Craniosynostosis: caring for infants and their families.

Craniosynostosis is a developmental anomaly with premature closure of the cranial sutures causing an abnormally shaped skull in an infant. Recommended surgical treatment involves cranial vault reconstruction to open the closed suture, increase intracranial volume, and allow the brain to grow normally. Parents work with a multidisciplinary team during the evaluation process and face various preoperative and postoperative stressors. Critical care nurses can improve the care of the infants and their families by being knowledgeable about the anatomy, assessment, and surgical and nursing management of infants with this anomaly and its impact on the patients' families. This article discusses the definitions, diagnosis, and treatment of craniosynostosis and support for parents of infants with this malformation.

Effectiveness and safety of independent pediatric nurse practitioners in evaluating plagiocephaly.

BACKGROUND: The American Academy of Pediatrics Back to Sleep program in 1992 dramatically increased the incidence of posterior plagiocephaly in infants. In 2000, the craniofacial disorders program at Oregon Health & Science University established a plagiocephaly screening clinic staffed by pediatric nurse practitioners. METHODS: Electronic medical records of patients seen in a single accredited craniofacial disorders clinic from 2005 to 2011 were reviewed retrospectively to identify patients screened independently by pediatric nurse practitioners for positional plagiocephaly versus synostosis. RESULTS: Over a 7-year period, 1228 patients were independently screened. Over half [638 patients (52 percent)] were followed through subsequent visits for craniofacial disorders without any direct surgeon input. Of these, 169 (26 percent) were treated with orthotic consultation for a molding helmet, 385 (60 percent) received a combination of counseling and/or physical therapy for torticollis, and 84 (13 percent) were lost to follow-up. No patient managed by pediatric nurse practitioners was identified to have cranial synostosis and no patient underwent early or delayed surgery. All returning patients [n=554 (86 percent)] showed improvement in, or resolution of, their presenting deformity. The remaining 590 patients (48 percent) were referred by the pediatric nurse practitioner for surgical consultation. Computed tomographic imaging revealed synostosis in 121 patients. CONCLUSIONS: Pediatric nurse practitioners accurately screened over half of patients referred to a high-volume craniofacial disorders program for positional plagiocephaly versus synostosis, without surgeon input. Based on available information, no synostosis diagnosis was overlooked using this approach. With specific training, pediatric nurse practitioners working in a craniofacial disorders program can safely and independently screen for positional versus synostotic plagiocephaly. CLINICAL QUESTION/LEVEL OF EVIDENCE: Diagnostic, III.

Recognizing craniosynostosis.

Craniosynostosis is characterized by the fusion of sutures. It presents with an abnormal head shape. This article examines this defect and discusses its embryologic origin. A systemic physical assessment guide serves as a tool to enhance early recognition of this defect. Pictorial examples increase understanding of the defect. A discussion of treatment and nursing implications, with an emphasis on family support, is provided.

Postoperative nursing care of the endoscopic nonsyndromic craniosynostosis surgery patient.

Craniosynostosis affects 1 in 2,100 children. The current methods for the treatment of craniosynostosis at Rady Children's Hospital, San Diego, are open cranial vault remodeling and the minimally invasive endoscopic technique. The primary advantages of the minimally invasive endoscopic technique are decreased operative time, blood loss, swelling, incision size, and inpatient hospital stay. Postoperative helmet therapy is a necessary part of the treatment for the refinement of the surgical result. The purpose of this article is to present current inpatient and outpatient nursing management considerations in the care of infants undergoing the minimally invasive endoscopic technique for the treatment of craniosynostosis.

Endoscopic strip craniectomy: a minimally invasive treatment for early correction of craniosynostosis.

Traditionally, surgical correction of craniosynostosis involves calvarial remodeling, large blood losses necessitating transfusions, hospital stays of several days, and less-than-satisfactory results. In this study, outcomes from a minimally invasive technique called endoscopic strip craniectomy, along with a postoperative molding helmet, to correct craniosynostosis in young infants were evaluated. The endoscopic strip craniectomy was performed on 185 patients with clinical signs of craniosynostosis, with the following distribution: 107 sagittal, 42 coronal, 37 metopic, and 7 lambdoid, for a total of 198 sutures. The mean blood loss was 29.4 cc, and only two patients underwent intraoperative blood transfusion. Fourteen patients underwent postoperative blood transfusion; none was life-threatening. There were no deaths, complications, neurological injuries, or infections. All but six patients were discharged on the first postoperative day. A majority of the patients achieved or approached normocephaly, and there were no complications. Neuroscience nurses need to be aware of this technique when they discuss treatment options with the families of infants with craniosynostosis.

Deciding on surgery: supporting parents of infants with craniosynostosis.

Families face a difficult decision when choosing whether or not to have surgery for their infant with isolated craniosynostosis. While the skull deformity may not affect the child's physical health, growth, or development, it may have psychosocial impacts on a child's social-emotional development, self-esteem, and interpersonal interactions. Parents are challenged to balance surgical risks with potentially positive effects on their child's psychosocial health. The purpose of this research study was to explore parental decision-making related to surgery for isolated craniosynostosis, and to identify strategies that facilitate their decision-making. A thematic content analysis of focus group interview data revealed four themes that encapsulated the process of parental decision-making. The decision for some parents was agonizing. However, others found the decision relatively straightforward. In all cases, parents spent time thinking and gathering information. Certain critical events helped parents make their decision. Parents identified a number of strategies that would be helpful to the decision-making process.

Calvarial vault remodeling for sagittal synostosis.

Sagittal synostosis is the most common form of craniosynostosis (i.e., premature fusion of cranial sutures). Sagittal synostosis, which is the premature fusion of the sagittal suture, occurs in 56% to 58% of all reported cases of craniosynostosis. This article describes calvarial vault remodeling, the procedure of choice at Children's Hospital Los Angeles for surgical intervention to correct sagittal synostosis. Perioperative interventions to minimize patients' risks and maximize benefits are explained. A case study is presented to describe the experience of one patient and his family members.

External cranial vault molding helmets.

Skull molding helmets can be used to correct abnormal skull shapes due to craniosynostosis. The helmets can be used alone, or in conjunction with surgery. The skull molding helmets are light-weight, inexpensive and custom-fitted. This article provides information regarding the history of these helmets and their construction, along with complications and the nurse's role in patient care.

Common pediatric craniofacial reconstructions.

The emphasis on beauty in our society also places demands on children to meet a certain appearance. With cleft lip and palate being one of the most common birth defects, it is likely that pediatric nurses or pediatric nurse practitioners will encounter children requiring a form of craniofacial surgery. This article discusses common reconstruction techniques and important features of care.

Craniosynostosis--corrective surgery for a cosmetic defect.

Premature fusion of the sutures of the skull is known as craniosynostosis, and is characterised by an abnormal skull shape. Early surgery allows the brain to resume its normal shape, encouraging a more natural pattern of development. Nursing care in a craniofacial unit aims to promote maximum physical recovery and improvement, while offering psychological support and understanding to the child and family.

Pediatric craniofacial reconstruction: an overview of perioperative management.

Pediatric craniofacial defects as a result of congenital malformation, tumor, or trauma are devastating to both the child and the family. These patients require extensive education, preparation, careful monitoring and psychological support. With continuing advances in surgical technology and technique, the effectiveness and utilization of pediatric craniofacial surgery is increasing. As this type of surgery is currently available at relatively few institutions, it has created a new nursing experience. A multi-disciplinary team approach has evolved at Texas Children's Hospital which necessitates active involvement of nursing staff. In this article the multi-disciplinary approach and nursing care utilized in the perioperative management of the craniofacial pediatric surgery patient will be discussed.