Faculty Bibliography

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.

We present a case of successful separation of craniopagus conjoined twins. The procedure was staged to permit each child to develop adequate independent cerebral venous drainage and to prevent deleterious, perioperative cerebral edema. Surgical hemorrhage, blood product delivery, and hemodilution were minimized.

Advances in medicine have enabled more craniopagus twins to survive separation, but the ultimate goal, to have both twins emerge from their separation with full neurologic function and a chance at leading independent and productive lives, remains elusive. The authors were contacted to evaluate craniopagus conjoined male infant twins for separation. Once it was determined that the brains were separate, they reviewed the literature and elected to design an open-ended multistaged separation. Four major stages over 9 and a half months led to the successful separation and preservation of neurologic function. To the authors' knowledge, this is the first such outcome in such a case. This article reviews the pertinent literature and discusses the authors' rationale and methodology

CASE REPORT: We present the case of 2-year-old craniopagus twins who are in the process of undergoing a staged separation of their craniums. In addition, they will undergo a full calvarial vault remodeling to reconstruct the lacking skull and scalp tissue in each child. We elected to do a staged separation rather than a single marathon operation for various reasons, which we will detail in this report. DISCUSSION: The philosophy and surgical management of each step will be discussed along with the pros and cons behind the reasoning that was used. At the time of this report the children have undergone three of what are projected to be four stages of separation. Each of the stages has had unique characteristics and surgical judgments involved, and those will be reviewed. CONCLUSION: Successful separation of viable conjoined twins has been historically a great rarity. Successful separation of twins where both have come out of surgery without any neurological deficit remains a rare occurrence and in our mind the ultimate goal