Rare Congenital Upper Lip Pit.

Congenital lip pits are characterized by sinuses or fistulas in the lips that can occur in isolation or as part of a genetic disorder. A 6-year-old girl with a right upper lip lesion present at birth presented with recurrent swelling and occasional erythema. Examination revealed a mildly swollen punctum at the right upper wet/dry vermillion with expressible serous drainage. There were no other phenotypic or cognitive concerns. The lesion was surgically excised using vertical wedge resection. The postoperative course showed well well-healed incision. The pathology report confirmed a lip pit. The family was referred to genetics for further evaluation. Van der Woude syndrome (VWS) is a genetic disorder associated with abnormal development of the paramedian lip. Most congenital lip pits are primarily found on the lower lips, with paramedian lip pits being the most common. Upper lateral lip pits with or without accompanying lip pits are considerably rarer. Though VWS is commonly associated with mutations in the interferon regulatory factor 6 or grainyhead-like protein 3 genes, ~25% of affected individuals lack an identified genetic etiology. A high index of suspicion for VWS is warranted if lip pits are present in the absence of other phenotypic abnormalities and should prompt genetic testing for interferon regulatory factor 6 and grainyhead-like protein 3 mutations. Multidisciplinary teams should consider patient self-esteem, quality of life, and potential family planning when deciding on surgical intervention for lip pits. Surgical management of pits should entail tissue-preserving techniques such as vertical wedge resection and inverted T-lip reduction to prevent whistle-lip deformity.

Three-Dimensional Computed Tomography Scan Analysis of Secondary Labial Deformities in Patients With Unilateral Cleft Lip/Cleft Palate After Revisional Surgery.

BACKGROUND: Surgical reconstruction of secondary labial deformities associated with isolated unilateral cleft lip (UCL) and/or UCL and palate (UCLP) is challenging. There have been few studies in the literature looking at labial soft tissues quantitatively to assess surgical results. OBJECTIVE: To apply a novel computer-aided, 3-dimensional reconstruction technique based on CT scan images to conduct quantitative preoperative and postoperative assessments in patients with UCL/UCLP undergoing surgical revision of secondary labial deformities. METHODS: Preoperative and postoperative spiral computed tomographic (CT) scans of the face were performed in 21 randomly selected UCL or UCLP patients, who underwent secondary lip revision surgery. The data was then imported to the SimPlant 11.04 software system. Fixed point-to-point, linear distance, and angles were measured, statistically analyzed and used to assess the effect of the surgery. RESULTS: Preoperative measurements showed that the thickness of the upper vermilion at the apex of the Cupid's bow on the affected side was greater than that on the unaffected side. The distance from the apex of the Cupid's bow to the ipsilateral subnasal point of the affected side was smaller than that of the unaffected side (P < 0.05). After surgery, the subjects were rescanned at an average of 9 months, and the curative effects were evaluated. The statistically significant preoperative differences between the affected and unaffected sides were not found postoperatively indicating surgical success. CONCLUSIONS: This study demonstrates the utility of a novel method to measure and assess results in the surgical revision of UCL/UCLP patients with secondary lip deformities. This knowledge can aid the surgeon in selection of treatment techniques.

Correction of sagittal synostosis using a modified Pi method.

The authors' technique for the treatment of sagittal synostosis, which involves total cranial vault reconstruction and is termed the Pi procedure, is a safe, effective method for correcting the deformities associated with sagittal synostosis. It provides an immediate correction that does not require any further manipulations to the skull, such as a molding helmet. The technique addresses all the aspects of the deformity. It increases the width as well as the central height of the skull and decreases the length of the skull to produce a rounder cranial vault.