Enhanced activity of osteoblast differentiation factor (PEBP2alphaA2/CBFa1) in affected sutural osteoblasts from patients with nonsyndromic craniosynostosis.

OBJECTIVE: Nonsyndromic craniosynostosis is characterized by premature closure of one or more cranial sutures in infants. The purpose of this investigation was to evaluate cellular and molecular events that lead to pathogenesis of nonsyndromic craniosynostosis. DESIGN: This study utilized discarded samples of normal and affected cranial sutures from 12 patients (7 boys, 5 girls) with nonsyndromic craniosynostosis. RESULTS: Histological evaluation of affected sutures revealed complete osseous obliteration instead of a zone of connective tissue and osteogenic cells as seen in normal sutures. Although proliferation of normal and affected osteoblasts did not vary substantially, elevated osteocalcin production and increased in vitro bone nodule formation indicated that the differentiation and the bone-forming potential of affected osteoblasts was significantly higher than that of normal cells. We therefore investigated the levels and activity of Cbfa1, a transcription factor that plays an integral role in osteoblast differentiation. Northern blot analysis of messenger RNA from both normal and affected sutural osteoblasts revealed a twofold increase in the expression of Cbfa1 in affected cells. This increase in the level of Cbfa1 transcript correlated with an increase in its transcriptional activity on the osteocalcin gene promoter, as assessed using gene transfer methods. CONCLUSION: Our results indicated that osteoblasts from synostosed sutures exhibit an increased potential for differentiation and bone formation. The increased level and activity of Cbfa1 could play a vital role in the aberrant function of these affected osteoblasts and may explain their altered behavior compared to the normal cells.

Total eyelid reconstruction with free dorsalis pedis flap after deep facial burn.

A case of severe facial and corneal burns with complete loss of upper and lower eyelids is reported together with the acute management and surgical options for total eyelid defects secondary to thermal injury. An acutely burned man with 78 percent total burn surface area presented with complete exposure of the left cornea. Because of the severe thermal injury, no facial tissues were available as donor sources for reconstructing the eyelid. A free dorsalis pedis flap was used to cover the exposed cornea after bilateral conjunctival advancement flaps, with septal cartilage graft for structural support. A conjunctivodacryocystorhinostomy was performed at the time of the coverage. The patient was unable to perform an exact visual acuity test; however, his gross vision was intact.

Transpalatal approach for the extracranial surgical repair of transsphenoidal cephaloceles in children.

The surgical treatment of transsphenoidal cephaloceles in children is controversial. Reduction and repair via a transcranial approach are associated with high postoperative rates of morbidity, mortality, and hypothalamic dysfunction. In this study, four patients, aged 3 to 35 months at surgery, underwent successful transpalatal repair of two encephaloceles and two meningoceles. Two patients presented with nasal obstruction in infancy, one presented with unexplained meningitis, and in one patient the lesion was found incidentally during evaluation for seizures. Two children had median cleft face syndrome, another had an associated Arnold-Chiari type I malformation, and the fourth had no other cranial abnormalities. All patients underwent preoperative evaluation including magnetic resonance (MR) imaging. Auditory, ophthalmological, genetic, endocrinological, or other evaluation was undertaken as indicated. Lesions were approached through the median raphe of the hard and soft palates. All cephaloceles were easily visualized and dissected after division of the nasal palatal mucosa. The dural sac and its contents were reduced by surface coagulation after division and dissection of the overlying mucosa. Once reduced, the bone defect was obliterated in three of four patients. The dura was not opened and anomalous neural elements were not resected. At follow-up evaluation, all patients demonstrated resolution of preoperative symptoms without evidence of infection or lasting morbidity. Follow-up MR imaging showed reduction in all cases. The authors conclude that this transpalatal approach is safe and reliable for the treatment of transsphenoidal cephaloceles in young children.

Laparoscopically assisted testicular autotransplantation for management of the intraabdominal undescended testis.

PURPOSE: The intra-abdominal testis continues to present a considerable urological challenge and the approach to its management continues to evolve. We report our initial experience with laparoscopically assisted testicular autotransplantation. MATERIALS AND METHODS: An intra-abdominal testicle was identified laparoscopically in 5 patients who subsequently underwent testicular autotransplantation. RESULTS: The success rate was 100% and median operative time was 5 hours. All patients were discharged home the day after surgery with no complications and a good result. CONCLUSIONS: Because of success with this technique, this procedure offers significant advantages (decreased hospital stay and lower morbidity) than an open or 2-stage Fowler-Stephens approach.

Testicular autotransplantation: a 17-year review of an effective approach to the management of the intra-abdominal testis.

PURPOSE: Patients with intra-abdominal testes represent a small but challenging group who require innovative therapy. We report our 17-year experience with testicular autotransplantation. MATERIALS AND METHODS: Testicular autotransplantation was performed in 23 patients with 27 intra-abdominal testes. RESULTS: The success rate was 96% and average operative time was 4.25 hours with 40 to 90 minutes for vascular anastomoses. A contralateral Fowler-Stephens procedure had previously failed in 3 cases. CONCLUSIONS: Since the variability of collateral blood supply in patients with high undescended testes may potentially compromise the Fowler-Stephens procedure, we believe that testicular autotransplantation should be strongly considered in such patients, particularly those with bilateral undescended testicles.

Testicular autotransplantation for the intra-abdominal testis.

Oftentimes patients with intra-abdominal testes require more than the standard procedure to accomplish orchiopexy. Division of the spermatic vessels has been one mainstay of operative approaches for the intra-abdominal testis since Fowler and Stephens (Congenital Malformations of Rectum, Anus, and Genitouriary Tracts, chapter 19, pp 306-320, 1963) provided an anatomically rational basis for this procedure. Silber and Kelly (J Urol, 115:452-454, 1976) first described using a microvascular anastomosis to bring extra blood supply to the testicle after mobilization of a high intra-abdominal testicle into the scrotum; however, this approach has not been adopted by many for a number of reasons. The microvascular skill and instrumentation required for a successful anastomosis are not universally available and there is a misconception that the procedure is a lengthy one (Bianchi, Br J Urol 56:521-524, 1984; Bogaert et al., Urology 42:182-188, 1993). We present our series for testicular autotransplantation used over a 17 year period with a greater than 95% success.

Use of the CORB needle biopsy for the harvesting of iliac crest bone graft.

A technique for iliac crest bone grafting has been described which allows rapid harvesting of bone with a significant reduction in the pain, scarring, and distortion traditionally associated with this technique.

Hypertrophic tonsils: the effect on resonance and velopharyngeal closure.

This paper presents a case of altered resonance secondary to hypertrophic tonsils. Through nasopharyngoscopy, the tonsils were found to be in the nasopharynx and interposed between the velum and posterior pharyngeal wall. This resulted in incomplete velopharyngeal closure and evidence of hypernasality. This large mass was also felt to obstruct sound transmission into both the oral and nasal cavities, causing a mixture of hyponasality and cul-de-sac resonance. Tonsillectomy resulted in an elimination of all of these characteristics. Resonance was judged to be normal on the postoperative assessment.

Tissue expanders in the lower face and anterior neck in pediatric burn patients: limitations and pitfalls.

Radovan's 1982 landmark work on the clinical use of tissue expanders was felt to be a panacea for multiple reconstructive problems. We have used and probably overused tissue expanders for reconstruction of many complicated pediatric facial burn problems. This has enlightened us to some of the limitations of their use, and we have, therefore, reassessed our indications for their use. From 1984 through 1990, 52 tissue expanders were used in 37 pediatric patients for face and anterior neck burn scar resurfacing. This experience, combined with the unique problems encountered with face and neck tissue expansion, provided the groundwork for operative guidelines. The long-term effects of gravity, growth, and scarring on facial features adjacent to expanded skin led to the following principles. (1) Caution should be used in advancing expanded neck skin beyond the border of the mandible. The risk of scar widening or possible lip or eyelid ectropion needs to be considered when planning these flaps. Extreme overexpansion is necessary to advance unburned neck flaps over the mandibular border to avoid these problems. (2) After advancement or rotational flaps neck flaps to the face, vertically directed suture lines in the neck may need redirection to prevent linear contracture. This correction may be performed during the primary operation or during revisions. (3) Expanded cheek or neck skin should preferably replace burned areas, but at the same time, not violate unburned facial aesthetic units. (4) To counteract the affects of gravity, expanded cheek skin in conjunction with expanded neck skin, if unburned, may be the best choice for face or mandibular border scar replacement.(ABSTRACT TRUNCATED AT 250 WORDS)

Congenital arteriovenous malformation of the finger resulting in cardiac decompensation: a case report.

Congenital arteriovenous malformations of the hand may be hemodynamically significant in the neonate. Progressive cardiac decompensation is an indication for surgical intervention. The cause and classification of congenital vascular lesions of the hand are discussed.

Complications of controlled tissue expansion in the pediatric burn patient.

All patients at the Burn Institute reconstructed with tissue expanders between June of 1984 and June of 1987 were included in this review. There were 122 expanders used in 77 patients. Complications were defined as "absolute" (23 of 122 expanders, 20 percent) if they resulted in loss of expanders or additional surgery or none of preoperative plan was satisfied or "relative" (14 of 122 expanders, 11 percent) if they included spotty alopecia or alopecia greater than 50 percent or the operative plan only partially satisfied, reflecting poor judgment. The most common absolute complication was prosthetic exposure secondary to wound dehiscence occurring in the scalp area. Complications relative to specific anatomic areas were neck and face, 2 of 20 (10 percent); lower extremity, 1 of 4 (25 percent); trunk, 0 of 6 (0 percent); and scalp, 20 of 92 (22 percent). We feel that this high complication rate in the use of tissue expanders may be unique to the pediatric burn patient. Knowledge of indications for use and potential complications is essential to add this entity to the armamentarium of the burn reconstructive surgeon.

Reconstruction following head and neck burns.

Burn reconstruction of the head and neck must first start with special care to this anatomic area in the early acute phase, with appropriate early débridement and coverage with sheet grafts of medium thickness into unit facial orientation. Postoperative garment and mask splinting, will help lessen the hypertrophic scar formation that frequently follows facial burns and skin coverage. Carefully planned reconstruction of these areas is indicated, with priority given first to the neck, then to the periorbital area, and then to perioral areas. Principles of scalp, ear, nasal, and cheek reconstruction following burns of the face are carefully outlined. The unit concept of burn scar resurfacing of the face has been the mainstay of our treatment. We have emphasized skin coverage of the face from similar donor site areas. The emotional and psychological effects of facial scarring secondary to severe burns are crippling to patients. Although numerous reconstructive surgical procedures may lessen the deformity, ultimately burn patients realize that their burn scars are permanent and no surgeon can give them back their original facial appearance. These patients need strong and continued support and reassurance from their physicians and nursing professional staff to maintain their self-identity and confidence.

Use of i.v. fentanyl in the outpatient treatment of pediatric facial trauma.

Fentanyl, a synthetic analgesic narcotic, was used in 2,000 cases of pediatric facial trauma between 1981 and 1984. A dose of 2 to 3 micrograms per kilogram of body weight was administered slowly intravenously to provide sedation and analgesia to facilitate the repair. The drug has advantages ideal for outpatient use, namely rapid onset, brief duration, and short recovery time. The major possible complication is that of apnea, which requires that resuscitation equipment be available. Three apneic episodes occurred in this series and all were successfully reversed with naloxone with no untoward effects.

Management of perioral burn scarring in the child and adolescent.

The experience gained in the treatment of 116 patients with perioral burn scarring is presented. Important considerations are the choice of unit release or simple release, the choice of donor site to match the prevailing skin of the remainder of the face, and the timing of the reconstruction. Delaying the reconstructive procedure with the routine use of pressure appliances until the scar was mature produced a more pleasing final result. In addition, we found that modifying the traditional aesthetic unit excised to include darts when there was severe burning of the surrounding facial cheek skin with loss of natural nasolabial folds improved the final result and reduced the need for secondary revision.

Acute management of severe hand injuries.

Mutilating hand injuries are extraordinarily complex, involving all structures of the hand. Immediate treatment priorities are described, and planning for staged reconstruction is formulated, based on the most reasonable and realistic goals involved in restoration of use for the patient.