The craniofacial anomalies archive at St. Louis Children's Hospital: 20 years of craniofacial imaging experience.

This article describes how the Craniofacial Imaging Laboratory at the Cleft Palate and Craniofacial Deformities Institute, St. Louis Children's Hospital, Washington University Medical Center, has developed an electronic archive for the storage of computed tomography image digital data that is independent of scanner hardware and independent of units of storage media (i.e., floppy disks and optical disks). The archive represents one of the largest repositories of high-quality computed tomography data of children with craniofacial deformities in the world. Archiving reconstructed image data is essential for comparative imaging, surgical simulation, quantitative analysis, and use with solid model fabrication (e.g., stereolithography). One tertiary craniofacial center's experience in the establishment and maintenance of such an archive through three generations of storage technology is reported. The current archive is housed on an external 35-GB hard drive attached to a Windows-based desktop server. Data in the archive were categorized by specific demographics into groups of patients, number of scans, and diagnoses. The Craniofacial Imaging Laboratory archive currently contains computed tomography image digital data for 1827 individual scans. The earliest scan was done in 1980; the most recently stored scan for the purposes of this report occurred in May of 2000. The average number of scans archived per complete year was 94, with a range of 59 to 138. Of the 1827 total scans, 74 percent could be classified into specific diagnostic categories. The majority of the archive (55 percent) is composed of the following five diagnoses: sagittal synostosis (17 percent), unilateral coronal synostosis (11 percent), hemifacial microsomia (10 percent), plagiocephaly without synostosis (10 percent), and metopic synostosis (7 percent). Storage of computed tomography image data in a digital archive currently allows for continuous upgrading of image display and analysis and facilitates longitudinal and cross-sectional studies, both intramural and extramural. Internet access for clinical and research purposes is feasible, but contingent on protection of patient confidentiality. The future of digital imaging regarding craniofacial computed tomography scan storage and processing is also discussed.

Long-term outcome of autogenous rib graft nasal reconstruction.

Whereas reconstruction of the hypoplastic nose with rib grafting is common, the long-term outcomes of nasal growth and aesthetics are unknown. This study assessed nasal morphometrics, patient satisfaction, and the perception of nasal appearance by others up to 15 years after nasal reconstruction using cantilevered autogenous chondro-osseous rib grafting with rigid internal fixation in children. Records of all patients who received nasal rib grafts between 1983 and 1998 by one senior surgeon were reviewed. Patients in this study were operated on before their late teens and had greater than 1-year follow-up including serial photographic documentation. Nasal growth was determined by comparing anthropometric measurements preoperatively, perioperatively, and postoperatively. Patient satisfaction was determined through a questionnaire that addressed memory, donor-site morbidity, and nasal perception. Independent, blinded skilled observers who reviewed frontal and lateral photographs of the preoperative, perioperative, and postoperative intervals assessed nasal aesthetics. Thirty-two patients who underwent 38 rib graft reconstructions of the nasal dorsum and tip at an average age of 8.8 years constitute the study population. Six patients underwent secondary augmentation. The average interval between initial nasal reconstruction and evaluation for this study was 7.9 years. Comparative anthropometric measurements before and after surgery documented increases in both tip projection (2.3 percent) and nasal length (3.0 percent) and a decrease in nasolabial angle (1.9 percent). Patient satisfaction interview response rate was 100 percent of those whom we were able to contact (28 of 32). The average age at interview was 17.2 years. Most patients recalled the operation and denied recollection of pain. Donor-site long-term morbidity was not an issue for 86 percent of patients. Sixty-four percent of patients remembered their preoperative nasal appearance and 89 percent of these preferred the postoperative change and were not concerned with nasal scars or texture. Almost two-thirds of the patients had fixation screws removed from the nasal dorsum because of skin erosion, easy palpability, or visibility. Although several patients expressed a desire to make minor additional changes to their nose, only one of these elected offered presurgical consultation and none have had such surgery. The postoperative nasal appearance compared with that preoperatively was rated as improved for 66.3 percent of responses, 26.5 percent as unchanged, and 7.2 percent as deteriorated. Cantilevered autogenous chondro-osseous rib graft reconstruction of the nasal dorsum is an effective means of reconstruction for the hypoplastic nose in childhood with respect to morphometric measurements, patient self-perception, and the assessment of nasal appearance by others.

Plasticity of the endocranial base in nonsyndromic craniosynostosis.

Limited in vivo data exist on the dysmorphology of the cranial base in nonsyndromic craniosynostosis. Few studies have documented the effect of calvarial surgery for synostosis on endocranial morphology. Previous work has suggested that the dysmorphology of the endocranial base is diagnostically specific for metopic, sagittal, and unicoronal sutures. The purpose of this study was to further evaluate the endocranial base in infants with nonsyndromic craniosynostosis by testing the hypothesis that the dysmorphology is, to some degree, a secondary deformation rather than a primary malformation. Three questions were addressed: (1) Can individuals reliably identify affected suture-specific endocranial-base morphology using standard templates? (2) Does calvarial surgery in infancy for craniosynostosis affect the perception of endocranial-base morphology? and (3) Does calvarial surgery in infancy for nonsyndromic craniosynostosis normalize the endocranial base?In this study, three-dimensional volumetric reconstructions from archived computed tomography digital data were processed using the ANALYZE imaging software. Dysmorphology was assessed by nine independent, blinded skilled observers who reviewed two separate sets of images of endocranial bases. Both sets contained images from the same patients: one set contained preoperative images, and the other contained images of the endocranial base 1 year after calvarial surgery. Observers were asked to sort each set into four suture-specific diagnostic groups: normal, unicoronal, metopic, and sagittal. Each set contained 10 patients with unicoronal synostosis, 10 with metopic synostosis, 10 with sagittal synostosis, and four normal patients. Seventy-eight percent of the total number of preoperative images were correctly sorted into the suture-specific diagnostic group, whereas only 55 percent of the total number of postoperative images were correctly matched. With regard to the individual sutures, the results were as follows (data are presented as preoperative accuracy versus postoperative accuracy): metopic, 76 percent versus 44 percent; sagittal, 58 percent versus 34 percent; unicoronal, 100 percent versus 79 percent; and normal, 83 percent versus 72 percent. Although 36 of 306 total images per group (12 percent) actually represented normal patients, the observers called 72 of 306 normal (24 percent) in the preoperative set versus 110 of 306 normal (36 percent) in the postoperative set. In conclusion, (1) the endocranial dysmorphology of nonsyndromic craniosynostosis is recognizably specific to the affected suture; (2) calvarial surgery for nonsyndromic craniosynostosis normalizes the endocranial base qualitatively with regard to the diminished ability of raters to identify the primary pathology; and (3) the documented postoperative changes in endocranial base morphology after calvarial surgery for nonsyndromic craniosynostosis in infancy indicates that a major component of that dysmorphology is a secondary deformity rather than a primary malformation.

Secondary reconstruction of a giant congenital lentiginous dermal nevus with serial, large-volume tissue expansion.

Giant congenital pigmented nevi pose a substantial reconstructive challenge for the treating physician. Due to the increased risk of malignant transformation in such lesions, complete excision with tissue expansion or skin grafting is the generally accepted treatment. These modalities can, however, leave the patient with secondary deformities that also require complex reconstructive procedures. The following case details a patient requiring secondary reconstruction with large-volume tissue expansion 12 years after excision of a giant nevus, and split-thickness skin grafting. This patient illustrates a severe secondary deformity and the usefulness of large-volume serial expansion in such patients.

Comparison of topical anti-ischemic agents in the salvage of failing random-pattern skin flaps in rats.

OBJECTIVE: To determine the efficacy of topical anti-ischemic drug therapy in the salvage of failing, random-pattern skin flaps. DESIGN: Prospective, randomized, placebo-controlled, therapeutic trial. SETTING: Academic medical center. SUBJECTS: Sixty-one adult male Sprague-Dawley rats. INTERVENTION: Each experimental rat underwent a caudally based random-pattern skin flap using the modified McFarlane technique. Rats were randomized to 1 of 6 treatment groups: topical nifedipine, topical trolamine salicylate, topical nitroglycerin, topical trolamine salicylate-nitroglycerin combination, topical nifedipine-trolamine salicylate-nitroglycerin combination, or inert carrier ointment (control). Treatment was initiated immediately following flap closure and continued every 6 hours for 7 days. At the end of the treatment period, animals were euthanized and flap survival was determined for each one. RESULTS: Topical anti-ischemic drug therapy resulted in a statistically significant reduction in ischemic flap necrosis for each drug (or combination) tested relative to the 44.2% mean necrosis observed in control animals. Treatment with the combination of topical nitroglycerin and topical trolamine salicylate resulted in the best salvage response (25.2% mean necrosis) with a statistically significant improvement in flap survival relative to both controls and nitroglycerin alone. CONCLUSIONS: Topical anti-ischemic agents are effective in reducing ischemic necrosis of failing, random-pattern skin flaps in the rat model. Although nitroglycerin, trolamine salicylate, and nifedipine possess unique pharmacologic mechanisms of action, each drug produced a statistically significant improvement in flap survival. The results of this study suggest that topical drug therapy may play an important role in clinical salvage of the failing skin flap. Further studies are needed to explore the potential of combination drug therapy.

Patterns of maxillofacial injuries in powered watercraft collisions.

Because of the widespread popularity of water sports, plastic and reconstructive surgeons can expect to manage an increasing number of injuries associated with these activities, particularly those related to powered watercraft vehicles. Although seat belts for motorists and helmets for motorcyclists may be efficacious, such devices currently do not serve a similar role in powered watercraft sports. In this study, a retrospective chart review of 194 consecutive patients who presented to the University of Miami/Jackson Memorial Hospital (Level I trauma center) as a result of powered watercraft collisions is presented. The purpose of this investigation was to assess the incidence, cause, demographics, and available management options for head and neck injuries secondary to powered watercraft. Identified were 194 patients who presented because of watersports-related injuries during the period January 1, 1991, through December 31, 1996. From this group, 81 patients (41.8 percent) sustained injuries directly attributable to powered watercraft collisions, including 41 personal watercraft collisions (50.6 percent), 39 boat collisions (48.1 percent), and 1 airboat collision (1.2 percent). The patient population, as expected, tended to be young and male with an average age of 29 years (range, 8 to 64 years old). Interestingly, 41 of the patients (50.6 percent) who presented to this trauma center as a result of powered watercraft collisions also sustained associated head and neck trauma. Of 74 injuries 24 were facial fractures (32.4 percent), 18 were facial lacerations (24.3 percent), 14 were closed head injuries (18.9 percent), 8 were skull fractures (10.8 percent), 4 were scalp lacerations (5.4 percent), 4 were C-spine fractures (5.4 percent), 1 was an ear laceration (1.4 percent), and 1 was a fatality (1.4 percent). Le Fort fractures were the most commonly identified facial fracture in this series. The number of these injuries seen in hospital emergency rooms will most likely increase in the future as the popularity of water-related recreational activities becomes even more widespread. Based on these findings, it is strongly recommended that future efforts be directed toward the prevention of these injuries through patient education and the eventual development of efficacious and safe protective equipment.

RU486 on an estrogen background blocks the rise in serum follicle-stimulating hormone induced by antiserum to inhibin or ovariectomy.

We used passive immunization with an antiserum to the alpha-subunit of inhibin (anti-I) or acute ovariectomy to investigate the relationship between serum inhibin levels and FSH secretion in the presence of the progesterone/glucocorticoid antagonist RU486. We demonstrated previously that 1) anti-I administered at 1700 h causes serum FSH to rise on the morning of estrus, even in the presence of a GnRH antagonist, when the two treatments are delivered on proestrus; and that 2) RU486 given on proestrus (1230 h), a time when serum estradiol levels are high, not only blocks the natural secondary FSH surge, but also suppresses the anti-I-induced rise in serum FSH on the morning of estrus. We have now extended our studies of the relationship between inhibin and RU486 to investigate treatment with RU486 and anti-I on a different day of the cycle, estrus, when serum estradiol levels are low. When both RU486 and anti-I were given on estrus (1230 and 1700 h, respectively), RU486 failed to block the anti-I-induced rise in serum FSH on the next morning of metestrus, in contrast to the blockade seen with RU486 treatment on the day of proestrus. However, pretreatment with estradiol benzoate (50 microgram) on the evening of proestrus, before the RU486 and anti-I treatment on estrus, caused RU486 to suppress the effects of anti-I on serum FSH, as it does when given on proestrus. We then repeated the study, using ovariectomy on proestrus or estrus (1700 h) to raise serum FSH, and assessed the effects of RU486 treatment at proestrus and estrus and estradiol benzoate treatment on proestrus. Our results indicate that treatment with RU486 can block the postovariectomy rise in serum FSH only in the presence of high circulating estradiol levels. We conclude that the inhibitory action of RU486 on FSH secretion after a fall in serum inhibin depends on a precedent estradiol background, probably due to induction of progesterone receptors by estradiol.

Mechanism of the inhibitory action of RU486 on the secondary follicle-stimulating hormone surge.

Recent evidence utilizing RU486 has implicated progesterone (P) and glucocorticoids, in addition to a drop in serum inhibin, in the development of the secondary FSH surge on the morning of estrus. To assess the role of these steroids, we treated proestrous female rats with the antiprogestin/antiglucocorticoid RU486 (6 mg/kg sc) at 1230 h, and with dexamethasone (dex; 8.4 or 16.2 mg/kg sc), or with the steroid biosynthesis inhibitor aminoglutethimide (AG; 150 mg/kg ip) at 1030 h, alone or in combination with RU486. The effects of these treatments on uterine ballooning and intraluminal fluid content (an index of P action), ovulation, and serum levels of P, corticosterone (B), FSH, LH, and inhibin-alpha at 1830 h proestrus and 0900 h estrus were examined. In accord with previous work from our laboratory, RU 486 caused uterine intraluminal fluid retention on the morning of estrus and significantly suppressed the preovulatory surges of both FSH and LH, and the secondary surge of FSH without affecting the fall in inhibin-alpha. Treatment with dex alone raised serum FSH at both 1830 h proestrus and 0900 h estrus, coincident with suppression of serum inhibin-alpha. When administered in combination with RU486, dex partially reversed the increased uterine intraluminal fluid retention at 0900 h estrus, but did not modify the inhibitory effect of RU486 on the primary gonadotropin surges or the secondary surge of FSH. AG alone significantly suppressed serum P, B, and gonadotropins (LH to a greater extent than FSH) at 1830 h proestrus and blocked ovulation and uterine intraluminal fluid release at 0900 h estrus; it did not, however, suppress the secondary FSH surge or prevent the fall in serum inhibin-alpha. When administered 2 h before RU486, AG did not prevent the RU486-induced inhibition of the primary gonadotropin surges or the secondary FSH surge. We conclude from these results that development of the secondary FSH surge does not require P or glucocorticoid action and that RU486 suppression of the secondary FSH surge does not involve blockade of binding of these steroids to their receptors. Our data are compatible with ligand-independent activation of the P receptor, susceptible to blockade by RU486, as the mechanism underlying the enhanced secretion of FSH from the gonadotrope on the morning of estrus.