Cleft Palate-Craniofacial Journal最新文献

Objective: To investigate condylar bone density (BD) in children with craniofacial microsomia (CFM) and identify factors that contribute to early stage condylar resorption (CR) after mandibular distraction osteogenesis (MDO).

Design: Retrospective study.

Setting: Craniofacial department of a plastic surgery hospital.

Patients: Fifty-one children with CFM classified as Pruzansky IIa based on complete pre-(T0) and post-MDO (T1) computed tomography (CT) data.

Intervention and main outcome measurements: Mimic 21.0 (Materialise Inc., Belgium) was used to measure bilateral BD and condylar height (CH) and volume (CV) of affected side. Children were split into groups based on either affected side BD or the distraction length (DL,25 mm as cutoff) .Bilateral BD was compared using a paired t-test in each group. The CH and CV of affected side at T0 and T1 were compared. The relative values of the CH and CV (CH ratio) and the volume (CV ratio) of the affected side were compared across the groups.

Results: The BD was lower on affected side than on unaffected side. Regarding BD, CH and CV decreased after MDO in group I, while the CH ratio and CV ratio of group I was lower than that of groups II and III. Regarding DL, the CV ratio was lower in Group L than Group S.

Conclusions: The condylar bone quality on affected side is compromised in type IIa CFM. A low BD in combination with a larger distraction distance may increase the risk of CR; therefore, MDO in patients with such characteristics should be postponed.

Objective: To identify characteristics of malpractice litigations involving skull deformity in infants (craniosynostosis and deformational plagiocephaly).

Design: Retrospective review of all lawsuits with jury verdicts or settlements involving infant skull deformity as the primary diagnosis using the Westlaw Legal Database.

Setting: United States.

Patients, participants: Plaintiffs with skull deformity as the primary diagnosis.

Main outcome measures: Litigation outcome and indemnity payment amount.

Results: From 1990 to 2019, 9 cases involving infant skull deformity met our inclusion/exclusion criteria. Among these cases, 8 (88.9%) cases resulted in indemnity payments to plaintiffs, totaling $30,430,000. Failure to diagnose (n = 4, 44.4%) and surgical negligence (n = 3, 33.3%) were the most common reasons for litigations.

Conclusions: There were a small number of malpractice lawsuits involving infant skull deformity over three decades. When cases go to court, physicians and hospitals have a high likelihood of judgment against them, frequently resulting in high indemnity payments.

Background: Children born with Trisomy 13 or 18 (T13/18) often have multiple congenital anomalies, many of which drastically shorten their lifespan. Among these defects are cleft lip and palate, the repair of which presents an ethical dilemma to the surgeon given the underlying comorbidities associated with T13/18. The authors present an ethical discussion and institutional experience in navigating this dilemma.

Methods: The authors analyzed existing literature on T13 and T18 surgery and mortality. A retrospective study over ten years was also conducted to identify pediatric patients who underwent surgical correction of cleft lip and/or palate secondary to a confirmed diagnosis of T13/18. The authors identified two patients and examined their treatment course.

Results: The authors' review of literature coupled with their institution's experience builds on the published successes of correcting cleft lip and palate in the setting of T13/18. It was found that both patients identified in the case series underwent successful correction with no surgical complications.

Conclusion: A careful balance must be struck between improved quality of life, benefits of treatment, and risks of surgery in children with T13/T18. Careful consideration should be given to the medical status of these complex patients. If the remaining medical comorbidities are well managed and under control, there is an ethical precedent for performing cleft lip and palate surgeries on these children. A diagnosis of T13/T18 alone is not enough to disqualify patients from cleft lip/palate surgery.

Objective: The objectives of this study were to assess the oral health status and parental perception of oral health needs of pediatric patients in an urban Craniofacial Center.

Design: This research utilized a prospective cross-sectional matched study design. The data was collected prospectively via clinical oral examinations measuring dental caries experience and gingival health status. Parental perception of oral health was assessed through a validated questionnaire.

Setting: The study was conducted at a Pediatric Dentistry Department and Craniofacial Center (CFC) in a large urban American city.

Patients/participants: Participants were recruited and enrolled from a CFC and Pediatric Dental Clinic.

Main outcome measure(s): The outcome measures were the oral health status and parental perception thereof.

Results: CFC patients' caries experience in primary teeth was significantly lower than that of a healthy matched cohort, but statistically similar in permanent teeth. CFC patients had significantly higher unmet dental treatment needs. CFC patients had poor oral hygiene and were shown to have significantly higher plaque levels and worse gingival health than that of a healthy matched cohort. Parental perception of oral health did not show a statistically significantly difference between the two groups.

Conclusions: Patients in our study in an urban CFC were found to have a high unmet dental and poor oral hygiene. Despite the poor oral health status, parents of children with craniofacial anomalies did perceive their oral health as different from a matched cohort of patients without these conditions.

Rickets results from defective bone mineralization, leading to skeletal deformities. Among those deformities, rickets has been associated with craniosynostosis, the premature closure of cranial sutures. Most of these patients have fusion of major sutures. Rarely, squamosal craniosynostosis in association with rickets has been described. Squamosal craniosynostosis is noted as lacking a definitive head abnormality and difficult visualization on standard imaging modalities, leading to poor recognition. Careful attention should be given to rickets patients to monitor for these unusual suture closures. Additionally, craniosynostosis could be a presenting feature of rickets, and further rickets evaluation of the patient is indicated.

Objective: Measuring disability as a concept of impaired global function enables beneficiaries of treatment, the impact of treatment, and targets of health system investment to be rigorously assessed. Measures of disability are not well established for cleft lip and palate. This study aims to systematically review disability weight (DW) studies pertaining to orofacial clefts (OFCs) and identify methodological strengths and shortcomings of each approach.

Design: Systematic literature review of studies that met the following criteria: (1) peer-reviewed publication, (2) focus on disability valuation, (3) mention orofacial clefts, and (4) publication January 2001-December 2021.

Setting: None.

Patients/participants: None.

Interventions: None.

Main outcome measure(s): Disability weight method of valuation and the value itself.

Results: The final search strategy yielded 1,067 studies. Seven manuscripts were ultimately included for data extraction. The disability weights used in our studies, including those newly generated or taken from the Global Burden of Disease Studies (GBD), ranged widely for isolated cleft lip (0.0-0.100) and cleft palate with or without cleft lip (0.0-0.269). The GBD studies limited their consideration of cleft sequelae informing disability weights to impact on appearance and speech-related concerns, while other studies accounted for comorbidities such as pain and social stigma.

Conclusions: Current measures of cleft disability are sparse, inadequately reflect the comprehensive impact of an OFC on function and socialization, and are limited in detail or supporting evidence. Use of a comprehensive health state description in evaluating disability weights offers a realistic means of accurately representing the diverse sequelae of an OFC.

Objective: Develop a model for the study of Electronic Nicotine Device (ENDS) exposure on craniofacial development.

Design: Experimental preclinical design followed as pregnant murine dams were randomized and exposed to filtered air exposure, carrier exposure consisting of 50% volume of propylene glycol and vegetable glycine (ENDS Carrier) respectively, or carrier exposure with 20 mg/ml of nicotine added to the liquid vaporizer (ENDS carrier with nicotine).

Setting: Preclinical murine model exposure using the SciReq exposure system.

Participants: C57BL6 adult 8 week old female pregnant mice and exposed in utero litters.

Interventions: Exposure to control filtered air, ENDS carrier or ENDS carrier with nicotine added throughout gestation at 1 puff/minute, 4 h/day, five days a week.

Main outcome measures: Cephalometric measures of post-natal day 15 pups born as exposed litters.

Results: Data suggests alterations to several facial morphology parameters in the developing offspring, suggesting electronic nicotine device systems may alter facial growth if used during pregnancy.

Conclusions: Future research should concentrate on varied formulations and exposure regimens of ENDS to determine timing windows of exposures and ENDS formulations that may be harmful to craniofacial development.

Objective: To determine the prevalence of treatment of early childhood caries (ECC) using general anesthesia (GA) in children with cleft lip and/or palate (CL/P).

Design: Retrospective chart review of children followed by the Manitoba Cleft Lip and Palate Program (MCLPP) to determine the frequency of treatment for ECC under GA.

Setting: Children's Hospital, Winnipeg, Canada (a tertiary care centre).

Patients: Children registered with MCLPP between January 1, 2008- December 31, 2019.

Interventions: The chart review collected data on the following variables: sex, date of birth, postal code, type of cleft, whether child had treatment of ECC using GA, age at the time of GA, and cost of treatment.

Main outcome measures: Association of CL/P with ECC.

Results: Overall, 441 children had CL/P. 17% had isolated cleft lip (CL), 46% had isolated cleft palate (CP), and 37% had both cleft lip and palate (CLP). Overall, 24.3% of children with CL/P underwent dental surgery using GA while 14.5% underwent dental surgery to treat ECC between 12-59 months of age. When compared to a reference of Canadian healthy children 12-59 months of age, a child with CL/P was 15 times more likely to require GA to treat ECC.

Conclusion: Treatment for caries under GA in children with CL/P is common. In the children with CL/P the rates of GA for treatment of ECC are significantly higher when compared to the general population. Children with CL/P require comprehensive oral health prevention to reduce the risk for caries and the need for treatment under GA.

Objective: The purpose of this study is to assess cleft rhinoplasty terminology across phases of growth.

Design/setting: A systematic review was performed on cleft rhinoplasty publications over 20 years.

Interventions: Studies were categorized by age at surgical intervention: infant (<1 year); immature (1 to 14 years); mature (>15 years).

Main outcome measures: Collected data included terminology used and surgical techniques.

Results: The 288 studies included demonstrated a wide range of terminology. In the infant group, 51/54 studies used the term "primary." In the immature group, 7/18 studies used the term "primary," 3/18 used "secondary." In the mature group, 2/33 studies used the term "primary," 16/33 used "secondary," 2/33 used "definitive," 5/33 used terms such as "mature," "adult," and "late," and 8/33 did not use terminology.

Surgical technique assessment demonstrated: cleft rhinoplasty at infancy used nostril rim or no nasal incision, immature rhinoplasty used closed and open rhinoplasty incisions; and mature rhinoplasty used a majority of open rhinoplasty. Infant and immature cleft rhinoplasty incorporated septal harvest or spur removal in <10% of cases, whereas these procedures were common in mature rhinoplasty. No studies in infants or immature patients used osteotomies or septal grafts, common techniques in mature rhinoplasty.

Conclusions: Current terminology for cleft rhinoplasty is varied and inconsistently applied across stages of facial development. However, cleft rhinoplasty performed at infancy, childhood, and facial maturity are surgically distinct procedures. The authors recommend the terminology "infant," "immature," and "mature" cleft rhinoplasty to accurately describe this procedure within the context of skeletal growth.

Background: Cerebro-costo-mandibular syndrome (CCMS) is a rare congenital syndrome consisting of the main features of micrognathia and posterior rib gaps. Due to multiple abnormalities, patients almost have difficulty breathing with upper airway obstruction, decreased thoracic capacity, spina bifida, and scoliosis.

Case presentation: We describe a case of a late preterm neonate boy presenting with low Apgar, respiratory distress, and complicated orofacial anomalies that had a poor outcome. His radiographic findings showed mandibular hypoplasia (micrognathia), chest deformity, multiple posterior rib gap defects, and abnormal costotransverse articulation. Based on physical examination and radiologic findings, the diagnosis of CCMS confirmed for the patient.

Conclusion: Physicians should always consider the diagnosis of CCMS in all infants with micrognathia and rib-gap defects. These infants need careful respiratory function monitoring. Early airway management improves growth and development. In addition, their physical and psychological development should be assessed regularly.