Faculty Bibliography

Background & Purpose: Given the great variability in perioperative management of craniosynostosis, a large-scale national survey of current practice patterns was conducted. Methods & Description: Using scaphocephaly as a test diagnosis, 115 craniofacial surgeons at all levels of career experience across the United States were invited to participate in an anonymous survey. Surgeons were asked about practices related to pre-operative evaluation and planning, intraoperative monitoring, operative team composition, and post-operative care. Results: Fifty-three surgeons (46%) completed the survey. The overwhelming majority of craniofacial surgeons work with pediatric neurosurgeons (100%), fellowship-trained pediatric anesthesiologists (95.8%), and use arterial lines (95.8%) and urinary catheters (97.9%). All respondents complete repair before 1 year of age with a majority operating between 4-8 months. Surgeons with greater than 10 years of experience were significantly more likely to perform open repair at extremes of age (<4 months and 8-12 months) (p=0.03) and reported shorter operative times (p=0.01) compared to their less experienced colleagues. More than two-thirds of surgeons (68.8%) obtain pre-operative imaging for every case; 83% of these prefer CT scans. Over a fourth of respondents (28%) routinely prescribe an extended course (>24 hours) of antibiotics. Overall transfusion rates remain high, with nearly two in three (65.2%) transfusing in 76-100% of operations. The overwhelming majority of respondents (93.6%) routinely send patients to an intensive care unit (ICU) post-operatively. Conclusions: We present the largest United States survey of craniosynostosis surgical practice patterns to date. General consensus exists regarding safety and emergency preparedness standards. Craniosynostosis repair remains a high-risk operation that can be performed safely. Additionally we identified several patterns that deviate from published evidence-based guidelines and impact on patient care and healthcare expenditures. Specifically, these practices relate to the routine use of high-dose radiation imaging, long-term antibiotics, blood transfusions, and intensive postoperative surveillance. For the first time, stratifying by surgeon experience revealed significant differences in clinical practice

Background/Purpose: Pediatric cleft lip and palate surgery can be stressful for both the child and the parents. Limited pain knowledge and certain parent psychological traits are associated with increased parental anxiety around surgery in older children. Increased parental anxiety has been associated with increased child pain, decreased ability of the child to cope with pain and worse outcomes in other surgical settings. Little is known about parental anxiety and child pain in preverbal children undergoing cleft lip and palate repair. The objectives of this study were to explore possible sociodemographic factors contributing to parental anxiety in the immediate postoperative period and to determine if there is a relationship between parental postoperative anxiety and infant postoperative pain. Methods/Description: Cross-sectional pilot study, semi-structured interview. Eight mothers of children under 18 months of age undergoing cleft lip/palate (CL/P) repair at an urban craniofacial center were recruited. Semi-structured interviews about their experience with their infant's surgery were conducted. Demographics were collected at a preoperative visit, while maternal anxiety scores, measured using the Hospital Anxiety and Depression Scale (HADS), and nurse-recorded child pain scores (Face, Legs, Activity, Cry, Consolability scale), were collected on postoperative day (POD) 1. Fisher's exact tests were used to compare demographics and Student's t-tests were used to analyze pain medication and doses given. Results: Mothers who were healthcare workers were more likely to have borderline/abnormal anxiety scores (HADS > 7) than mothers who were non-healthcare workers (p = .035) on POD1. Mothers of infants undergoing a bilateral CL/P repair tended to be more anxious than mothers of infants undergoing a unilateral CL/P repair (p=.090). Infants of anxious mothers tended to have more variation in pain scores, more pain scores recorded (95% CI -1.74, 4.0) (p = .19) and more pain medication given (95% CI 2.!

We report a case of lipoblastoma of the hand in a 19-month-old female patient with a history of cleft palate. The incidence of lipoblastoma and cleft palate individually is extremely rare. To the best of our knowledge, only 1 other case of a patient with both cleft palate and lipoblastoma exists in the literature. Lipoblastoma is a rare benign neoplasm in adipose tissue almost exclusively found in children younger than 3 years. Cytogenetic testing has shown that lipoblastomas characteristically share a clonal chromosomal rearrangement affecting the long arm of chromosome 8. Furthermore, recent research has shown that the 8q chromosome is an important genetic risk factor for cleft palate development. We describe the second case linking cleft palate with this rare tumor and provide evidence for a potential genetic association.

A subset of hydrocephalic patients in whom shunts are placed at an early age will develop craniocerebral disproportion (CCD), an iatrogenic mismatch between the fixed intracranial volume and the growing brain. The lack of a reliable, reproducible method to diagnose this condition, however, has hampered attempts to treat it appropriately. For those practitioners who acknowledge the need to create more intracranial space in these patients, the lack of agreed-upon therapeutic end points for cranial vault expansion has limited the use of such techniques and has sometimes led to problems of underexpansion. Here, the authors present a definition of CCD based primarily on the temporal correlation of plateau waves on intracranial pressure (ICP) monitoring and headache exacerbation. The authors describe a technique of exploiting continued ICP monitoring during progressive cranial expansion in which the goal of distraction is the cessation of plateau waves. Previously encountered problems of underexpansion may be mitigated through the simultaneous use of ICP monitors and gradual cranial expansion over time.

BACKGROUND: Microtia reconstruction remains one of the most challenging procedures encountered by the reconstructive surgeon. A national report on the current management of microtia has never been presented before. The purpose of this project was to survey members of the American Society of Plastic Surgeons (ASPS) to identify their preferences and practices and report their opinions regarding issues related to microtia reconstruction. METHODS: An anonymous web-based survey consisting of 19 questions was distributed to the members of the ASPS. Questions focused on the management of microtia. The study design was descriptive, using categorical data analysis. RESULTS: Thirty-eight percent of all respondents perform microtia reconstruction; 91 % learned the autogenous cartilage-based reconstruction technique, while only 16 % were exposed to alloplastic reconstruction. Seventy percent of all respondents learned autogenous cartilage-based ear reconstruction exclusively. Fifty percent of respondents who perform microtia reconstruction reported a steep learning curve. In the pediatric patient population, 49 % of microtia surgeons prefer performing the surgery when the patient is between 7 and 10 years of age, while 40 % of microtia surgeons prefer the patient to be 4-6 years of age. Fifty-nine percent of all respondents believe that in 15 years tissue engineering will represent the gold standard of microtia reconstruction. CONCLUSION: Staged microtia repair using autogenous cartilage remains the heavily favored method of microtia reconstruction among plastic surgeons. Moreover, there is a deficiency in training the newer surgical techniques, such as alloplastic and osseointegrated options. This study also highlights the continuing need to elucidate the optimal timing for microtia repair in the pediatric patient to mitigate the potential psychosocial morbidity well described in the literature. LEVEL OF EVIDENCE V: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

ABSTRACT: The separation of craniopagus conjoined twins is a very rare and complex challenge. As with many rare challenges, it presents initially as a deceptively simple problem requiring only the most basic clinical techniques. As in many reconstructive problems, this paradigm mandates that the neurosurgical team performs the separation with the plastic surgeons providing closure at the end of the separation. Historically, these approaches have included, as with the separation of many other types of conjoined twins, the use of tissue expansion before separation followed by separation surgery. In the best hands, at the most capable medical centers, the mortality reported in the literature for the past 50 years is greater than 50%. Craniofacial surgery frequently demands a coordinated effort between plastic surgery and neurosurgery and many other specializations; separating craniopagus twins takes this coordination to a stratospheric level. It is, however, this coordination that is of paramount importance. Success clearly requires an understanding of the complex interrelationship between the "separation" and the "reconstruction" and that decisions made for 1 aspect of the surgery will have a profound impact on another aspect of the surgery. The impact can be disastrous or, if planned well, can be advantageous.We were contacted to evaluate craniopagus conjoined male infant twins for separation. Radiographic studies suggested that the brains were separate, and their medical team suggested that they were "fit for separation." We reviewed the literature and reviewed our colleagues' experiences with similar cases around the world. It became clear that whether separation had been unsuccessful or successful, a variety of issues accompanied surgery as follows: (1) massive intraoperative hemorrhage, (2) cerebral edema, (3) venous infarcts, (4) swelling of flaps, and (5) dehiscence of repairs with cerebrospinal fluid (CSF) leak, meningitis, or brain exposure. Although the initial plan was to separate the twins in the same fashion as in previous cases (ie, single-stage separation surgery preceded by tissue expansion of the scalp), it was clear that this approach increases cerebral venous pressure during the separation component of surgery and therefore set up a cascade of events favoring failure rather than success. Wishing to favor success, we elected to design an open-ended multistaged separation to improve venous collateral circulation. We believe that this would improve venous drainage, prevent increased venous pressure, diminish cerebral edema, and favor the integrity of the dura and flap repair that would in turn lessen the risk of CSF leak. The stages would also allow the twins to recover from each stage before progressing to the next stage while continuing to receive nutritional support and physical therapy. Four major stages for 9 (1/2) months led to their successful separation. There has been no CSF leak or meningitis. To our knowledge, this technique has since been applied to 2 other sets of craniopagus with similar outcomes.A review of the pertinent literature, our rationale, and methodology are discussed in this article.

INTRODUCTION: Beginning in 2004, we modified our surgical technique for a cranial vault remodeling in sagittal and lambdoid synostosis. Beginning in the early 1990s, we started using a calvarial vault remodeling technique in sagittal and lambdoid synostosis that involves removing the posterior two thirds of the skull, extending from the coronal suture to below the lambdoid suture to within 1-1.5 cm of the foramen magnum. Up until 2004, the bone fixation evolved from wire fixation, then micro-metallic fixation plates and resorbable sutures. DISCUSSION: Over the last 9 years, we have used a novel technique of absorbable fixation plates and a polydioxanone suture trellis or lattice network, which has reduced operating times significantly and continued to give excellent results. Additional advantages include the absence of a need for molding or protective helmets, the absence of bony defects at the completion of the procedure, the absence of age limitation, and the ability to correct the tightly constricted occiput. CONCLUSION: To date, we have had no significant complications, no return to operating room, and the aesthetics have held up well since its introduction.