The Role of the Nurse in Endoscopic Craniosynostosis Surgery

Introduction

The role of the nurse in endoscopic craniosynostosis surgery began in 1996, after the first surgery was performed at the University of Missouri-Columbia. The role involved not only patient care and family communication, but also coordinating a new type of program. Many of the lessons learned during the early years of program development are still applicable today. The role of the Advanced Practice Registered Nurse (APRN) is particularly suited for care coordination, pre- and postoperative teaching, follow-up care, nursing staff education, data collection, and research. The nurse should be knowledgeable about infant skull anatomy and the physiology of skull bone and brain growth, as it is essential in explaining the minimally invasive endoscopic treatment of craniosynostosis. Prompt, accurate communication with the parents is essential to decrease their anxiety. Parents report that step-by-step information about what to expect from diagnosis to discharge and follow-up is extremely helpful. Perhaps most important, listening and addressing parents’ concerns, answering their questions, and supporting their decisions provide the greatest support to the parents at such a difficult time for them.

Making the Diagnosis

Craniosynostosis occurs in approximately 1 out of 2500 live births. As the brain grows rapidly during the first year of life, the skull bones are normally pushed apart and new bone is formed perpendicular at the suture sites. Genetic mutations cannot be found in 85% of the infants with craniosynostosis and those are called “nonsyndromic.” A stenosed (closed) suture prevents skull growth (perpendicular) to the suture and results in the deformities seen in an infant with craniosynostosis. Parents may notice some molding of the skull at birth due to childbirth, but the shape usually normalizes by 3 months of age or sooner. Although the diagnosis of craniosynostosis is usually made by physical examination, it is often apparent in photographs furnished by parents. Skull radiographs or a computed axial tomography (CT) scan can be ordered to confirm a questionable diagnosis. Further imaging, such as a magnetic resonance imaging (MRI), can be done if there is a concern for hydrocephalus or other brain anomalies. Care must be taken when considering radiodiagnostic imaging as ionizing radiation can have a negative effect on a growing child’s brain. The Joint Commission (2011) recommends using ALARA guidelines, meaning that the dose should be “as low as reasonably achievable” to minimize exposure to ionizing radiation.

Parents frequently recognize there is something wrong with their baby’s head at birth, or within a few weeks afterward. It is extremely important to listen to parents’ concerns about their infant’s head shape so the diagnosis can be made early enough that they can have a choice of treatment options. Some parents have reported they noticed that the eyes were not leveled, or the nose was twisted, or they felt a ridge on top of their baby’s head, but their concerns were dismissed by their healthcare provider. Unfortunately, not all healthcare providers are trained to recognize craniosynostosis. Oftentimes providers adopt the “watch and wait” attitude which can delay the diagnosis of craniosynostosis past 6 months of age, thereby practically eliminating the window of opportunity for the minimally invasive option of endoscopic surgery. This can present a dilemma for parents who suspect that there is something wrong with their infant’s head soon after birth. In reviewing the many craniosynostosis websites and parental blogs, the anger and frustration of parents whose concerns were put off until their baby was too old for the minimally invasive endoscopy-assisted procedure can be seen. Some parents express feelings of guilt for not insisting on a referral to a craniofacial center earlier. Best results are obtained when the endoscopic surgery is performed by age 3 months and typically no later than 6 months, followed by strict use of a custom-made cranial molding helmet. This emphasizes the need for early diagnosis. Parents must also be made aware of the availability of the endoscopic alternative, so the surgery can be done before the infant becomes too old for it to be effective. Although the authors of this book are proponents of the endoscopic technique, some neurosurgeons and plastic surgeons advocate the open procedures, and there are centers where the minimally invasive technique is not performed. If done correctly and with appropriate follow-up, both types of surgery (endoscopic and open) will produce good results. There are many advantages, however, to the endoscopic technique, which have been discussed throughout this book. Hence, parents must be given accurate information so they can decide what is the best treatment for their child.

Although nonsyndromic craniosynostosis typically involves only one suture and is not associated with other anomalies seen with syndromic craniosynostosis, the nurse should be aware that receiving the diagnosis of craniosynostosis can have a profound effect on families. Being told your infant needs surgery is frightening for parents in general, but surgery on the skull or face seems to evoke a particular level of anxiety and fear. Parents are faced with making the choice of surgery to change their child’s facial appearance or choosing no surgery with questionable risks of damage to neurocognitive development. It is important to listen to their concerns and provide them with evidence-based information, so they can make an informed decision. Many parents struggle with whether to submit their infant to surgery for “cosmetic” reasons, stating they would love their child no matter how he or she looked. However, there can be psychological and physical implications if nothing is done. Children who look different than their peers are often teased or bullied, which can negatively affect their self-esteem. Hats and sports helmets may not fit appropriately due to the abnormal shape, further adding to the feeling of being different from their peers.

Parents may have concerns about cognitive development as well as cosmesis, as some studies have suggested there is cognitive impairment and neurodevelopmental delay related to single suture craniosynostosis. Many centers recommend neurodevelopmental screening for patients with single suture craniosynostosis. In a qualitative study of parents of infants with single suture, nonsyndromic craniosynostosis, Kuta found that parents appreciated the support of healthcare professionals, especially having access to a specialized nurse to ask questions of during the process. Talking with other families who have been through a similar experience was found to be particularly valuable. Information from the craniofacial team is helpful, but parents often want to hear the perspective of others who have been through the entire process. Most parents of infants who have undergone endoscopic craniosynostosis surgery are very willing to share their experience with other families. Obtain written permission according to hospital policy when connecting families whose children have the same types of craniosynostosis.

Websites

Websites can be an important tool in educating parents and other healthcare providers. There are numerous websites that reference the endoscopic-assisted procedure, including those sponsored by hospitals, surgeons, or parents. Unfortunately, some of them contain inaccurate or misleading information, and may even claim that the procedure does not work, or that patients will have to undergo further reconstructive surgery in the future. The nurse should be prepared to answer questions arising from such misinformation. Reviewing the website of the craniofacial team planning to do the surgery yields the most accurate information on outcomes from that particular center. Parents may select a center based largely on its website, so the information should be regularly updated.

The APRN should be a content expert for the craniosynostosis website and collaborate with the institution’s website designer or communication/marketing department. It is essential the website accurately describes the various types of craniosynostosis and treatment options. Pre- and postoperative photographs of each type of synostosis help parents understand the anomaly and outcomes from surgery. Parent testimonials can provide relatable information for parents considering craniosynostosis surgery. Emphasis must be placed on the importance of seeing the infant in craniosynostosis clinic as soon as possible to give parents the option of the endoscopic surgery. For the purpose of consistency, it is generally preferable for the APRN who coordinates the program to respond to parent inquiries. Including a contact number or email address on the website and responding promptly to queries is extremely gratifying to anxious parents seeking information about surgical options.