Faculty Bibliography

Background & Purpose: Traditional neurosurgical access to tumors or vascular anomalies of the anterior cranial fossa and/or ethmoid sinus requires coronal incision and extensive frontal dissection. Here we detail a limited supra-brow approach, focusing on operative technique, anatomic exposure, and clinical considerations. Methods & Description: Operative Technique: After epineph-rine infiltration, a supra-brow incision is made. Intermuscular dissection separates preorbital orbicularis oculi from inferior frontalis. Frontal periosteum is identified and supraperiosteal exposure is obtained from glabella medially to deep temporalis fascia laterally. The periosteum surrounding the supraorbital nerve is incised and the nerve is reflected inferiorly with periorbita (making an osteotomy for true foramina). Next, a medially based pericranial flap is raised, exposing frontal bone for mini-craniotomy; this flap is kept protected beneath the medial frontalis muscle. After neurosurgical intervention and dural repair, cranial bone is rigidly restored. Overlying soft tissue is closed in layers. Anatomic Exposure: Before craniotomy, various maneuvers provide additional exposure. Subperiosteal dissection within the supero-medial orbit permits supraorbital craniotomy and access to the ethmoid sinus. Elevating anterior temporalis permits more lateral craniotomy and access to neurosurgical targets within the lateral anterior cranial fossa. Clinical Considerations: To prevent injury to the fronto-temporal branch of the facial nerve, dissection over the frontal bone is supraperiosteal and dissection over temporalis is just above deep muscle fascia. When the craniotomy includes lateral frontal sinus, mucosa is burred off the removed bone and in situ sinus; the nasofrontal outflow tract is obliterated with the pericranial flap and sealed with fibrin glue. The preserved pericranial flap can also be used to restore dural integrity. When bone is deficient, the removed cranium can be split for additional graft. Results: We used the supra-brow approach in 14 patients to provide sufficient access for definitive neurosurgical management of an anterior clinoid meningioma, three lateral frontal lobe meningioma, nine aneurysms of the anterior communicating artery, and an intra-ethmoidal arterio-venous malformation. Blood loss during exposure was minimal in all cases. There was no injury to the ophthalmic division of trigeminal nerve or frontal branch of facial nerve. Split calvarial grafts were used in nine of fourteen patients. At one year follow-up, all patients had excellent frontal contour, bony union, and an aesthetic scar. Conclusions: A supra-brow approach limits extensive dissection and permits sufficient neurosurgical exposure to tumors and vascular anomalies of the entire anterior cranial fossa and ethmoid sinus

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

Agnathia-otocephaly complex (AOC) is characterized by mandibular hypo- or aplasia, ear abnormalities, microstomia, and microglossia. Though rare and often fatal, this is the first report detailing various reconstructive strategies beyond infancy as well as longitudinal follow-up into adulthood.All patients with AOC treated at our institution over a 30 year period were reviewed. Four patients were identified, one with agnathia, one with micrognathia. Two males with nanognathia (defined as a symphyseal remnant without body nor ramus) were also included. The mean follow-up was 17 years. All four underwent perinatal tracheostomy and gastrostomy-tube placement. Commissuroplasties were typically performed before 3 years of age and repeated as necessary to allow for oral hygiene. Mandibular reconstruction was most successful with rib between ages 3 and 8, after which time, free fibula transfer was utilized. Due to some resoprtion or extrusion, all patients underwent repeated bone grafting procedures. Tissue expansion of the neck was used to restore the lower third of the face, but was most successful in the teenage years. At last follow-up of the eldest patients, one was in college while another was pursuing graduate education.AOC need not be a fatal nor untreatable condition; a reasonable quality of life can be achieved. Although the lower-facial contour may be improved, and a stoma created, the lack of musculature make deglutition virtually impossible with current therapies. Just as transplantation has emerged as a modality for facial restoration following severe trauma, so too may it be a future option for congenital deformities.

BACKGROUND: Given the great variability in perioperative management of craniosynostosis, a large-scale national survey of current practice patterns was conducted. METHODS: 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. RESULTS: Fifty-three surgeons (46%) completed the survey. All respondents complete repair before 1 year of age with a majority operating between 4 and 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 with their less experienced colleagues. More than two-thirds of surgeons (68.8%) obtain preoperative imaging for every case; 83% of these prefer computed tomography scans. More than one-fourth of respondents (28%) routinely prescribe an extended course (>24 hours) of antibiotics. Overall transfusion rates remain high, with nearly 2 (65.2%) in 3 transfusing in 76% to 100% of operations. The overwhelming majority of respondents (93.6%) routinely send patients to an intensive care unit postoperatively. CONCLUSIONS: We present the largest US survey of craniosynostosis surgical practice patterns to date. General consensus exists regarding safety and emergency preparedness standards. In addition, we identified several patterns that deviate from published evidence-based guidelines. 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.

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 .

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.