Renato da Silva Freitas

Poland’s Syndrome: Different Clinical Presentations and Surgical Reconstructions in 18 Cases

BackgroundThe literature reports many variations of Poland’s syndrome. This article describes 18 cases of Poland’s syndrome in different stages of treatment, with variable clinical presentations and reconstructive techniques.MethodsThis study evaluated 15 females and 3 males, ages 2 to 43 years, for breast deformity, nipple–areolar complex position, pectoralis muscle malformation, thoracic deformities, and the presence of brachysyndactyly. Surgical treatment was performed for 14 patients, individualized for each case.ResultsFor the women, the hypoplastic breast was treated with a latissimus dorsi muscular flap associated with silicone gel implant in five cases. Two other patients still are receiving tissue expansion for a future muscular and prosthetic reconstruction. Prosthetic implants alone were used on the affected side in four cases. The nipple–areolar complex was reconstructed for two patients. Seven women underwent contralateral breast surgery: reduction mammoplasty in three cases, mastopexy in two cases, and prosthetic implants in two cases. The only man who underwent surgery was treated with endoscopic rotation of the latissimus dorsi muscle flap.ConclusionsThis study demonstrated several breast reconstruction options for patients with Poland’s syndrome, reinforcing the importance of an individualized treatment to achieve complete and adequate rehabilitation.

Human bone morphogenetic protein-2 use for maxillary reconstruction in cleft lip and palate patients.

Background The conventional methods of maxillary alveolar reconstruction in patient with cleft are the periosteoplasty and autologous bone grafting. As an important alternative of bone substitution, there is the recombinant human bone morphogenetic protein-2 (rhBMP-2). This study compares the rhBMP-2 with periosteoplasty and autologous bone grafting. Methods Patients with cleft and alveolar defect were divided into 3 groups of 6 patients who underwent to autologous iliac crest bone grafting, resorbable collagen sponge with rhBMP2, and periosteoplasty, respectively. The analysis was performed through computed tomographic scan preoperatively and at months 3, 6, and 12 postoperatively. The variables analyzed were the alveolar defect volume, formed bone volume, bone formation rate, maxillary height repair rate, and the formed bone density mean. Results The formed bone volume was similar comparing the bone graft and BMP groups at 1-year postoperative analysis (P = 0.58). Both of them had the formed bone volume significantly larger than the periosteoplasty group at 3 and 6 months postoperatively. In this last group, the 1-year follow-up was canceled because the bone formation was insufficient. The bone formation rate, the maxillary height repair rate, and the mean of density of the formed bone were similar in the bone graft and BMP groups at 1-year follow-up with P values of 0.93, 0.90, and 0.81, respectively. Conclusions The amount of formed bone in the periosteoplasty group was insufficient. There was no difference among the bone graft and rhBMP-2 therapy considering the parameters analyzed.

Surgical correction of Tessier number 0 cleft.

The no. 0-14 cleft involves the midline of the face and cranium. It may include both a true and a false median cleft lip, with or without associated hypotelorism or hypertelorism. The no. 0 cleft is the most common of the craniofacial clefts. The objective of this study was to review the functional outcome and aesthetic results of the different techniques applied for each case. We have conducted a retrospective analysis of our series consisting of 32 cases of Tessier no. 0 cleft, in the period between 1997 and 2007. The patients were divided into 2 groups: those with the true median cleft and those with the false median cleft. The clinical findings, lip malformation, alveolar cleft, nasal appearance, septal involvement, associated deformities, and surgical procedures, were all reviewed. Holoprosencephaly was present in 9 cases, with a false median cleft upper lip and an absence of the premaxilla, septum, and columella (only 1 patient underwent lip and columella reconstruction at 2 years of age). Nine patients had an incomplete median cleft lip. Seven of these cases had associated median alveolar cleft, and 1 had an intranasal tumor, associated with lipoma of corpus callosum, characteristic of the Pai syndrome. Six cases of a bifid nose were seen, 2 of which were associated with an alveolar median cleft and hypertelorism. An isolated median alveolar cleft was present in 7 cases, 2 of them associated with a no. 30 cleft. This article presents a large series of Tessier no. 0 cleft, describing the differences between the false and the true median cleft. The surgical procedures may vary in relation to the type of involvement.

Evaluation of molar teeth and buds in patients submitted to mandible distraction: long-term results.

Background: Despite all benefits offered by mandible distraction, complications and long-term consequences need to be evaluated to define its safety and morbidity. Forty mandible distractions were studied. Panoramic mandible radiographs obtained preoperatively, during distraction, and during the postoperative period were reviewed, with the intention of evaluating development and complications of molar buds and teeth in the distraction area. Methods: The mean patient age was 8.1 years. Twenty-five patients had craniofacial microsomia (one associated with a no. 10 facial cleft), five had temporomandibular joint ankylosis, two had familiar cases of auriculocondylar syndrome, one had a Tessier no. 30 facial cleft, and one had Treacher Collins syndrome. The severity of mandible hypoplasia was Pruzansky grade I in four cases, grade IIA in eight cases, grade IIB in 16 cases, and grade III in one case. Mean radiographic follow-up was 44.8 months. Results: Molar buds located in the distraction area erupted without any deformity or displacement in 18 sides (45 percent). Fourteen cases presented distalization of a dental bud to a superior position in the mandibular ramus (four migrated back to the original position). Six molar buds presented perforations, four had shape deformities (two caused by dental fracture), and two had dental root injuries followed by root absorption lately. One case developed a dentigerous cyst. Conclusions: Almost half of the patients did not have any molar bud or tooth alterations after mandible distraction, and more than 20 percent presented only bud distalization. Therefore, preventive bud enucleation or tooth extraction should be avoided before mandible distraction.

The Tessier Number 5 Facial Cleft : Surgical Strategies and Outcomes in Six Patients

The Tessier no. 5 facial cleft is an extremely rare congenital malformation. Only 26 cases have been described in the English-language literature. The cleft begins in the upper lip just medial to the oral commissure, extending across the cheek as a groove ending at the junction of the middle and lateral thirds of the lower eyelid. The bone involvement usually includes an alveolar cleft in the premolar region, extends across the maxilla lateral to the infraorbital nerve, up to the infraorbital rim and orbital floor. The goals of the surgical procedure include reconstructing the lower eyelid, repositioning the lateral canthus, closure of the labiomaxillary cleft, and restoration of the skeletal continuity (including the orbital floor defect) with bone grafts. We present six patients with the Tessier no. 5 facial cleft who have been treated in our combined centers and discuss the surgical options and difficulties faced in the reconstruction of this rare and challenging craniofacial malformation. To date, we have treated six patients (two with bilateral and four with unilateral clefts). Three of the patients with unilateral clefting had an associated no. 4 cleft and one patient with a bilateral cleft had an associated no. 3 cleft. This paper represents the largest series to date documenting surgery for patients with the Tessier no. 5 facial cleft.

Tessier no. 4 facial cleft: evolution of surgical treatment in a large series of patients.

Background: Tessier no. 4 facial cleft is a rare, complex, and challenging craniofacial malformation. The present article aims to describe different clinical features evidenced in 21 cases of this malformation, discussing a 20-year experience with and evolution of its surgical treatment. Methods: Some demographic data, clinical features, and reconstructive results were evaluated retrospectively. These patients have been evaluated and treated in three specialized Brazilian craniofacial centers. Nineteen were already operated on, with a mean follow-up of 3.5 years (range, 1 to 20 years). Results: Sex distribution showed a male prevalence (2:1). The average age of initial treatment was 5.4 years. Four cases were affected on the right side of the face, seven on the left, and 10 bilaterally. Six patients had other rare associated facial clefts, including nos. 5 (three patients), 7, 9, and 10. Cleft upper lip was evidenced in all patients, and maxillary hypoplasia was present in five and maxilla cleft in eight. Lower eyelid coloboma was seen in almost every case (19 patients); 10 of these had medial canthus dystopia. Four patients had amniotic bands in the limbs. Surgical repair was individualized to each patient. Surgical experience gained with these patients allowed the authors to develop some technical modifications, which have improved aesthetic results, camouflaging scars into natural folds and anatomical units, without compromising functional outcomes. Conclusions: The great majority of Tessier no. 4 facial clefts can be appropriately treated using local flaps. Classic techniques are extremely useful, but long-term results could be improved if the technical modifications described were adopted.

Oral-nasal-ocular Cleft: The Greatest Challenge Among the Rare Clefts

Introduction:Number 3 cleft or oral-nasal-ocular cleft is a well-known entity that was described by Morian over a century ago. This malformation is a paranasal-medial orbitomaxillary cleft running across the lacrimal segment of the lower eyelid and over the lacrimal groove. The Tessier number 3 naso-ocular cleft represents one of the most difficult and challenging malformations to correct for the reconstructive surgeon. We have conducted a retrospective analysis of our series consisting of 21 cases. Objective:The objective was to review the functional outcome and aesthetic results of the different techniques applied for each case. Materials and Methods:From 1997 to 2007, 21 patients with a Tessier number 3 cleft were treated in our craniofacial units. The clinical findings, tomographic studies, and surgical procedures were reviewed and analyzed. We have discussed our protocol of the treatment. Results:We have treated facial malformation in 2 craniofacial centers. Fourteen patients were evaluated in the first year of their life, with an average age at presentation of 3 years. Twelve patients were female, and 9 were male; 6 patients had amniotic bands in limbs, 5 patients had an association with Tessier number 11 cleft, 3 patients with number 9 cleft, and 1 with number 7 cleft. Related to cleft lip, 10 patients had bilateral cleft lip, and 8 patients had unilateral cleft lip. Three patients did not have any involvement of the upper lip. The alar base was deviated upward in 19 patients, 11 cases had severe anatomic alteration with the lateral border of the ala above the medial canthus, and 8 cases had a mild dislocation. Nine cases of lacrimal duct obstruction and 8 cases of lacrimal duct extrophy were identified. Twelve patients had a lower eyelid coloboma of varying grades, and there were 2 cases of microblepharia. Aiming the soft tissue reconstruction, eyelid, nose, and upper lip were evaluated regarding their position, absence of tissue, and position of medial canthus and ala. Twelve of our patients underwent correction in the same moment, their medial canthus rotated upward and the ala downward, using the contralateral side as the reference. The lip was treated using a Millard-like technique. Neo-conjunctivorhinostomy was performed in the same moment in 2 patients or later in 1 case. Four patients had plagiocephaly due to the cranial involvement, and they were submitted to cranioplasty. Three had neurosurgical approach and advancement of the frontal bandeau. One adult patient received an acrylic plate to reshape the frontal area. Conclusions:Tessier number 3 cleft is one of the most difficult and challenging malformations to correct for the reconstructive surgeon. Besides the difficulties of its treatment, patients with Tessier number 3 cleft may achieve good results when the team has good skills.

Familial lambdoid craniosynostosis between father and son.

Lambdoid craniosynostosis is an uncommon condition, with an incidence of 1 per 33,000 live births. Its etiopathology remains controversial. Although many forms of syndromic and nonsyndromic craniosynostosis demonstrate an inherited pattern, few articles have reported lambdoid craniosynostosis in the same family. In this article, we report lambdoid synostosis in a father and son. A case review is performed. A full-term male infant was delivered by cesarean delivery because of failure to progress. He presented at 3 years of age with right unilateral lambdoid craniosynostosis with facial asymmetry and lateral deviation of his jaw, with occlusal abnormality. At presentation, the father reported skull surgery during his infancy for unilateral lambdoid craniosynostosis. Review of the computed tomographic scan of the child demonstrated a plagiocephalic appearance of the calvarium with frontal bossing and a fusion of the lambdoid suture on the right sides. The brain parenchyma showed no abnormality. The review of his fathers surgical record from 33 years ago and of his computed tomographic scan ordered by our team confirmed the diagnosis of previous lambdoid craniosynostosis. Only 2 previous cases of familial isolated lambdoid craniosynostosis have been previously described in literature. Although the genetic basis has been established for many types of craniosynostosis, the etiopathogenesis of isolated lambdoid synostosis has not yet been established. We report the third case of inherited unilateral lambdoid craniosynostosis. The genetic pathogenesis of lambdoid craniosynostosis will be discussed.

Blindness as a complication of monobloc frontofacial advancement with distraction.

Abstract The monobloc frontofacial osteotomy provides aesthetic and functional improvement in the treatment of various craniofacial deformities. This procedure, through highly complex, has had some significant associated complication, such as cerebrospinal fluid leakage, hematoma, infection, and bone resorption. Distraction has been successfully used to gradually elongate bone and soft tissue. This method seems to provide improved results over conventional surgery, with less morbidity. We present a case of a patient with Apert syndrome who underwent monobloc advancement using the Rigid External Device (RED) device and who developed a transient bilateral amaurosis on the fourth postoperative day before distraction. A second procedure was performed to push back the frontal bandeau, maintaining the device in position. The blindness was resolved with this procedure as well as treatment with systemic steroids. The distraction was started thereafter, and the desired improvement was acquired. To our knowledge, this is the first case of transient bilateral amaurosis in a patient undergoing monobloc distraction.

Autologous Fat Grafting for Treatment of Breast Implant Capsular Contracture: A Study in Pigs

BACKGROUND Capsular contracture (CC) is a common complication after breast augmentation. Autologous fat grafting may be effective for restoring tissue vascularization and function. OBJECTIVE The authors evaluated the efficacy of autologous fat grafting in a porcine model as a treatment for CC after breast augmentation. METHODS This prospective study was performed in 20 female 30-day-old pigs. Each animal was implanted with three 30-cc textured silicone implants (stage 1 of the experiment). Group A served as the untreated control group. To induce CC, 2 mL of autologous fibrin glue was applied to the pericapsular space in group B and C animals at implantation. Three months after implantation (stage 2), the CCs of all groups were assessed by Baker classification and applanation tonometry (AT). Liposuction was performed in group B to harvest fat for these animals. Three months after group B underwent fat grafting, all 3 groups were reevaluated. Reassessments included Baker classification, AT, histologic analysis, and tensiometry (stage 3). RESULTS The deposition of mature and immature collagen was similar for the 3 groups. The amount of fat remaining around the implanted capsules did not differ significantly between the groups. At stage 3, group B exhibited significantly larger tonometry areas than did group C. The CCs in groups B and C were significantly thicker than those of group A, but the difference between groups B and C was not significant. Capsule rupture forces did not differ significantly between groups A and B but were significantly higher in group C compared with the other groups. CONCLUSIONS Results in this animal model indicate that pericapsular lipoinjection may be a promising treatment for CC in humans.