Jorge Forte, Antonio

Background: Lymphovenous anastomosis (LVA) is an accepted microsurgical treatment for lymphedema of the upper extremity (UE). This study summarizes and analyzes recent data on the outcomes associated with LVA for UE lymphedema at varying degrees of severity. Methods: A literature search was conducted in the PubMed database to extract articles published through June 19, 2020. Studies reporting data on postoperative improvement in limb circumference/volume or subjective improvement in quality of life for patients with primary or secondary lymphedema of the UE were included. Extracted data consisted of demographic data, number of patients and upper limbs, duration of symptoms before LVA, surgical technique, follow-up, and objective and subjective outcomes. Results: A total of 92 articles were identified, of which 16 studies were eligible for final inclusion comprising a total of 349 patients and 244 upper limbs. The average age of patients ranged from 38.4 to 64 years. The duration of lymphedema before LVA ranged from 9 months to 7 years. The mean length of follow-up ranged from 6 months to 8 years. Fourteen studies reported an objective improvement in limb circumference or volume measurements following LVA, ranging from 0% to 100%. Patients included had varying severity of lymphedema, ranging from Campisi stage I to IV. The maximal improvement in objective measurements was found in patients with lower stage lymphedema. Conclusion: LVA is a safe, effective technique for the treatment of UE lymphedema refractory to decompressive treatment. Results of LVA indicate greater efficacy in earlier stages of lymphedema before advanced lymphatic sclerosis.

Background: We propose a landmark-based, virtual coordinate system, specifically designed for assessment of asymmetrical craniofacial anatomy associated with unicoronal synostosis. Method: CT scans of 33 patients with nonsyndromic unicoronal synostosis were included. Proposed mid-sagittal plane was compared with commonly used sagittal planes: (1) nasion, sella, and basion (N-S-BA); (2) midplane of bilateral frontozygomatic sutures (midFZ); and (3) the skull gravity center plane, to evaluate reliability and validity in the assessments of the anterior and posterior skull base. Results: The proposed midplane is similar to the midFZ plane in describing the direction of the anterior skull base. However, it has less bias than the N-S-BA (P < 0.001), and the gravity center planes (P < 0.001). The proposed midplane measures the direction of the posterior skull base plane, similar to the midFZ and gravity center planes, but it has less measurement deviation than the N-S-BA plane (P < 0.001). The most protrusive point on the frontal bone in unicoronal patients is contralateral to the fused suture and distant from the mid-sagittal plane by 13.93 ± 4.01 mm. In addition, it is more anteriorly positioned, by 5.32 mm (P < 0.001), when compared with the corresponding point on the synostotic side. The uppermost point of the supraorbital rim on the synostotic side is cephalic to that of the contralateral side by 4.09 mm (P < 0.001). Conclusions: Prioritized orientation of an averaged Frankfort horizontal plane, followed by the location of the mid-sagittal and coronal planes, can generate a reliable and valid coordinate framework for the assessment of asymmetric skull shape in unicoronal synostosis.

Background: Considerable craniofacial features of Crouzon syndrome are attributed to the dysmorphology of the cranial base. As cephalometric studies have focused mainly on the facial deformity, rather than the cranial base, the underlying cause of deformity is not as well understood. Therefore, the authors compared the cranial base development of Crouzon syndrome to controls to trace the timing of deformity in the cranial base and face, to analyze their temporal correlation. Methods: Ninety computed tomographic scans were included (Crouzon, n = 36; controls, n = 54) and divided into five age subgroups. Craniofacial cephalometric measurements were analyzed by Materialise software. Results: The overall cranial base length in Crouzon syndrome compared with controls decreased 8 percent (p = 0.014) on average. The posterior cranial fossa shortening accounted for most of this reduction. The cranial base displaced with the distances from basion, sella, and ethmosphenoid to posterior nasal spine shortened by 21%, 18%, and 16%, respectively (all p < 0.01) during life. Although the cranial base angle on intracranial surface remains normal, the angles on facial surface narrowed were reduced. Conclusions: The cranial base deformity of Crouzon syndrome consists of the whole skull base and particularly anterior skull base shortening early, leading to a compensatory widened anterior skull base. However, when this widening did not compensate fully for the rapid enlargement of the brain, the posterior skull base displaced inferiorly and became kyphotic. The cranial base deformity develops sequentially anterior to posterior in a probable cascade of influence pattern. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

Background: Apert syndrome patients are different in clinical pathology, including obstructive sleep apnea, cleft palate, and mental deficiency. These functional deficiencies may be due to anatomic deformities, which may be caused by different forms of associated suture fusion. Therefore, a classification system of Apert syndrome based on the type of craniosynostosis pattern might be helpful in determining treatment choices. Methods: CT scans of 31 unoperated Apert syndrome and 51 controls were included and subgrouped as: class I. Bilateral coronal synostosis; class II. Pansynostosis; and class III. Perpendicular combination synostosis: a. unilateral coronal and metopic synostosis; b. sagittal with bilateral/unilateral lambdoid synostosis; and c. others. Results: Class I is the most common (55%) subtype. The cranial base angulation of class I was normal; however, the cranial base angulation on the cranium side of the skull in class II increased 12.16 degrees (P = 0.006), whereas the facial side cranial base angle of class IIIa decreased 4.31 degrees (P = 0.035) over time. The external cranial base linear measurements of class I showed more evident reduction in anterior craniofacial structures than posterior, whereas other subtypes developed more severe shortening in the posterior aspects. Conclusions: Bicoronal synostosis is the most common subtype of Apert syndrome with the normalized cranial base angulation. Combined pansynostosis patients have flatter cranial base, whereas the combined unilateral coronal synostosis have a kyphotic cranial base. Class I has more significant nasopharyngeal airway compromise in a vertical direction, whereas classes II and III have more limited oropharyngeal space.