Youssef Tahiri

A critical evaluation of long-term aesthetic outcomes of fronto-orbital advancement and cranial vault remodeling in nonsyndromic unicoronal craniosynostosis.

Background: This study reports long-term aesthetic outcomes with fronto-orbital advancement and cranial vault remodeling in treating unicoronal synostosis over a 35-year period. Methods: Retrospective review was performed on patients with isolated unicoronal synostosis from 1977 to 2012. Demographic, preoperative phenotypic, and long-term aesthetic outcomes data were analyzed with chi-squared and Fisher’s exact test for categorical data and Wilcoxon rank-sum and Kruskal-Wallis rank for continuous data. Results: A total of 238 patients were treated; 207 met inclusion criteria. None underwent secondary intervention for intracranial pressure. At definitive intervention, there 96 (55 percent) Whitaker class I patients, 11 (6 percent) class II, 62 (35 percent) class III, and six (3 percent) class IV. Nasal root deviation and occipital bossing each conferred an increased risk of Whitaker class III/IV [OR, 4.4 (1.4 to 13.9), p = 0.011; OR, 2.6 (1.0 to 6.8), p = 0.049]. Patients who underwent bilateral cranial vault remodeling with extended unilateral bandeau were less likely Whitaker class III/IV at latest follow-up compared with those undergoing strictly unilateral procedures [OR, 0.2 (0.1 to 0.7), p = 0.011]. Overcorrection resulted in decreased risk of temporal hollowing [OR, 0.3 (0.1 to 1.0), p = 0.05]. Patients with 5 years or more of follow-up were more likely to develop supraorbital retrusion [OR, 7.2 (2.2 to 23.4), p = 0.001] and temporal hollowing [OR, 3.7 (1.5 to 9.6), p = 0.006] and have Whitaker class III/IV outcomes [OR, 4.9 (1.8 to 12.8), p = 0.001]. Conclusion: Traditional fronto-orbital advancement and cranial vault remodeling appears to mitigate risk of intracranial pressure but may lead to aesthetic shortcomings as patients mature, namely fronto-orbital retrusion and temporal hollowing. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Clarifying the relationships among the different features of the OMENS+ classification in craniofacial microsomia.

Background: The OMENS+ classification is commonly used to describe the phenotypically diverse craniofacial features of craniofacial microsomia. The purpose of this study was to evaluate associations among the individual components of the OMENS+ criteria. Methods: An institutional review board–approved retrospective chart review was performed for patients who presented with a diagnosis of unilateral or bilateral craniofacial microsomia to the craniofacial clinic from January of 1990 to December of 2012. Demographic, diagnosis, classification, treatment, and radiographic data were abstracted for all patients who met inclusion criteria. Associations and correlations were evaluated using the Spearman rank test and a logistic regression model. Results: One hundred five patients (61 male and 44 female) with craniofacial microsomia met inclusion criteria. Eighty-one patients (77.1 percent) had unilateral microsomia and 24 (22.9 percent) had bilateral microsomia. Twenty-eight patients (26.7 percent) had macrostomia. Correlations were all significantly interrelated (p = 0.000 to p = 00.018) between the degree of orbital, mandibular, and soft-tissue deformities. Moreover, the severity of ear deformity and facial nerve involvement were also significantly correlated (p = 0.008). Between these two groupings, there was a significant correlation between soft-tissue deficiency and nerve involvement (p = 0.010). Macrostomia was associated with the individual components of the group orbit (p = 0.008), mandible (p = 0.000), and soft tissue (p = 0.005). Conclusions: The association between structures using the OMENS+ classification may be caused by their branchial arch origin. Structures mainly developed from the first branchial arch (orbit, mandible, and soft tissue) are associated in degree of severity, as are the structures mainly derived from the second branchial arch (facial nerve and ear). CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

Maxillary involvement in hemifacial microsomia: an objective three-dimensional analysis of the craniofacial skeleton.

Abstract The aim of our study is to better understand the maxillary involvement in relation to the mandibular deformation in hemifacial microsomia (HFM). To do so, consecutive patients with HFM treated at The Children’s Hospital of Philadelphia from 2000 to 2012 were included in our study. Both two-dimensional and three-dimensional analyses of the bony and sinus structures of the midface and mandible were performed using three-dimensional segmentation software. Patients were stratified into groups based on the Kaban-Pruzansky classification—mild (0–1), moderate (2A), and severe (2B–3)—as well as rank ordering based on overall severity. Analyses involved paired t-tests within severity groups, 1-way analysis of variance when assessing across groups (ipsilateral/contralateral ratio), and regression to assess for trends. Thirty patients were included (4 mild, 12 moderate, and 14 severe). The mandibular volume ratio differed across all patient groups (P < 0.001) and trended with rank order (P < 0.001). No significant difference in maxillary bony volume ratio was found across all patient groups (P = 0.16). In patients with severe disease, the maxillary bone volume was found to be significantly decreased on the ipsilateral side as compared with the contralateral side (P = 0.0123). There was no difference in maxillary sinus volume between ipsilateral and contralateral sides within any patient groups or in comparing across groups (P = 0.10). No significant trend was found in the volume ratio of mandible and maxilla (P = 0.41). To conclude, the maxillary sinus seems to show no difference in volume when comparing between laterality and severity groupings. These findings suggest that there may be alternative influences other than the vascular insult acting as the driving force behind the mandibular deformity and the additional classic clinical findings of HFM.

New Pattern of Sutural Synostosis Associated With TWIST Gene Mutation and Saethre-Chotzen Syndrome: Peace Sign Synostosis.

AbstractThe authors present a new and unique pattern of sutural fusion “peace sign synostosis” (PSS) characterized by synostosis of the metopic, bicoronal, and sagittal sutures and associated with abnormalities of the TWIST1 gene known to be associated with Saethre-Chotzen syndrome (SCS). To do so, we performed a retrospective review of patients with bicoronal, metopic, and at least partial anterior sagittal synostoses at the Childrens Hospital of Philadelphia and Seattle Childrens Hospital. Patients’ demographics, genetic analysis, perioperative and clinic notes were reviewed. Five patients were identified with PSS and abnormalities of TWIST1 consistent with SCS. One patient, with the longest follow-up of 7 years, underwent 5 intracranial procedures and required a ventriculoperitoneal (VP) shunt. The remaining 4 patients underwent posterior cranial vault distraction as the initial procedure, followed by anterior cranial vault remodeling. Two patients required a VP shunt. To conclude, synostosis of the metopic, bicoronal, and sagittal sutures (PSS) appears to be associated with SCS and produces a characteristic skull morphology that can be readily identified on physical examination. Early data suggest a high rate of reoperation, increased necessity for a VP shunt, and potential complications. Of note, this novel phenotype had not been previously observed at our respective institutions, reported in the literature, or observed in association with TWIST1 abnormalities as described in association with SCS.

Simultaneous unicoronal and sagittal distraction osteogenesis for the treatment of nonsyndromic multisutural craniosynostosis.

AbstractWe present a case of multiplanar distraction osteogenesis for the simultaneous treatment of sagittal and unicoronal craniosynostosis in a nonsyndromic 2-month-old boy. Unilateral fronto-orbital advancement and sagittal suturectomy were performed. Distracters were fixed orthogonally in the sagittal and coronal positions to distract the affected coronal and sagittal sutures. The devices achieved 20 and 22 mm of advancement in the coronal and sagittal locations. A total intracranial volume increase of 62% was noted at 6 months’ follow-up. This preliminary report demonstrates the procedure’s short-term safety; future investigation is needed over the long term to determine its efficacy.

Myofascial Closure of Intradural Inclusion Cysts following in utero Myelomeningocele Repair.

Myelomeningocele is one of the most common congenital malformations. A randomized controlled trial, known as the Management of Myelomeningocele Study (MOMS), demonstrated that closure during the fetal period can be performed relatively safely and be of significant benefit to patients. However, postnatally, patients can develop resultant symptoms from a tethered cord and inclusion cysts; this often requires surgical treatment. Repeat surgery in this population can be challenging due to the age of the patients, the extent of surgical exposure needed and the need for resection of dermal and epidermal tissues in the midline. We describe our approach for closure of these complex defects using lateral fasciocutaneous flaps with relaxing incisions made in the posterior axillary line, in order to minimize tension and maximize soft tissue coverage of the midline.

Analysis of the 50 most cited papers in craniofacial surgery

The intent of this study is to discuss the most prominent literature in craniofacial surgery. To do so, using the ISI Web of Science, a ranking by average number of citations per year of the top 50 craniofacial surgery articles was compiled. All plastic surgery journals listed in the Surgery category in the ISI Web of Knowledge Journal Citation Reports 2013 Science Edition were considered. Journal of publication, country of origin, collaborating institutions, topic of interest, and level of evidence were analyzed. The total number of citations ranged from 47 to 1017. Average number of citations per year ranged from 46.2 to 8.6. The oldest article in the top 50 was published in 1988 and the most recent in 2009. The majority of the articles came from Plastic and Reconstructive Surgery with 28 of the 50. The majority of the articles, originated from the United States (56%). Reconstruction of acquired defects was the most commonly examined topic at 46.2%; followed by articles discussing reconstruction of congenital defects (23.1%). The most common level of evidence was level 3. This extensive examination of the craniofacial literature highlights the important part that craniofacial surgery takes in the field of plastic surgery.

Ophthalmic considerations in patients with Pfeiffer syndrome

Purpose We report here a newborn male infant with striking features consistent with severe Pfeiffer syndrome type II, including cloverleaf skull deformity with pansynostosis, extreme proptosis, upper extremity contractures, broad big toes and thumbs with varus deviation and genetic mutation in the FGFR2 gene. The authors review the ophthalmic complications in Pfeiffer syndrome and discuss the unique surgical strategies used for obtaining adequate corneal coverage in these unique patients. Observations Ophthalmic considerations in Type 2 Pfeiffer Syndrome include vision loss secondary to increased intracranial pressure, and extreme proptosis as a result of orbitostenosis and midfacial retrusion. Our patient has undergone multiple ophthalmic/oculoplastic, neurosurgical, and midfacial surgeries as a result of corneal deterioration due to extreme exorbitism. Conclusions and importance It is important for ophthalmologists to be aware of the ophthalmic complications associated with patients with craniosynostosis syndromes. Our case identifies the importance of close communication between ophthalmology and plastic reconstructive surgery to help formulate the most successful plan in treating corneal decompensation and proptosis in Pfeiffer Syndrome patients.

Fat injection to correct contour deformities of the reconstructed breast: a single surgeon experience

Aim: Autologous fat grafting has gained acceptance as a technique to improve aesthetic outcomes in breast reconstruction. The purpose of this study was to share our clinical experience using autologous fat injection to correct contour deformities during breast reconstruction. Methods: A single surgeon, prospectively maintained database of patients who underwent autologous fat injection during breast reconstruction from January 2008 to November 2013 at McGill University Health Center was reviewed. Patient characteristics, breast history, type of breast reconstruction, volume of fat injected, and complications were analyzed. Results: One hundred and twenty-four patients benefited from autologous fat injection from January 2008 to November 2013, for a total of 187 treated breasts. The patients were on average 49.3 years old (u0381 8.9 years). Fat was harvested from the medial thighs (20.5%), flanks (39.1%), medial thighs and flanks (2.9%), trochanters (13.3%), medial knees (2.7%), and abdomen (21.9%). An average of 49.25 mL of fat was injected into each reconstructed breast. A total of 187 breasts in 124 patients were lipo-infiltrated during the second stage of breast reconstruction. Thirteen breasts (in 12 separate patients) were injected several years after having undergone lumpectomy and radiotherapy. Of the 187 treated breasts, 118 were reconstructed with expanders to implants, 45 with deep inferior epigastric perforator flaps, 9 with latissimus dorsi flaps with implants, 4 with transverse rectus abdominis myocutaneous flaps, and 13 had previously undergone lumpectomy and radiotherapy. Six complications were noted in the entire series, for a rate of 3.2%. All were in previously radiated breasts. Average follow-up time was 12 months (range: 2-36 months). Conclusion: Fat injection continues to grow in popularity as an adjunct to breast reconstruction. Our experience demonstrates a low complication rate as compared to most surgical interventions of the breast and further supports its safety in breast reconstruction. However, caution should be used when treating previously radiated breasts.