Faculty Bibliography

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

Construction of the congenitally absent ear is a particular challenge. It represents the true essence of plastic surgery in that it not only requires sound surgical principles but artistic skill. Although a solid familiarity with the stages is important, the surgeon's own experience will direct gradual modification

BACKGROUND: We present a case of partial facial duplication in a male infant. METHODS: The clinical, radiological, and laboratory findings for this patient are described, followed by a review of the literature. RESULTS: Craniofacial duplication is a rare form of conjoined twinning and presents in a wide spectrum, from dicephalus to diprosopus to partial facial duplication. Many of these cases can be diagnosed prenatally. Prenatal assessment of our patient revealed only agenesis of the corpus callosum. CONCLUSIONS: The pathogenesis is believed to involve duplication of the notochord. Where there are more severe associated anomalies, the prognosis is poor. Partial facial duplication, as in our case, is associated with fewer anomalies, and the prognosis is better. Symmetry and an excess of tissue, rather than deficiency, favor a positive result