The purpose of this study was to analyze the geometry of the primary cleft lip nasal deformity using three-dimensional computerized tomography in a group of 3-month-old infants with complete unilateral cleft lip and palate before surgical intervention. Coordinates and axes were reconfigured after the three-dimensional image was oriented into neutral position (Frankfurt horizontal, true anteroposterior, and vertical midline). Display and measurement of skin surface and osseous tissues were achieved by adjusting the computed tomographic thresholds. S-N, N-ANS, S-N-O, and S-N-ANS were measured from true lateral views. Biorbital (LO-LO), interorbital (MO-MO), intercanthal (en-en), and nasal (al-al) widths were measured from the anteroposterior view. The bony alveolar cleft width was measured from the inferior view. The study group was divided into two groups on the basis of skeletal alveolar cleft width: six patients with clefts narrower than 10 mm and six patients with clefts wider than 10 mm. Only the S-N-ANS angle differed between the two groups, i.e., it was greater in the group with the wider clefts (p < 0.05). Coordinates of six landmarks at the base of the nose [sellion (se), subnasale (sn), cleft-side and noncleft-side subalare (sbal-cl and sbal-ncl), and the most posterior point on the lateral piriform margins (PPA-CL and PPA-NCL)] were obtained for analysis of the nasal deformity. On average, the subnasale point was anterior to sellion and deviated to the noncleft side; the cleft-side sbal point was more medial, posterior, and inferior than the noncleft-side sbal point; and the PPA point on the cleft-side piriform margin was more lateral, posterior, and inferior than the PPA point on the noncleft side. These discrepancies were not universally observed. However, in all patients, four findings were observed without exception (p < 0.01): (1) subnasale (sn) was deviated to the noncleft side (mean distance from midline, 5.0 mm; range, 2 to 9.5 mm), (2) the cleft-side alar base (sbal-cl) was more posterior than the noncleft-side alar base (sbal-ncl) (mean difference, 3.6 mm; range, 1 to 5.5 mm), (3) the noncleft-side alar base (sbal-ncl) was further from the midline than the cleft-side alar base (sbal-cl) (mean difference in lateral distances of sbal-ncl and sbal-cl from the midline, 2.8 mm; range, 0.5 to 7 mm), and (4) the cleft-side piriform margin (PPA-CL) was more posterior than the noncleft side piriform margin (PPA-NCL) (mean difference, 2.1 mm; range, 0.5 to 4 mm). In conclusion, the nasal deformity in unilateral cleft lip and palate that has not been operated on is characterized by these four features and increased S-N-ANS angle with increased alveolar cleft width.

The Le Fort I, split-palate approach provides intraoral surgical access to a region of the midline skull base ranging from the upper clivus to the second cervical vertebra. Although this approach provides perhaps the largest exposure of all the intraoral techniques, there is little concerning it in the literature. Furthermore, there are no detailed descriptions of case histories, complications, and outcome. The purpose of this study was to evaluate this procedure's effectiveness and identify associated complications as well as outcome. Seven cases of patients who underwent eight skull base surgeries using the Le Fort I, split-palate approach were evaluated retrospectively. Particular attention was paid to postoperative occlusion, speech, mouth opening, infection, tumor recurrence, postoperative recovery period, and viability of maxillary bone and teeth. Follow-up ranged from 4 months to 7 years with a mean of 3.9 years. Pathologic diagnoses included three chordomas (two recurrent), one recurrent meningioma, one liposarcoma, one chondrosarcoma, and one inflammatory mass. One patient with chordoma underwent a second operation using the same approach. No deaths or major neurologic problems related to the procedure occurred. One patient who had known local metastases at the time of operation died several months after surgery. All other patients are still living. Duration of hospital stay ranged from 5 to 53 days with a mean of 25.4 days. Postoperative complications included one case of meningitis with an associated cerebrospinal fluid leak, three cases of malocclusion, one case of velopharyngeal insufficiency, and one extracranial soft-tissue infection. The case of meningitis was successfully treated by antibiotics. The malocclusions were corrected by conservative treatment. No problems with mouth opening or bone or tooth viability occurred. Tumor recurred in both cases in which malignancy was involved, whereas only one recurrence was noted among the benign cases. It is concluded that the Le Fort I-palatal split technique is a relatively safe and effective means for approaching midline skull base tumors. Several modifications to the surgical protocol and surgical technique are detailed herein.

Infantile myofibromatosis is a mesenchymal disorder characterized by the fibrous proliferation of the skin, bone, muscle and viscera. It is the most common fibrous tumor in childhood. We present a newborn with skin and bone disease without visceral involvement, who showed good response to vinblastine and methotrexate. Clinical features, etiology, diagnosis, and treatment are reviewed.