Unilateral Coronal Craniosynostosis in an Apert-Like Patient.

BACKGROUND AND SIGNIFICANCE: Apert syndrome is a congenital disorder of patients who typically present with bilateral coronal craniosynostosis and varying degrees of complex syndactyly of the hands and feet, among other features. We describe a unique presentation of a rare Apert-like patient with unilateral coronal craniosynostosis and complex syndactyly of the hands and feet. CASE REPORT: A 2-year-old male patient presented to the craniofacial clinic with his mother due to a concerning head shape. The patient also had bilateral syndactyly of the hands and feet and underwent prior surgical release of the third web space. Computerized tomography of the head illustrated a small open anterior fontanelle, a left harlequin orbit, complete left coronal craniosynostosis, and a patent right coronal suture. The patient subsequently underwent fronto-orbital advancement for expansion of the cranial vault and correction of the asymmetric forehead and orbit. The procedure resulted in improvement of his deformity. CONCLUSION: This case illustrates a unique presentation of an acrocephalosyndactyly (ACS) syndrome with asymmetric, unilateral coronal craniosynostosis and complete complex syndactyly of the hands and feet that is most consistent with Apert syndrome. Although the majority of patients with ACS can be categorized into known syndromes, other more unusual presentations must still be considered. Such unique cases are exceedingly rare and only through additional reporting and review of unique phenotypes can new subtypes of common ACS syndromes be classified.

HISTORIQUE ET SIGNIFICATION: Le syndrome d'Apert est un trouble congénital chez les patients qui, entre autres, ont généralement une craniosynostose coronale bilatérale et divers degrés de syndactylie complexe des mains et des pieds. Les auteurs décrivent la présentation unique d'un patient ayant un rare pseudosyndrome d'Apert qui se manifestait par une craniosynostose coronale unilatérale et une syndactylie complexe des mains et des pieds. RAPPORT DE CAS: Un garçon de deux ans a consulté à la clinique craniofaciale en compagnie de sa mère parce que la forme de sa tête était préoccupante. Il présentait également une syndactylie bilatérale des mains et des pieds et avait déjà subi une libération du troisième espace interdigital. La tomodensitométrie de la tête a révélé une petite fontanelle antérieure ouverte, un œil gauche méphistophélique, une craniosynostose coronale gauche complète et une suture coronale droite ouverte. Le patient a ensuite subi un avancement fronto-orbitaire pour élargir la voûte crânienne et corriger le front asymétrique et l'orbite. L'intervention a atténué ses malformations. CONCLUSION: Ce cas démontre une présentation unique de syndrome d'acrocéphalosyndactylie (ACS) avec craniosynostose coronale unilatérale asymétrique et syndactylie complexe complète des mains et des pieds très évocatrices d'un syndrome d'Apert. Même si la majorité des patients ayant une ACS peuvent être classés dans des syndromes connus, il faut tout de même envisager d'autres présentations plus inhabituelles. Ces cas uniques sont d'une extrême rareté, et ce n'est que par de nouveaux signalements et par l'analyse de phénotypes uniques qu'on pourra classer de nouveaux sous-types de syndromes d'ACS courants.

The Cartilage Warp Prevention Suture.

BACKGROUND: Costal cartilage graft warping can challenge rhinoplasty surgeons and compromise outcomes. We propose a technique, the "warp control suture," for eliminating cartilage warp and examine outcomes in a pilot group. METHODS: The warp control suture is performed in the following manner: Harvested cartilage is cut to the desired shape and immersed in saline to induce warping. A 4-0 or 5-0 PDS suture, depending the thickness of the cartilage, is passed from convex to concave then concave to convex side several times about 5-6 mm apart, finally tying the suture on the convex side with sufficient tension to straighten the cartilage. First an ex vivo experiment was performed in 10 specimens from 10 different patients. Excess cartilage was sutured and returned to saline for a minimum of 15 min and then assessed for warping compared to cartilage cut in the identical shape also soaked in saline. Then, charts of nine subsequent patients who received the warp control suture on 16 cartilage grafts by the senior author (BG) were retrospectively reviewed. Inclusion of study subjects required at least 6 months of follow-up with standard rhinoplasty photographs. Postoperative complications and evidence of warping were recorded. RESULTS: In the ex vivo experiment, none of the 10 segments demonstrated warping after replacement in saline, whereas all the matching segments demonstrated significant additional warping. Clinically, no postoperative warping was observed in any of the nine patients at least 6 months postoperatively. One case of minor infection was observed in an area away from the graft and treated with antibiotics. No warping or other complications were noted. CONCLUSION: The warp control suture technique presented here effectively straightens warped cartilage graft and prevents additional warping. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Effects of unilateral vertical mandibular distraction osteogenesis on airway anatomy in children with hemifacial microsomia.

PURPOSE: To assess the two- and three-dimensional airway changes following unilateral vertical mandibular distraction osteogenesis (vMDO) in patients with hemifacial microsomia (HFM). METHODS: A retrospective evaluation was performed for consecutive patients over an 18-month period with HFM and with Kaban-Pruzansky Type-II mandibular deformities who underwent unilateral vMDO for correction of vertical mandibular asymmetry. Patients with complete records and a minimum of 12 months of clinical follow-up post-consolidation were included. Pre-operative airway dimensions (diameters, cross-sectional areas, and volumes) were measured for the oropharynx and nasopharynx. Pre-operative airway dimensions were compared to post-distraction measurements taken from 3D-CT data obtained at a minimum of 3 months post-consolidation. RESULTS: Five patients met inclusion criteria. Median age was 12.6 years, 3 were female, and median distraction length was 21.3 mm. Median final follow-up CT was performed 5 months after completion of distraction. There were no major or minor complications. Median total airway volume change was +33.7%, corresponding to median volume changes of 80.5% and 10.5% for the oropharyngeal and nasopharyngeal airways, respectively. CONCLUSION: Unilateral vMDO expands the minimum diameter and volume of the oropharyngeal to a greater extent than the nasopharyngeal airway in HFM patients with Kaban-Pruzansky Type-II mandibular deformities.

Leaving the Skin Incision Open May Not Be as Beneficial as We Have Been Taught.

BACKGROUND: Currently, various methods of skin closures are used in contaminated and dirty abdominal wounds without solid, evidence-based guidance. This study investigates whether closure methods affect surgical site infection (SSI) and other incisional complications. We hypothesize that open management of the skin would have the lowest complications, including SSI. PATIENTS AND METHODS: Patients age ≥18 who underwent trauma laparotomy (TL) or damage control laparotomy (DCL) from 2008-2013 and had class III/IV wounds were included. Demographic, injury, treatment, and outcome variables were compared based on skin closure methods: Primary closure, intermittently stapled with wicks, or open management. Subgroup analyses for TL, DCL, and high-risk patients with stomach, colon, or rectal injuries were performed. Bivariable and multivariable logistic regression (MLR) analyses were performed to identify risk factors for superficial/deep SSI and surgical incision complications. RESULTS: A total of 348 patients were included. The median age was 47 years; 14% were female; 21% had blunt injuries. Overall SSI was highest for open incisions (p < 0.05), but there was no difference in superficial/deep SSI. Primary closures healed a median of 20 days, compared with 68 and 71 days for the intermittently stapled and open groups, respectively (p < 0.001). Primary closure in TL and high-risk patients also had the lowest SSI rates (all p < 0.05), but there were no differences in superficial/deep SSI in any subgroup. In TL patients, diabetes mellitus and colon injuries were independently associated with the development of superficial/deep SSI and surgical incision complications; however, skin closure method was not. CONCLUSION: In class III and IV wounds, primary closure was associated with the lowest SSI, shortest length of stay and healing time. Method of skin closure, however, did not have an independent effect on the development of superficial/deep SSI or surgical incision complications. These suggest that primary skin closure in contaminated and dirty abdominal wounds may be performed more safely than commonly perceived.

Soluble guanylate cyclase activation during ischemic injury in mice protects against postischemic inflammation at the mitochondrial level.

The aim was to determine whether treatment with BAY 60-2770, a selective activator of oxidized soluble guanylate cyclase (sGC), near the end of an ischemic event would prevent postischemic inflammation and mitochondrial dysfunction in wild-type (WT) and heme oxygenase-1 KO (HO-1(-/-)) mice. This protocol prevented increases in leukocyte rolling (LR) and adhesion (LA) to intestinal venules along with elevated TNFα and circulating neutrophil levels that accompany ischemia-reperfusion (I/R) in both animal models. We further hypothesized that a component of BAY 60-2770 treatment involves maintenance of mitochondrial membrane integrity during I/R. Measurements on isolated enterocytes of calcein fluorescence (mitochondrial permeability) and JC-1 fluorescence ratio (mitochondrial membrane potential) were reduced by I/R, indicating formation of mitochondrial permeability transition pores (mPTP). These effects were abrogated by BAY 60-2770 as well as cyclosporin A and SB-216763, which prevented mPTP opening and inhibited glycogen synthase kinase-3ß (GSK-3ß), respectively. Western blots of WT and HO-1(-/-) enterocytes indicated that GSK-3ß phosphorylation on Ser(9) (inhibitory site) was reduced by half following I/R alone (increased GSK-3ß activity) and increased by one-third (reduced GSK-3ß activity) following BAY 60-2770. Other investigators have associated phosphorylation of the GSK-3ß substrate cyclophilin D (pCyPD) with mPTP formation. We observed a 60% increase in pCyPD after I/R, whereas BAY 60-2770 treatment of sham and I/R groups reduced pCyPD by about 20%. In conclusion, selective activation of oxidized sGC of WT and HO-1(-/-) during ischemia protects against I/R-induced inflammation and preserves mucosal integrity in part by reducing pCyPD production and mPTP formation.