have an influence on wound infection rate as well, but distinct recommendations regarding the duration of PAP in craniofacial surgery are missing in the most recently published guideline ( 12 ). A recent systematic review of 39 studies with a total of 4,336 patients after head and neck surgery sought to elicit the optimal prophylactic antibiotic regimen for surgical site infections.…”

Section: Discussionmentioning

confidence: 99%

The Influence of Perioperative Antibiotic Prophylaxis on Wound Infection and on the Colonization of Wound Drains in Patients After Correction of Craniosynostosis

et al. 2021

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Objective: Evidence for the duration of perioperative antibiotic prophylaxis (PAP) after the correction of craniosynostosis in children is scarce. We evaluated the necessary duration of PAP to ensure a minimal rate of postoperative wound infections.Methods: In this monocentric, retrospective, and prospective pilot study, two PAP protocols were compared. From August 2017 to May 2018, treatment group 1 (TG 1) was treated using the standard PAP protocol with at least three doses of antibiotics. Between May 2018 and March 2019, a shortened PAP with a single-shot administration was given to treatment group 2 (TG 2a and b). Endpoints of this study were wound infection rate, colonization rate of wound drains, and the course of treatment reflected by clinical and laboratory data.Results: A cohort of 187 children underwent craniosynostosis correction: 167 were treated according to protocols-−95 patients with at least three doses (TG 1) and 72 patients with a single-shot of cefuroxime (TG 2a). Baseline characteristics were similar for both groups. We could not detect significant differences, neither for wound infection rates (TG 1: 1.1%, TG 2a: 0.0%, p = 0.38) nor for colonization rates of wound drains (TG 1: 4.8%, TG 2a: 10.5%, p = 0.27).Conclusions: Single-shot PAP had no adverse effects on the wound infection rate or the colonization rate of the wound drains compared with prolonged perioperative antibiotic prophylaxis. As a result, single-shot preoperative PAP is now applied to the majority craniosynostosis patients undergoing surgical correction in our unit.

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“…It can be divided into non-syndromic and syndromic craniosynostosis, with syndromic craniosynostosis being characterized by additional congenital anomalies, such as limb anomalies and neurodevelopmental delays (24,25). As part of our standard clinical care, all patients diagnosed with craniosynostosis are offered targeted genetic analysis (26). In a female patient with both CDH as well as craniosynostosis, we observed a genetic variant of the B cell leukemia 11b gene (BCL11B; OMIM 606558) resulting in an amino acid change at position 667 [p.(Gly667Glu)].…”

Section: Introductionmentioning

confidence: 99%

Case Report and Review of the Literature: Congenital Diaphragmatic Hernia and Craniosynostosis, a Coincidence or Common Cause?

et al. 2021

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Congenital diaphragmatic hernia (CDH) is a life-threatening birth defect that presents as either an isolated diaphragm defect or as part of a complex disorder with a wide array of anomalies (complex CDH). Some patients with complex CDH display distinct craniofacial anomalies such as craniofrontonasal dysplasia or craniosynostosis, defined by the premature closure of cranial sutures. Using clinical whole exome sequencing (WES), we found a BCL11B missense variant in a patient with a left-sided congenital diaphragmatic hernia as well as sagittal suture craniosynostosis. We applied targeted sequencing of BCL11B in patients with craniosynostosis or with a combination of craniosynostosis and CDH. This resulted in three additional BCL11B missense mutations in patients with craniosynostosis. The phenotype of the patient with both CDH as well as craniosynostosis was similar to the phenotype of previously reported patients with BCL11B missense mutations. Although these findings imply that both craniosynostosis as well as CDH may be associated with BCL11B mutations, further studies are required to establish whether BCL11B variants are causative mutations for both conditions or if our finding was coincidental.

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Updated Guideline on Treatment and Management of Craniosynostosis

Cited by 71 publications

References 10 publications

Robot-assisted frontofacial correction in very young children with craniofacial dysostosis syndromes: a technical note and early functional outcome

Robot-assisted frontofacial correction in very young children with craniofacial dysostosis syndromes: a technical note and early functional outcome

The Influence of Perioperative Antibiotic Prophylaxis on Wound Infection and on the Colonization of Wound Drains in Patients After Correction of Craniosynostosis

Case Report and Review of the Literature: Congenital Diaphragmatic Hernia and Craniosynostosis, a Coincidence or Common Cause?

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