Forte, Antonio J.

Abstract Complicated craniofacial malformations interfacing with multiple intracellular regulatory mechanisms, lead to ambiguous growth patterns in Apert syndrome. This study aims to explore the chronology and pathogenesis of the development of craniofacial anatomic relationships and to verify the positional correlates between skull and facial structures in Apert syndrome. Fifty-four computed tomography scans (Apert, n = 18; control, n = 36) were included and divided into 3 age subgroups. Craniofacial 3-dimensional cephalometries were analyzed by Materialize software. The angle between sella-nasion plane and maxillary plane widens 7.74° (P = 0.003) prior to 6 months of age; thereafter, this widening increases by 10.36° (P < 0.001) in 6 months to 2 years of age, and remains increased by 8.9° (P = 0.046) throughout childhood. The angle between Frankfort horizontal plane and maxillary plane widens 5.17° (P = 0.022) before 6 months. Angles SNA, SNB, and ANB showed decreases, averaging 12.23° (P < 0.001), 5.19° (P = 0.004), and 6.72° (P = 0.001), respectively. The linear measurements showed synchronicity and continuing deformity into adulthood. Between 6 months to 2 years of age, the distance from sella to nasion (S-N), anterior nasal spine (S-ANS), and posterior nasal spine (S-PNS) decreased 8% (P = 0.006), 16% (P < 0.001), and 19% (P = 0.002), respectively, and remained shortened into adulthood. The angulation changes occur earlier in development than linear distance reduction in Apert syndrome patients compared with controls. Angular adjustments were not sufficient to maintain normal cranial base length. Facial deformity of Apert syndrome temporally begins with the midface, and affects orbit and mandible later in life.

Background:In Crouzon’s syndrome, cranial base deformities begin sequentially in the anterior cranial fossa initially, and later to the posterior cranial base. Facial characteristics are likely related to cranial base development. The temporal correlation between cranial base development and facial features is in need of clarification in Crouzon’s patients, to clarify initial sites of deformity, which may impact surgical decision making.Methods:Thirty-six computed tomography scans of unoperated Crouzon’s syndrome patients and 54 controls were included and divided into 5 age-subgroups. All the planes used for analysis were set as perpendicular to a defined “midplane” to offset the confounding factor caused by potential asymmetry.Results:The angle between Sella-Nasion plane and Frankfort horizontal plane was significantly increased before 6 months of age (P= 0.014), with an average 70% (P< 0.001) increase ultimately into adulthood. The angle between SN and maxillary plane and the angle between Sella-Nasion and occlusal planes increased consistently through infancy to adulthood (124% and 42%, respectively, bothP< 0.001). The relative angle of mandibular plane to Frankfort horizontal plane increased before 6 months (28%,P= 0.007) with a peak timeframe from 2 to 18 years. Facial lateral curvature related measurements indicate the whole face is inclined posteriorly and inferiorly direction in relation to the anterior cranial base.Conclusion:Crouzon’s facial malformation development is synchronous and positionally correlational with cranial base deformity. It transmitted from orbit to mandible, with the most evident morphologic changes are in the orbit and midface.

Background Venous congestion after deep inferior epigastric artery perforator (DIEP) flap breast reconstruction is a complication that may be partially attributable to variations in venous abdominal wall anatomy. In previous work, we have shown that ferumoxytol may be used as a bloodpool contrast agent to perform high-resolution venous imaging. Our current aim was to use this technology to perform a detailed analysis of the venous anatomy among patients undergoing DIEP flap breast reconstruction. Methods All patients undergoing DIEP flap reconstruction with preoperative ferumoxytol-enhanced magnetic resonance angiography (FE-MRA) were retrospectively reviewed. A detailed anatomic analysis of each abdominal wall on FE-MRAwas performed before review of operative findings. Statistical analysis was used to determine venous characteristics associated with superficial inferior epigastric vein (SIEV) augmentation. Results From 2012 to 2016, 59 patients underwent preoperative FE-MRA. This resulted in imaging for 118 hemiabdomen and 99 flaps. Superficial-deep communication was identified in 117 of 118 hemiabdomen. Fifty (93%) of 59 patients had greater than 1-mm venous communication of the superficial system across midline. Reconstructed breasts were based on dominant medial row perforators in 82 (83%) of 99 flaps. The mean diameters of the SIEVand dominant venous perforator were 3.8 and 2.8mm, respectively. Anatomic characteristics associated with SIEVaugmentation included SIEVdiameter ( P = 0.01), dominant perforator diameter ( P = 0.04), and the ratio between these 2 variables ( P = 0.001). Conclusions Ferumoxytol-enhanced magnetic resonance angiography provides excellent imaging of the venous system. Anatomic characteristics such as the diameter of the SIEVand the diameter of the dominant perforator may be useful in determining which flaps require venous augmentation using the SIEV.