Binary nature and radiographic identifiability of craniosynostosis.

RATIONALE AND OBJECTIVES: Two independent gold standards and diagnoses from three-dimensional computed tomography (CT) images were used to examine the possibility that craniosynostosis is a binary abnormality that potentially may be diagnosed without error. METHODS: Surgical reports, histology of excised sutures, and three-dimensional CT images were compared for 25 children undergoing surgical management of craniosynostosis. Surgical reports identified sutures as normal or abnormal. Histology reported suture closure on a 5-point scale. Four radiologists used three-dimensional CT images to diagnose sutures on a 6-point rated response scale. RESULTS: Sutures with histology 0, 1, or 2 were normal on surgical reports, and those with histology 3 or 4 were abnormal. Most readers achieved nearly perfect sensitivity and specificity. Reader confidence was unrelated to degree of pathology. CONCLUSION: Craniosynostosis appears to be binary in our sample. Surgical reports, pathology results, and three-dimensional CT images read by experienced viewers achieved nearly perfect agreement.

Craniosynostosis: diagnostic imaging with three-dimensional CT presentation.

PURPOSE: To measure diagnostic performance and preference of two three-dimensional CT reconstruction modalities (voxel-gradient and surface-projection) displayed two ways (conventional and unwrapped) in craniosynostosis confirmed by surgical inspection and histologic analysis of resected sutures. METHODS: High-resolution 2-mm contiguous CT sections were obtained and three-dimensional reconstruction images generated for 25 infants and children with skull deformities before surgical treatment of craniosynostosis. Two pediatric radiologists and two neuroradiologists first ranked images by their own preferences for diagnostic use. Then they diagnosed craniosynostosis from images presented in random order and blinded. The standard of reference was inspection during surgery and histologic evaluation of excised sutures. Finally, reviewers repeated their subjective preference tests. RESULTS: The least experienced radiologist had 100% sensitivity for all imaging modalities and specificities ranging from 43% to 83%. The two most experienced radiologists performed nearly identically, with sensitivities of 96% and specificities of 100%. After performing diagnostic tasks using all image types, all radiologists preferred conventional surface projections. CONCLUSION: Experienced readers can achieve nearly perfect diagnostic performance using the latest three-dimensional CT reconstruction images, making it a contribution to the diagnostic process. Although performance is nearly identical for all modalities, readers strongly prefer conventionally presented surface-projection images.

Microsurgery for macrodefects: microvascular free-tissue transfer for massive defects of the head and neck.

Despite defect location and the fear of creating complex massive defects, coverage of large areas of soft tissue loss, with or without exposed calvaria, dura, and brain, can be reconstructed reliably with microvascular free tissue transfer. This technique permits separation of the oronasopharynyx from the intracranial contents, coverage of dural grafts, restoration of composite tissue loss, and achievement of superior aesthetic results in a single stage. When choosing vascular anastomotic sites, free-flap transfer permits a greater latitude in flap orientation, tailoring, and inset than is possible when using fixed pedicled rotation flaps. When confronted with a deeply invasive or gigantic malignancy, a multidisciplinary team approach is optimal. The risk associated with sophisticated ablative and reconstructive operative procedures is justified when dealing with potentially curable lesions. The success of these extensive procedures is related not only to the functional result and the aesthetic appearance, but most importantly to the resultant quality of life they allow.

A new method for microvascular anastomosis: report of experimental and clinical research.

Principles of microvascular anastomotic surgery are uncertain in contrast to the standardized suture methods for the repair of large arteries. Complications of early thromboses or late stricture at the microvascular anastomotic line can be related to the inherent biologic response of these delicate tissues to penetrating needle and suture. A new method for microvascular reconstruction based on the principle of flanged, nonpenetrated, intimal approximation by an arcuate-legged clip has proven biologically and technically superior to the penetrating microsuture. These conclusions are based on extensive testing in multiple surgical laboratories of the following parameters: long- and short-term patency, morphology of wound repair, and burst and tensile strength. Details of the new surgical system and experimental studies are described.

Unicoronal synostosis. A surgical intervention.

Computer-assisted medical imaging technology provides useful tools for the in vivo study of congenital craniofacial deformities. Using these tools, a standardized operation for infants with nonsyndromal unicoronal synostosis has been shown to consistently normalize orbital dimensions and decrease exocranial base angulation. Normalization of endocranial base angulation is less consistent. The plasticity of the infant's cranial base after cranial vault surgery suggests a locus of the primary pathology at a site other than the cranial base.

Secondary craniomaxillofacial deformities. Current principles of management.

This article focuses on secondary reconstruction of naso-orbital frontal injuries. Pertinent clinical anatomy, physical examination/documentation, and radiographic imaging are examined first. Pretreatment planning, operative exposures, and case examples are discussed in the second part of the article.

Rotation-advancement repair of the unilateral cleft lip. One center's perspective.

Most surgeons agree that the Millard rotation-advancement is the procedure of choice for treatment of the incomplete cleft lip and milder complete cleft forms. It has been found to be an effective procedure for essentially all cleft cases, regardless of width, when the staged approach described is employed. To reach the goal of establishing near-perfect anatomy and function, a protocol, which capitalizes on the strengths of Millard's repair while minimizing its weaknesses, has been adopted. Three aspects of the protocol are emphasized in this article: (1) presurgical manipulation of the maxillary alveolar segments via passive molding appliances; (2) a staged approach to lip repair for wide complete clefts, which includes preliminary lip adhesion; and (3) adherence to a standardized technique, which will allow for a long-term clinical study.

Secondary cleft deformities.

Cleft palate fistulas are an unfortunate complication that may follow repair of a cleft palate. The presenting symptoms may be variable, and a full evaluation is necessary to identify which symptoms are directly attributable to the fistula. A multidisciplinary approach is recommended when formulating a treatment plan, which takes into consideration symptomatology, speech, growth, dentition, aesthetics, and individual medical and psychosocial needs.

Assessment of the patient with cleft lip and palate. A developmental approach.

Children with cleft lip and palate require interdisciplinary team care from infancy through adolescence. An understanding of developmental stages allows the cleft palate team to adapt and integrate its services into the rapidly changing life of the child. This article discusses the maturational, developmental stages of childhood and the services the child with cleft lip and palate and the child's family deserve through each stage. Health care providers in all settings may continue to provide appropriate care for all patients with cleft lip and palate, despite the challenges of a changing health care environment, by emphasizing the needs of the child in all developmental stages.

Prospectives in cleft lip and palate repair.

A recent focus on fetal surgery has raised the possibility of fetal cleft lip and palate repairs. Because of the fetus's marked plasticity, fetal repairs may have results that more closely approximate normality. Furthermore, the impact on cost management could be dramatic with a decreased need for extensive postoperative care, orthodontia, and speech therapy. This article considers the future prospects of intrauterine cleft lip repair as the next generation of surgical advances.

Macrocephaly and minor congenital anomalies in children with learning problems.

To determine whether existing data which indicate a significant prevalence of abnormal head circumference and minor congenital anomalies in referred learning disabled populations could be replicated in a nonreferred population, 75 children enrolled in learning disabilities resource programs in an elementary school district were compared with a matched group of 73 children. Macrocephaly (occipitofrontal head circumference of 2 or more SD above the mean) was noted significantly more often in resource than in control children (12 versus 3) (p less than 0.05). Macrocephalic resource children had significantly lower achievement scores than either normocephalic resource or control children (p less than 0.05). There were no significant differences between resource and control children for mean number of anomalies per child, frequency of individual anomalies per group, or evidence of recurring anomaly patterns in the resource children. The macrocephalic resource children revealed the same prevalence of anomalies as normocephalic resource and control children, with no evidence of recurring anomaly patterns. Resource children with four or more anomalies did not differ from those with three or fewer anomalies on any psychometric parameters. Clinical, familial, radiological, and psychometric investigation of several macrocephalic resource children indicated similar patterns of learning deficits and documented evidence of familial macrocephaly but did not show any recognizable CNS abnormalities. This study confirms a significant prevalence of macrocephaly in children with learning problems. It indicates the need for further research into the prevalance and patterns of learning deficits in children with familial macrocephaly. The results add to recent concerns about the psychoeducational criteria of specific learning disabilities.(ABSTRACT TRUNCATED AT 250 WORDS)

Craniofacial onlay bone grafting: a prospective evaluation of graft morphology, orientation, and embryonic origin.

A prospective study using 46 young adult New Zealand rabbits was designed to evaluate onlay bone grafts to the craniofacial skeleton with respect to embryonic origin (membranous or endochondral), gross morphology (unicortical or bicortical), and orientation (cortex-to-bed relationship). Quantitative and qualitative data were analyzed and contrasted at both periods of evaluation (1.5 and 3.0 months). The embryonic origin of onlay bone grafts to the rabbit snout is significantly correlated with graft surface area, volume, weight, and recipient bed union for up to 3 months postoperatively. Over this interval, membranous bone (calvaria) grafts either persist in their entirety or increase, whereas endochondral bone (iliac) grafts resorb. Neither the number of cortices (unicortical or bicortical) nor the orientation of unicortical grafts (cortex-to-bed relationship) affected graft fate regardless of embryonic origin. Bone density remained unaltered during both resorption and deposition. Osteogenesis, demonstrated by serial fluorochrome markers, occurs in both membranous and endochondral bone grafts. Histologically, bone grafts of membranous and endochondral origin differ greatly in their cortical to cancellous diploe ratios and architectural configuration. We hypothesize that the differences found are related to the three-dimensional osseous architecture rather than to the embryonic origin of bone per se.

Autoradiographic analysis of expanded skeletal muscle in rats.

The use of tissue expanders in reconstructive surgery is now common. However, the physiologic mechanisms by which expansion is achieved are not well understood. A recent study demonstrated that rapid expansion of skeletal muscle is accompanied by an increase in the number of sarcomeres within a muscle fiber. This is in contrast to previous animal studies whose results suggested that synthesis of sarcomere units was limited to the perinatal period. To further investigate potential increases in sarcomeres and attempt to localize the active sites of sarcomere synthesis, labeled adenosine (3H) was injected into rats during the expansion of skeletal muscle. Adenosine was taken up by the muscle fibers and incorporated in the newly formed actin as part of light chains. An autoradiographic analysis of histologic sections of the expanded muscle demonstrated a statistically significant increase in radioactivity within the expanded muscle. The distribution of the radioactivity followed a proximal-to-distal gradient, with the proximal sections exhibiting more than 50 percent greater activity than the distal aspects. These data suggest a preference for sarcomere synthesis in the proximal portion of the expanding skeletal muscle. The significance of this finding is uncertain. However, we suspect that sarcomere synthesis is tension-dependent and likely to be related to local tension applied to a portion of the muscle fibers rather than to an anatomic site of preference.

Upper limb salvage using a free radial forearm flap.

Microsurgical transfer of a skin flap salvaged from a nonreplantable upper extremity that would otherwise be discarded may provide essential soft-tissue coverage of the amputation stump, so maintaining a functional range of motion in the elbow joint. A radial forearm free flap measuring 24 cm long by 9 cm wide was salvaged from the degloved forearm skin of a patient who sustained a proximal forearm amputation that was considered unsuitable for replantation. This allowed coverage of the proximal radius and ulna, preservation of a functional elbow joint, and successful fitting of a below-elbow prosthesis.

Elevated IGF-II and TGF-beta concentrations in human calvarial bone: potential mechanism for increased graft survival and resistance to osteoporosis.

Calvarial bone grafts may have greater survival as donor tissue than bone from other sites. Furthermore, calvarial bone is resistant to osteoporosis. Because bone contains growth factors that may play an important role in the regulation of bone repair, we proposed that bone from calvaria may be enriched in one or more growth factors. To test this hypothesis, samples of bone from 10 men 64 years of age or older that were obtained at autopsy from three skeletal sites (calvaria, iliac crest, and vertebral body) were cleaned, extracted by demineralization, and assayed for growth factors insulin-like growth factor I, insulin-like growth factor II, and transforming growth factor-beta. Insulin-like growth factor II and transforming growth factor-beta concentrations were significantly higher in calvaria than in iliac crest or vertebral body. We conclude that the increased concentrations of growth factors in calvarial bone may lead to a greater capacity for bone repair and graft retention.

Extensive and complex defects of the scalp, middle third of the face, and palate: the role of microsurgical reconstruction.

Twenty-one patients with gigantic defects of the scalp and middle third of the face and palate following excision of neglected or recurrent tumors, burns, and infections have undergone microsurgical reconstruction. Wide resection of the middle third of the face, orbit, and palate requires "complex" three-dimensional volume reconstruction, whereas extensive defects of the scalp and skull (exceeding 80 cm2) require coverage of the larger surface area soft-tissue defect and the exposed brain and dura. The latissimus dorsi free-muscle flap and split-thickness skin graft have become our methods of choice for extensive scalp and skull defects. The latissimus dorsi musculocutaneous free flap is preferable for reconstruction of complex palatal and external skin and orbital defects of the middle third of the face. Microsurgical free-tissue transfer reliably frees the oncologic surgeon from the constraints imposed by conventional reconstructive techniques and may therefore allow improved curative or at least palliative resection of these extensive tumors.

Endoscopic excision and repair of simulated bilateral cleft lips in fetal lambs.

The use of nonpenetrating clips to accomplish wound closure as an alternative to suture in the repair of simulated cleft lips in partially exteriorized fetuses has been described previously. In this study, the fetus is approached endoscopically, and clipped (n = 8) and sutured (n = 4) intrauterine endoscopic repairs in six lambs (90- to 95-day gestation) are compared. Also used was a newly developed harmonic scalpel to create the defects in the fluid environment. Clipped repairs were nearly 10 times faster than sutured repairs (2.7 +/- 0.5 minutes compared with 24 +/- 4 minutes, respectively). Furthermore, suture incited foreign body inflammation, recruited monocytic inflammatory cells, and exhibited notable scarring. The comparison between clipped and sutured repairs extends the previous observations to the realm of endoscopy and reinforces the previous conclusions of this group that the nonpenetrating clip is more rapid and incites less inflammation than suture in fetal wound approximation and repair.

Radiographic and aerodynamic measures of velopharyngeal anatomy and function following Furlow Z-plasty.

Recent studies have shown that the Furlow double-opposing Z-plasty has several advantages that make it an attractive procedure for cleft palate repair and treatment of velopharyngeal insufficiency in selected cases. The anatomic changes associated with this procedure have never been documented prospectively. The purpose of this study was to describe radiographic dimensions of the velopharynx and aerodynamic measures of velopharyngeal function in a group of patients before and after Furlow Z-plasty for the treatment of velopharyngeal insufficiency. Twelve consecutive patients with cleft palate and velopharyngeal insufficiency, ranging in age from 3 to 19 years, were selected as candidates for Furlow Z-plasty based on perceptual, endoscopic, and radiographic findings. Eight patients had repaired cleft palate with a residual muscle diastasis and four patients had unrepaired submucous cleft palate. Subjects received aerodynamic and cephalometric assessments before and after Z-plasty. Cephalometric x-rays were measured for velar length, thickness, and pharyngeal depth. Mean nasal airflow during pressure consonants (Vn) was calculated from pressure/flow studies, and patients were categorized as having complete closure (<10 cc/sec Vn) or incomplete closure (>10 cc/ sec Vn). After Z-plasty, there was a significant increase in velar length (p = 0.002) and velar thickness (p = 0.001). After surgery, patients with complete velopharyngeal closure had significantly greater velar length than the incomplete closure group (p = 0.05) with nearly twice the increase in length. Similarly, following surgery, the complete closure group had significantly greater thickness than the incomplete closure group (p = 0.01), with a greater postoperative increase in velar thickness (p = 0.005). Finally, there was a significant negative correlation between percent increase in length and percent increase in thickness for patients in the complete closure group (r = -0.91, p = 0.03). Findings demonstrate that following Furlow Z-plasty, patients with cleft palate and velopharyngeal insufficiency obtained significant increases in velar length and thickness. Greater velar length and greater velar thickness both were associated with complete velopharyngeal closure. Patients in the complete closure group tended to demonstrate large percent gains in either length or thickness or moderate gains in both. Patients in the incomplete closure group tended to demonstrate relatively small percent gains in both dimensions. Results suggest there may be important anatomic features (such as pharyngeal depth/velar length ratio) that can be evaluated before surgery to predict which patients may be most likely to benefit from Furlow Z-plasty as a form of treatment for velopharyngeal insufficiency.

Tissue reconstruction with nonpenetrating arcuate-legged clips. Potential endoscopic applications.

A new system for tissue approximation consisting of a nonpenetrating arcuate-legged clip applied to everted tissue edges to form an elastomeric flanged joint is described. The flanged joint has unusual physical and morphologic properties. Novel systems for tissue eversion, clip application and clip removal have been tested at the micro scale in blood vessels and the rat vas deferens (vasovasostomy). Human applications have been successful (cerebrovascular reconstruction, free-flap transfer, skin grafting, A-V access). The system is biologically and technically equivalent to or superior to the needle-and-suture technique. Avoidance of intimal or mucosal penetration or intraluminal foreign body is associated with prompt wound healing and the reconstitution of tubular integrity. The system is readily adaptable for endoscopic surgical reconstructions, providing the surgeon with enhanced reconstructive abilities.