Challenging the Norm: Is Routine Use of Cranial CT in Evaluation of Craniosynostosis Necessary?

BACKGROUND: Cranial CT is routinely taught to be the gold standard for diagnosis of craniosynostosis and used by craniofacial teams for suspected nonsyndromic single suture craniosynostosis. Given the risks associated with infant CTs, do these scans provide significantly enhanced diagnostic accuracy compared to the physical exam when performed by an experienced clinical provider? METHOD: A retrospective chart review was performed for children who underwent corrective surgery for nonsyndromic, single-suture craniosynostosis over an 11 year period by a single craniofacial team. Ages at presentation and surgery, preoperative clinical diagnosis and imaging, co-existing radiographic findings, and correlation with the intraoperative diagnosis were analyzed. RESULTS: A total of 138 patients were included in this study. The mean age was 4.2 months at initial craniofacial evaluation, and 8.0 months at time of surgery. Twenty-seven patients received imaging prior to our clinic. Of those, 21 had plain radiography and 6 had CT scans. Of the remaining 111 patients referred without imaging, craniosynostosis was clinically diagnosed in 102 (92%), whereas 9 (8%) had an unclear clinical diagnosis. Of these 9, 1 (1%) was diagnosed clinically at follow-up exam, and the remaining 8 (7%) were diagnosed using radiography (3 CT scans, 5 plain radiographs). In all patients, the preoperative diagnosis was confirmed during intraoperative assessment. CONCLUSIONS: Cranial CT was not needed by experienced craniofacial providers in 93% of nonsyndromic, single-suture craniosynostosis. Imaging obtained before craniofacial clinic referral may have been unnecessary. These findings question the classic teaching that preoperative cranial CT is the gold standard for diagnosis in infants with nonsyndromic, single-suture craniosynostosis.

My Introduction to Mission Surgery: A Diary.

I experienced my first international surgical mission trip in 1993. It was a turning point in my surgical career and has been followed by many missions in many countries over 25 years. Often I am asked by young surgeons what is it like to work on an international mission and what one should expect. Although each mission is different, the sense of accomplishment is always the same and the emotional high one gets from performing the work is always present. Different organizations have different team models. The description here is based on my first mission with Operation Smile, a global nonprofit medical service organization.

2020 guidelines for conducting plastic reconstructive short-term surgical projects in low-middle income countries.

Many low- or middle-income countries (LMICs) continue to suffer from a lack of safe and timely essential and emergency surgery despite growing attention to this problem. Short-term surgical projects (STSPs) continue to play an important role in addressing LMIC unmet surgical need and strengthening local healthcare systems. Guidelines here present recommendations for performing plastic reconstructive STSPs for pediatric patients in a safe, ethical, and effective manner. These guidelines represent consensus physician expert opinions, assembled collaboratively by members of Volunteers in Plastic Surgery and the Society for Pediatric Anesthesia's global health committee, with broad input from physicians practicing daily in LMICs. Organizations must partner with hosts to thoughtfully plan and carefully execute STSPs. We outline crucial items to STSP success, including choice of host facility, team selection, patient selection, staffing, ensuring proper equipment and supplies, disinfecting reusable equipment, creation of a safety culture, and data collection for quality assessment/improvement and research. Patient factors are discussed and recommendations given for developing exclusion criteria, as well as for determining which patients and procedures may require the team to include expertise in pediatric anesthesia or critical care. We recommend that educational opportunities for hosts are sought and advanced to optimize education/training at both the resident and post-trainee levels. Host education during STSPs has become crucial as LMICs ramp up training at a time when their surgical volumes remain grossly behind well-resourced countries. Recommendations here aim to assist organizations, hosts, and volunteers as they navigate the enormously complex and ever changing STSP environment. Patient safety and transfer of knowledge and skills should be central concerns of all who participate in this highly rewarding endeavor.

The Venetian blind technique: modification of the Pi procedure for the surgical correction of sagittal synostosis.

Numerous methods of surgical repair for scaphocephaly (sagittal synostosis) have been reported in the literature, from strip craniectomies to more complex methods of calvarial vault remodeling. While good cosmesis and restoration of a normal anteroposterior diameter may be obtained with these methods, a more rounded contour of the biparietal areas is often more difficult to achieve. We describe a modification of the Pi technique, described by Jane in 1976, that results in a more rounded contour of the biparietal areas. We report our experience on cranial vault remodeling for the correction of scaphocephaly in 51 patients from 1998 to 2011.

Volunteers in plastic surgery guidelines for providing surgical care for children in the less developed world.

BACKGROUND: A significant need is met by volunteer groups who provide free reconstructive plastic surgery for underserved children in developing countries. However, at present there are no consistent guidelines for volunteer groups in plastic surgery seeking to provide high-quality and safe care. METHODS: With these quality and safety standards in mind, in 2006, the Volunteers in Plastic Surgery Committee of the American Society of Plastic Surgeons/Plastic Surgery Educational Foundation undertook a project to develop a detailed set of guidelines for volunteer groups from developed countries seeking to provide plastic surgery services to children in developing countries. To make the guidelines include both surgical and anesthetic needs, they were developed in conjunction with the Society for Pediatric Anesthesia. RESULTS: Guidelines for the delivery of plastic surgery care by volunteer groups to developing countries have been reviewed and approved by the boards of both organizations (the American Society of Plastic Surgeons/Plastic Surgery Educational Foundation and the Society for Pediatric Anesthesia). These include guidelines for the initial site visit, site and patient selection, staff and equipment that should be available, and procedures that can be safely performed based on the site and available facilities. Guidelines for assessment of outcomes, dealing with adverse outcomes, and quality improvement are also provided. CONCLUSIONS: Any plastic surgery group undertaking an international mission trip should be able to go to one source to find a detailed discussion of the perceived needs in providing high-quality, safe care for children. The present document was created to satisfy this need.

Pfeiffer syndrome twins: despite improved correction in one twin, growth disturbance results in similar need for subsequent monobloc advancement.

BACKGROUND: Twins with Pfeiffer syndrome (or acrocephalosyndactyly) had a similar phenotypic appearance with findings of classic or type 1 Pfeiffer syndrome, including bicoronal and sagittal craniosynostoses, midface hypoplasia, and broad thumbs/toes. We document their treatment with 2 monobloc advancements and discuss growth disturbances in craniofacial dysostosis. METHODS: At 6 months, both twins underwent release of multisuture (bilateral coronal and sagittal) fusions for improvement of scaphocephalic shape and multisuture release; however, one twin had a more aggressive procedure with advancement of the frontal-orbital region. Despite improved initial correction by one twin, at 5 years of age, both twins presented with midface hypoplasia and exorbitism and underwent a monobloc distraction procedure with similar 20-mm advancements. Comparative analysis by our craniofacial multidisciplinary team included perioperative reports, computed tomographic scans, cephalograms, parent questionnaires, and physician surveys. RESULTS: Both twins had an improved confidence interval scores from 84 to 68 and 82 to 69 postoperatively. In 6-month follow-up, the Whitaker score of the first twin was 2.8, whereas that for the second twin with the frontal-orbital advancement was 1.2. Preschool expressive and receptive tests yielded 97 and 95, and 97 and 98, and developmental testing was similar between the twins. Global evaluations were equivalent to age-matched controls, and memory and attention skills were within normal limits. Parental surveys showed a high level of satisfaction after all procedures in both twins. CONCLUSIONS: Our study demonstrates that the phenotypic outcome for both twins remained unchanged when comparing a more aggressive surgery to less aggressive surgery as an infant. The genetic mutation may have overridden the different surgical interventions. Both twins ultimately required 2 subsequent monobloc corrections.

Correction of frontonasoethmoidal encephalocele: the HULA procedure.

BACKGROUND: The frontonasoethmoidal encephalomeningocele deformity involves central herniation of a glial mass that "pushes outward" and deforms the medial orbit, medial canthus, nasomaxillary process, and nasal structures without resulting in hypertelorbitism. The authors studied a modification of the "Chula" repair, called the HULA procedure (H = hard-tissue sealant, U = undermine and excise encephalocele, L = lower supraorbital bar, A = augment nasal dorsum), which provided complete correction of the midline hard and soft-tissue structures using an intracranial and extracranial approach. METHODS: Filipino patients with frontonasoethmoidal encephalomeningoceles were treated by a civilian/military humanitarian team at Tripler Army Hospital (n = 12). Operative technique followed the four steps of the HULA frontoethmoidal encephalocele procedure. Postoperative and follow-up assessments were based on examination, photographic images, computed tomography scans, parental surveys, Whitaker score, and developmental testing. RESULTS: Patient ages ranged from 5 to 12 years; 67 percent were female and 33 percent male. Sixty-seven percent required excisions of poor-quality, hyperpigmented skin along with the large glial mass; the other 33 percent had a "closed" resection of the smaller mass through a gingivobuccal sulcus incision. No patients manifested cerebrospinal fluid leaks, infection, or elevated intracranial pressures postoperatively. Skeletal correction showed improved medial orbit distance, with a mean correction of 14 mm (42 percent). Whitaker score was 1.3 (no or minor soft-tissue revision necessary). Parental survey showed a high degree of satisfaction with the aesthetic and functional outcomes. Follow-up developmental tests showed normal global evaluations for all but one child with normal memory and attention skills. CONCLUSION: The authors' outcomes demonstrated that the HULA technique was a safe and effective approach for the complete correction of frontonasoethmoidal encephalomeningoceles.

Posttraumatic facial restoration: a philosophy of care.

Posttraumatic facial restoration requires the marriage of both aesthetic and reconstructive principles. An outline for the treatment of traumatic facial injuries is presented.

Sagittal synostotic twins: reverse pi procedure for scaphocephaly correction gives superior result compared to endoscopic repair followed by helmet therapy.

A unique situation of twins with similar sagittal synostosis pathology who underwent different surgical corrective procedures allowed us an opportunity to compare an endoscopic technique to the more traditional technique of a modified cranial vault remodeling (CVR). At 4 months of age, 1 twin underwent an endoscopic-assisted extended strip craniectomy with postoperative helmet therapy for 12 months, and the other underwent a reverse pi CVR procedure. Cephalic index, the Whitaker Aesthetic score, and developmental tests were used for comparison during a 6-year follow-up. The modified CVR had a superior outcome compared to the endoscopic procedure with regard to cephalic index measurements (CVR = 77; endoscopic = 63; P < 0.05) and the Whitaker score (CVR = 1 or no revisions necessary; endoscopic = 2.3 or minor to major bony work required for correction). Patients had similar intelligence quotient test scores; however, the endoscopic patient had lower scores in both preschool tests and global evaluations compared with the normal scores of the CVR patient. The modified CVR procedure was superior to the endoscopic procedure, at least in these twins with sagittal synostosis.

Unilateral vision impairment from a carotid-cavernous fistula after a monobloc osteotomy in a patient with Apert syndrome.

A patient with Apert syndrome who underwent a monobloc osteotomy with distraction advancement sustained partial unilateral vision loss as a complication from a direct carotid-cavernous fistula. Successful embolization was used to treat the fistula. Precaution should be taken by craniofacial surgeons in performing similar procedures in patients with Apert syndrome because of their unique skull abnormalities.