Three-dimensional computed tomographic scanning and major surgical reconstruction of the head and neck.

The major advantage of three-dimensional computed tomographic imaging is the ability to obtain accurate anatomic measurements of distance, area, and volume. This accuracy is possible because the three-dimensional relationships of soft tissue and skeleton are retained. This feature is particularly useful in reconstruction of the upper facial skeleton, where standard roentgenograms and computed tomographic scans are of limited value. Computer-interactive surgical procedures have been used for correction of congenital deformities and for removal of orbital neurofibromas and subsequent reconstruction. Because three-dimensional imaging is expensive and exposes the patient to high levels of irradiation, this approach is recommended only when a specific indication exists and when it can contribute to problem solving.

Traumatic carotid artery dissection causing blindness.

A case of delayed postoperative visual loss due to bilateral traumatic carotid artery dissection is presented. In patients with a major craniofacial injury due to a high-speed motor vehicle accident, we suggest that carotid artery duplex ultrasonography be used in the initial evaluation for possible carotid artery dissection. Magnetic resonance imaging of the head and neck with magnetic resonance angiography should be performed subsequently if indicated. Early diagnosis and initiation of therapy can minimize complications.

Modified pudendal-thigh flap for correction of penoscrotal transposition.

OBJECTIVES: In patients with penoscrotal transposition, an occasional postoperative problem has been a deficiency of skin on the proximal penile shaft that results in penoscrotal fusion and tethering. METHODS: We describe a new operation using a modified neurovascular pudendal-thigh flap for correction of incomplete penoscrotal transposition. RESULTS: This procedure has been used in 6 children, and an excellent cosmetic and functional result has been achieved in each patient. CONCLUSIONS: The flaps provide a reliable blood supply, maintain normal innervation, and correct the problem of postoperative penoscrotal fusion and tethering. This technique preserves sufficient penile skin for a tension-free second-stage urethroplasty.

Decompression for dysthyroid ophthalmopathy via the orbital rim approach. Technical note.

A technique is described wherein the approach to the orbital cavity, with resection of its roof and lateral wall, is facilitated by a single burr hole and local en bloc removal of the lateral and supraorbital margins. A satisfactory decompression with reduction of proptosis of the orbital contents and a good cosmetic result is achieved without the need for a large dural exposure. The approach may be combined with removal of the anterior wall of the frontal sinus in cases where the lateral aspect extends appreciably laterally. Access to the orbital roof and lateral wall is straightforward and can be coupled with further removal of the floor lateral to the infraorbital nerve and medial wall. Advancement of the orbital rim upon bone replacement adds to orbital volume, creating better mechanical advantage for eyelid closure.

Orbital rim and malar advancement for unilateral coronal synostosis in the older pediatric age group.

The authors describe a technique for lateral orbital rim and malar advancement in patients in the older pediatric age group. The technique makes use of a strip craniotomy containing the supraorbital margin, greater sphenoid wing, and temporal bone, with en bloc inclusion of the lateral orbital rim, zygoma, and malar prominence. The method allows a contoured yet stable construction secured in a tongue-in-groove fashion with plate-and-screw fixation. It creates a symmetrical reconstruction of both frontal and lateral orbital aspects in the untreated or inadequately treated older plagiocephalic child with orbital dystopia. The accompanying malar recession is likewise corrected.

The clinical behavior of desmoplastic melanoma.

BACKGROUND: Desmoplastic melanoma (DM) is a rare variant of malignant melanoma. A better understanding of the clinical course of DM will impact on its treatment. METHODS: We reviewed the medical records of 59 patients with DM seen at the Mayo Clinics Scottsdale and Rochester since 1985. RESULTS: Thirty-seven (63%) patients were male with a mean age of 62.8 years. The mean DM thickness was 6.5 mm. A total of 23 patients (39%) experienced local recurrence (LR). LR correlated with positive, unknown, or <1 cm margins. Fifty percent of patients who locally recurred subsequently developed metastatic disease. No patients were found to have positive nodal disease during ELND (16) or SLN biopsy (12). Only 1 patient (2%) developed delayed regional node metastases. Sixteen patients developed metastatic disease. The most common site was the lungs (81%). CONCLUSIONS: LR is a significant problem and correlates with an increased risk of systemic metastatic disease. With the rare occurrence of lymphatic spread, we recommend patients undergo SLN biopsy only. DM appears to preferentially metastasize to the lungs and should be targeted when evaluating the patient for metastatic disease.

En block forehead reconstruction with split-thickness cranial bone.

This report demonstrates the utility of split-thickness cranial grafts harvested by craniotomy in the reconstruction of the entire forehead in an en bloc fashion. The technique allows a cosmetic recontouring of large surfaces of the cranial vault in particular, with little or no need for multiple extracranial grafts. Computer imaging and three-dimensional graphic reconstruction lend themselves well to preoperative planning.

Microorbitalism: a technique for orbital rim expansion.

A simple technique for orbital aperture expansion to facilitate placement of ocular prostheses is described. Both superolateral and inferolateral orbital margins are released by means of a single burr hole craniectomy of the frontosphenoid bone behind the orbital process of the frontal bone. Vertical and horizontal marginal lengthenings are performed by a rotatory displacement of one bone segment alongside the other. The expanded osseous aperture is secured with wire and plate-and-screw fixation following a supraorbital rim craniectomy to allow an adequate fit. The result provides for easier access of ocular prostheses and tissue expanders. The method has been applied to a series of patients with microorbitalism due to unilateral or bilateral congenital anophthalmia over the past 3 years without complication and with excellent results. Three-dimensional re-formatted CT reconstructions of the craniofacial skeleton are shown preoperatively and postoperatively.

Mixed heterotopic gastrointestinal and respiratory cyst of the lip: case report and review of the literature.

A congenital heterotopic gastrointestinal and respiratory cyst arising from the upper lip is reported. The lesion was excised directly with a satisfactory aesthetic result. Long-term follow-up is recommended because of a report of recurrence of this type of lesion after 13 years.

Metopic and sagittal synostosis: intracranial volume measurements prior to and after cranio-orbital reshaping in childhood.

This study applied a proven method for obtaining intracranial volume measurements using CT scans to (1) measure the intracranial volume of a consecutive series of children with either metopic or sagittal synostosis prior to any craniofacial procedure, (2) perform a standard cranio-orbital operation in each child, follow them longitudinally, and remeasure their intracranial volume 1 year later, and (3) compare their intracranial volumes with those of an age- and gender-matched cohort and review their cranial growth velocity. The study included 18 children who presented sequentially with untreated isolated nonsyndromic sagittal (n = 8) and metopic (n = 10) synostosis between 1987 and 1990 and who subsequently underwent cranio-orbital reconstruction by the senior author (Posnick) in conjunction with a pediatric neurosurgeon. The primary method of osteotomy and bone-graft fixation varied (i.e., wires, miniplates and microplates and screws). The series included 5 females and 13 males with an average age at the time of operation of 27 months (range 6 to 87 months). The postoperative clinical follow-up ranged from 13 to 47 months at the close of the study. Comparison of our patients' preoperative intracranial volumes with age- and gender-matched volumes available through Lichtenberg showed that 16 of 18 (89 percent) had volumes at or greater than the mean, with 44 percent (7 of 16) exceeding 2 standard deviations above the mean. When comparing our patients' late postoperative volumes with the Lichtenberg normative measurements, 94 percent achieved values at or greater than the mean. All patients achieved increased intracranial volume in association with the surgery performed and the time that lapsed between scan intervals. The majority of our patients (16 of 18) followed an intracranial volume growth curve that closely approximated the norm but with a starting point determined by the preoperative value. Two of 18 followed a growth curve that exceeded the rate of expansion expected for normal children. Our findings suggest that premature closure of either the sagittal or metopic suture does not result in diminished intracranial volume prior to or after the cranio-orbital procedures carried out in childhood. For the majority of the children in our study, both the preoperative intracranial volume and the rate of cranial expansion approximated or surpassed that of children without synostosis. The surgical techniques of skull and upper orbital reshaping with varied forms of osteotomy and graft fixation did not result in a global form of growth restriction. These findings are contrary to standard thinking about the biologic effects of craniosynostosis and raise new questions about our rationale for treatment.

Crouzon and Apert syndromes: intracranial volume measurements before and after cranio-orbital reshaping in childhood.

This study applied a proven method for obtaining intracranial volume (ICV) measurements using computed tomographic scans to document ICVs in children with Crouzon and Apert syndrome before and after cranio-orbital reshaping procedures. Their ICV also was compared with those of an age- and gender-matched cohort and reviewed their rate of cranial expansion. The study included 21 children who presented sequentially with Crouzon (n = 13) or Apert (n = 8) syndrome between 1987 and 1991 and who subsequently underwent a standard first-stage cranio-orbital reconstruction by the senior author (J.C.P.) in conjunction with a pediatric neurosurgeon. The primary method of osteotomy and bone graft fixation varied (i.e., wires, microplates, miniplates, and screws). The series included 16 females and 5 males with an average age at the time of operation of 13 months (range, 6 to 46 months). The postoperative clinical follow-up ranged from 12 to 60 months at the time of the study's completion. Comparison of the IVCs of the Apert patients preoperatively and postoperative with age and gender control volumes showed that six of eight had volumes at least 2 SD above the mean. Postoperatively, the trend continued with all eight patients followed longitudinally; all maintained volumes in excess of 2 standard deviations above the mean. Preoperatively, for the Crouzon children evaluated, 12 of 13 had ICV values greater than the mean. When comparing the patients' postoperative volumes to the normative data, all 13 maintained values at or greater than the mean at the time of their postoperative determination. Ten of the 13 achieved ICVs at or greater than 2 SD above the mean. When reviewing each Crouzon patient's cranial capacity over time, 5 of the 13 approximated the normal growth curve whereas 6 of the 13 exceeded it. This study confirms that Apert patients are macrocephalic before and after standard cranio-orbital procedures carried out in childhood. For the majority of children born with Crouzon syndrome, the cranial capacity will exceed the mean early in life and expand at a rapid rate after cranio-orbital decompression. The biologic explanation for these findings remain unclear.

Paget's disease of the groin: report of seven cases.

Only 27 cases of Paget's disease of the groin have been reported to the present. Our aim was to describe the clinical behavior and treatment of this disease. A retrospective analysis of patients seen at the Mayo Clinic over a period of 25 years (January of 1970 to December of 1995) was undertaken. We included patients with lesions of the groin (isolated or associated with penile/scrotal locations) and with histologic confirmation of the diagnosis. We found seven patients, all male; three patients had isolated lesions. Their mean (SD) age was 73 (8.3) years. Special stains and immunohistochemistry confirmed the diagnosis in all patients. A wide local excision was performed in every patient. Three patients had recurrence; one of them died with multiple pulmonary metastases 4 years later. Two patients presented with history of associated malignancy (prostatic and renal cell carcinoma). Paget's disease of the groin is extremely infrequent. Its origin seems not to be a simple extension from the genital area. Most diagnoses can be made by light microscopy. Wide local excision with free margins is the treatment of choice. Local recurrence occurs in half of patients with tumor-free margins by frozen section; long-term follow-up is warranted. Distant metastases occur rarely, although they can be fatal.

Indirect intracranial volume measurements using CT scans: clinical applications for craniosynostosis.

This paper describes a method for obtaining indirect intracranial volume measurements using CT scans with CTpak, a software package for quantitative analysis of CT scan data. The validity of this technique was confirmed by comparing direct measurement of the intracranial volume of five dry skulls with axial scans at 1.5- and 4-mm slice intervals to determine indirect volume. The indirect intracranial volume measurement technique was then used to compare preoperative and postoperative intracranial volume in 30 patients with craniosynostosis who underwent cranial vault and orbital osteotomies with reshaping and advancement. Our findings show that the suture release and simultaneous reshaping procedures usually carried out are, in fact, associated with increased intracranial volume. The observed intracranial volume gain is attributable to a combination of factors, including the surgical procedure carried out and ongoing growth. These factors are further modified by the diagnosis, age of the patient, and time interval between CT scans.

Massive abdominal-wall hernia reconstruction with expanded external/internal oblique and transversalis musculofascia.

We describe a technique for expansion and primary closure of massive and large recalcitrant abdominal-wall hernias in the middle and lower abdomen utilizing expanders placed in the lateral abdominal wall between the external oblique and the deeper complex of the internal oblique and transversalis fasciae. Since this technique describes expansion of the lateral abdominal wall, insertion incisions are made in the lateral abdominal wall away from the primary zone of injury surrounding the abdominal hernia and without interrupting the blood supply or innervation to the abdominal-wall muscle, fascia, or skin. This technique, described in four patients with massive abdominal-wall hernias, has been used successfully for primary closure with vascularized autogenous abdominal-wall fascia, obviating the need for interposition of prosthetic material or extraabdominal flaps.

Fistula formation and repair after palatal closure: an institutional perspective.

We retrospectively reviewed 119 consecutive patients who underwent cleft palate repair at the Mayo Clinic to determine the incidence of postoperative fistula formation, to assess possible contributing factors, and to review the methods of surgical management. Fistulas of the secondary palate were included, but nasal-alveolar fistulas and intentionally unrepaired anterior palatal fistulas were excluded. Six patients whose repairs were performed after 2.5 years of age were excluded to ensure a more uniform patient population. Cleft palate fistulas occurred in 13 of the 113 patients (11.5 percent). The median age at repair was 8.2 months, and the median follow-up period was 5.2 years. Several variables were analyzed by means of the log-rank test to determine their significance in postoperative fistula formation. Sex, extent of clefting (as estimated by the Veau classification), and type of palatal closure did not significantly affect the rate of fistula formation. However, patients who had palatal closure at an age younger than 12 months had a lower incidence of fistula formation (7.8 percent) than children whose closures were performed between the ages of 12 and 25 months (19.4 percent) (p = 0.058). The strongest predictor of the occurrence of a cleft palate fistula was the surgeon performing the procedure (p = 0.008). Fistula repair was deemed necessary in 11 of 13 patients, and 91 percent of these fistulas were healed with a single operation. Most of these fistulas were closed by using local flaps and two-layered closures. Cleft palate repair carries a significant but acceptable risk of fistula formation, which can be managed with local flaps. Fistula occurrence is related most to the experience level of the operating surgeon.

Familial gigantiform cementoma.

Familial gigantiform cementoma is a rare autosomal dominant tumor that is benign but can result in disfigurement of the facial skeleton. Two families with a total of five patients presented for treatment. Because of a lack of opportunity to obtain treatment early, three of the patients presented in adult life with massive tumors requiring extensive resection and complex reconstruction in multiple stages. The two female patients had chronic anemia caused by multifocal polypoid adenomas of the uterus and required hysterectomy before treatment. The last three patients had elevated alkaline phosphatase levels before tumor resection, and these levels decreased after surgery. With extensive resection of the tumors and reconstruction of both the soft tissues and facial skeleton, good functional and aesthetic results can be obtained. There has been no tumor recurrence with 3 years of follow-up.

Orbital volume measurements in enophthalmos using three-dimensional CT imaging.

The purpose of this study was to investigate enophthalmos by measuring the volume of various orbital structures using off-line computer techniques on images generated by a CT scanner. Eleven patients with enophthalmos had CT scans of the orbits consisting of 30 to 40 adjacent 1.5-mm slices. The data from the scans were analyzed on a Nova 830 stand-alone computer system using software programs that allowed measurement of total bony orbital volume, total soft-tissue volume, globe volume, orbital fat volume, neuromuscular tissue volume, and apex-to-globe distance in the horizontal plane. These data were analyzed comparing the volumes in the normal eye with the volumes in the enophthalmic eye in each patient. The analysis demonstrated a statistically significant increase in bony orbital volume in the enophthalmic eye, but the total soft-tissue volume, fat volume, neuromuscular tissue volume, and globe volume were the same as in the normal eye. The apex-to-globe distance, a measure of the degree of enophthalmos, was less in the enophthalmic eye than in the normal eye. These results suggest that in the majority of patients, the cause of posttraumatic enophthalmos is increased bony orbital volume rather than by soft-tissue loss or fat necrosis. (Several patients showed no volume discrepancies, and it is likely that cicatricial contracture is responsible for the enophthalmos in these cases.) This study suggests that the objective of surgery for correction of enophthalmos in patients with a volume discrepancy should be to decrease the volume of the bony orbit and to increase the anterior projection of the globe.

The role of tissue expanders in an anophthalmic animal model.

A study of orbital bony expansion using a custom tissue expander was performed in the anophthalmic cat model. Twelve 6-week-old kittens underwent right unilateral enucleations. Six kittens had immediate insertion of a tissue expander into the orbit. The remaining six served as controls. Every 2 weeks 0.5 cc saline was injected into the expander to a maximum of 5 cc. External horizontal and vertical orbital dimensions were obtained by palpation technique weekly. All animals had preoperative and study conclusion head CT scans with three-dimensional reconstructions performed. Dry skull preparations were done at the study conclusion at 24 weeks. Results demonstrated that tissue expanders were successful in maintaining normal orbital growth and size relative to the contralateral control orbit. The animals with enucleation only had an average difference in vertical and horizontal orbital measurements of -27 and -13 percent when compared with the contralateral normal orbit. In contrast, the enucleation and tissue-expansion animals had vertical and horizontal measurements of +4 and +2 percent (p less than 0.05) when compared with the contralateral orbit. Head CT scans with three-dimensional reconstructions demonstrated normal orbital geometry and volume for the animals with tissue expanders, whereas animals with enucleation only had small hypoplastic orbits. In conclusion, orbital tissue expanders offer a promising new technique in the treatment of anophthalmos.

Oropharyngeal hairy polyp with meningothelial elements.

The so-called hairy or teratoid polyp is a rare lesion of bigerminal origin that comprises elements derived from both ectodermal and mesodermal cell lines. In this article we report the presence of meningothelial elements in a hairy polyp, a previously undescribed component of this entity. The lesion was characterized by a pedunculated outgrowth from the hard palate. The surface of the outgrowth was covered by squamous epithelium and a central core of fibroadipose tissue, pilosebaceous glands, cleftlike pseudovascular spaces, and groups of epithelioid cells. These reticulated and cellular foci had the immunohistochemical and ultrastructural features of meningothelial tissue.