Sclerotherapy for venous malformations using a "negative subtraction" technique.

We describe a technique of injecting alcohol into venous malformations (VM) under slow digital subtraction angiography (DSA) which eliminates the need to pre-mix the alcohol with a contrast agent. The technique presented here is especially helpful when Metrizamide is not available or not permitted for use. It offers an elegant way to visualize, in real time, the alcohol injected into the VM, while preserving its high concentration and potency as a sclerosing agent.

Anthropometric study of synostotic frontal plagiocephaly: before and after fronto-orbital advancement with correction of nasal angulation.

Surgical correction of synostotic frontal plagiocephaly (unilateral coronal synostosis) focuses on the asymmetry of the forehead and orbits. However, there is controversy regarding whether nasal angulation should be addressed during primary fronto-orbital advancement in infancy. This prospective study was undertaken to answer that question. Preoperative and postoperative anthropometric measurements were obtained for 19 infants with nonsyndromic synostotic frontal plagiocephaly. The measurements included nasal angulation, nasion-to-endocanthion distance, nasion-to-exocanthion distance, and exocanthion-to-tragion distance. All patients underwent bilateral parallelogrammatic fronto-orbital correction. Closing wedge nasal ostectomy was performed for group I (n = 14) and was not performed for group II (n = 5). The average age at the time of follow-up assessments was 3 years 8 months (range, 1 to 14 years) in group I and 5 years 5 months (range, 2 to 15 years) in group II. A statistically significant change was observed for patients who underwent primary correction of nasal angulation; the change correlated with improved naso-orbital symmetry, as judged with nasion-to-endocanthion and nasion-to-exocanthion measurements (p < 0.01 and p < 0.05, respectively). Group I patients exhibited an average preoperative nasal angulation of 9.15 +/- 0.8 degrees that decreased to 3.1 +/- 0.6 degrees postoperatively (p < 0.01). Group II patients exhibited an average preoperative nasal angulation of 6.4 +/- 0.7 degrees that was unchanged postoperatively at 7.2 +/- 1 degrees. The improvement in nasal angulation in group I was particularly striking because the patients in group II exhibited, on average, a lesser degree of preoperative nasal deviation (p < 0.01). This prospective comparison of fronto-orbital correction of synostotic frontal plagiocephaly with and without nasal correction confirmed an earlier study and demonstrated that angulation of the nasal pyramid does not self-correct within 5 years after traditional bilateral fronto-orbital repair. Closing wedge nasal ostectomy results in improved nasal angulation and naso-orbital symmetry, without evidence of distortion or inhibition of nasal growth.

Binderoid complete cleft lip/palate.

A small subset of infants with complete cleft lip/palate look different because they have nasolabiomaxillary hypoplasia and orbital hypotelorism. The authors' purpose was to define the clinical and radiographic features of these patients and to comment on operative management, classification, and terminology. The authors reviewed 695 patients with all forms of incomplete and complete cleft lip/palate and identified 15 patients with nasolabiomaxillary hypoplasia and orbital hypotelorism. All 15 patients had complete labial clefting (5 percent of 320 patients with complete cleft lip/palate), equally divided between bilateral and unilateral forms. The female-to-male ratio was 2:1. Of the seven infants with unilateral complete cleft lip/palate, one had an intact secondary palate and all had a hypoplastic septum, small alar cartilages, narrow basilar columella, underdeveloped contralateral philtral ridge, ill-defined Cupid's bow, thin vermilion-mucosa on both sides of the cleft, and a diminutive premaxilla. Of the eight infants with bilateral complete cleft lip, one had an intact secondary palate. The features were the same as in patients with unilateral cleft, but with a more severely hypoplastic nasal tip, conical columella, tiny prolabium, underdeveloped lateral labial elements, and small/mobile premaxilla. Central midfacial hypoplasia and hypotelorism did not change during childhood and adolescence. Intermedial canthal measurements remained 1.5 SD below normal age-matched controls. Skeletal analysis (mean age, 10 years; range, 4 months to 19 years) documented maxillary retrusion (mean sagittal maxillomandibular discrepancy, 13.7 mm; range, 3 to 17 mm), absent anterior nasal spine, and a class III relationship. The mean sella nasion A point (S-N-A) angle of 74 degrees (range, 65 to 79 degrees) and sella nasion B point (S-N-B) angle of 81 degrees (range, 71 to 90 degrees) were significantly different from age-matched norms ( = 0.0007 and = 0.004, respectively). The ipsilateral central and lateral incisors were absent in all children with unilateral cleft, whereas a single-toothed premaxilla was typically found in the bilateral patients. Several modifications were necessary during primary nasolabial repair because of the diminutive bony and soft-tissue elements. All adolescent patients had Le Fort I maxillary advancement and construction of an adult nasal framework with costochondral or cranial graft. Other often-used procedures were bony augmentation of the anterior maxilla; cartilage grafts to the nasal tip and columella; and dermal grafting to the median tubercle, philtral ridge, and basal columella. Infants with complete unilateral or bilateral cleft lip/palate in association with nasolabiomaxillary hypoplasia and orbital hypotelorism do not belong on the holoprosencephalic spectrum because they have normal head circumference, stature, and intelligence, nor should they be referred to as having Binder anomaly. The authors propose the term cleft lip/palate for these children. Early recognition of this entity is important for counseling parents and because alterations in standard operative methods and orthodontic protocols are necessary.

Resorbable internal splint: an adjunct to primary correction of unilateral cleft lip-nasal deformity.

There is usually some relapse in position of the alar cartilage after primary repair of unilateral cleft lip. Therefore, preoperative or postoperative external splinting has been recommended to supplement either closed or open suspension of the alar cartilage. The authors present a method using a resorbable internal nostril splint to shield the positioned alar cartilage from deformational forces caused by scar, and thus avoiding the problems associated with external splinting. An internal nasal splint was placed in 15 infants during repair of unilateral complete cleft lip and nasal deformity. The nasal morphology was compared with that of 15 control patients who had the same nasolabial procedure without internal splinting. Average follow-up time was 20.4 months (range, 4 to 30 months). Photogrammetric analysis showed that asymmetry of the alar contours averaged 8.6 percent in the splinted patients, as compared with 23 percent for controls (p <0.01). Thus, alar asymmetry was decreased two-thirds in the splinted group. An internal resorbable nasal splint is an adjunct to open alar suspension in primary repair of the unilateral cleft lip nasal deformity. An internal nasal splint protects the corrected alar cartilage longer than an external splint and eliminates drawbacks, such as necrosis, cutaneous depression of the nostril sill, and patient noncompliance. This strategy of temporary internal support of healing cartilage has other applications.