Transnasal Medial Canthopexy Supported with Autogenous Bone Graft: A New Method for Repair of Traumatic Telecanthus.

INTRODUCTION: The medial canthus is an important structure to maintain the shape of the eye and assist in drainage of the lacrimal sac. Traumatic telecanthus is a difficult deformity to treat which involves both esthetic and functional aspects. Transnasal wiring remains the gold standard for repair of the medial canthal tendon (MCT) avulsion; however, it is often complicated by canthal drift, extrusion of wires, and in-fracture of the contralateral orbital bones from pressure by tied wires. In order to overcome traditional transnasal wiring technique pitfalls, this study proposes a transnasal wiring fixation method supported with a bone graft to treat patients presenting with telecanthus following complex nasoorbitoethmoid fractures. PATIENTS AND METHODS: From December 2018 to October 2020, 12 patients with traumatic telecanthus underwent transnasal wiring fixation of the MCT supported with bone graft. The wire holding the MCT was delivered through a single hole to the contralateral side and secured to a small bone graft by passing the wire through 2 holes in the graft in a button-like fashion. Then the wire end is pulled again to the affected side through the same hole and the 2 wires ends are tightly twisted after setting the MCT posterior to the lacrimal crest. RESULTS: Restoration of the MCT to its normal position was achieved in all patients. There were no observed major complications. Only 2 cases of wound infection and 1 case of dacrocystitis were encountered, which settled with conservative management. The preoperative palpebral fissure width (mean: 28.33mm ± 2.188mm) was significantly lower than the postoperative palpebral fissure width (mean: 34.17mm ± 1.03mm) ( P< 0.05). CONCLUSIONS: This study introduces a modified technique of transnasal wiring fixation to restore palpebral shape and intercanthal distance. The proposed technique could eliminate most of the frequently observed complications of the traditional methods.

Clinical Findings and Outcomes of Palatal Repair in 9 Patients With Van der Woude Syndrome.

INTRODUCTION: Van der Woude syndrome (VWS), an autosomal dominant condition associated with clefts of the lip and/or palate and lower lip pits, is caused by mutations in interferon regulatory factor 6 gene. It is reported to be the most common syndromic cleft worldwide. This case series presents the phenotypic characteristics and treatment outcomes in a group of 9 patients diagnosed with VWS. METHODS: A retrospective review was performed on records of patients given a diagnosis of VWS presenting to the Department of Plastic Surgery of Sohag University between July 2009 and November 2019. Data analyses included age and sex of affected patients, type of the cleft, associated anomalies, presence of lower lip pits, and history of lower lip pits/cleft in the family and treatment outcomes. RESULTS: The study identified 9 patients (male = 2, female = 7). Age at first presentation ranged between 1 week and 7 years (mean = 1.3 years). Four patients had bilateral cleft lip and palate and 1 patient had unilateral cleft lip and palate (UCLP), whereas 4 patients had isolated cleft palate. Bilateral lower lip pits were presented in 8 patients, whereas the ninth patient has unilateral pit. Family history was positive in 3 patients. Most patients in this study developed after palatal repair speech problems with an increased rate of secondary surgical procedures needed for correction of velopharyngeal insufficiency. CONCLUSIONS: All reviewed cases of VWS presented with lower lip pits and cleft palate with or without cleft lip. A positive family history of similar conditions was demonstrated in 33.3% of our patients. This study suggests an increased incidence of secondary palatal surgeries for velopharyngeal insufficiency in patients with VWS; this warrant further studies including control group of nonsyndromic cleft patients.

Use of neuroimaging measurements of optic nerve sheath diameter to assess intracranial pressure in craniosynostosis.

PURPOSE: This study aims to evaluate accuracy of optic nerve sheath diameter (ONSD) measurements obtained by magnetic resonance imaging (MRI) in patients with craniosynostosis (CS) for detection of high intracranial pressure (ICP) and to correlate MRI-derived ONSD measurements with those obtained by computed tomography (CT) scans in CS patients. METHODS: A retrospective review was conducted on CS patients who had MRI- and age-matched controls with normal MRI. Diagnosis of intracranial hypertension was based on presence of papilledema, direct ICP monitoring, and/or lumbar puncture. The search also included patients with MRI and CT done within 30 days apart. ONSDs were measured 3 mm behind the globe on both modalities. RESULTS: The study identified 56 CS patients (mean age 3.8 ± 3.47 years) and 49 controls (mean age 3.7 ± 3.62 years). Mean ONSD in patients with high ICP was significantly higher than in patients without high ICP (P = 0.0001) and in controls (P < 0.0001). The optimal ONSD threshold for predicting raised ICP in patients >1 year old was 6 mm (71.4% sensitivity, 89.7% specificity). Nineteen patients with 38 single-eye MRI/CT pairs were identified. Substantial agreement between both modalities resulted (r = 0.959, 95% CI 0.923-0.978), and Bland and Altman Plot analysis showed that 95% of measurements fell within limits of agreement (1.96 SD; ± 0.6 mm). CONCLUSION: In CS patients, ONSD measured by MRI represent indirect non-invasive means of ICP assessment. Both MRI and CT measurements of ONSD gave comparable results, and the use of CT-derived ONSD measurements may give some idea about ICP in CS patients.

Survival of Nasal Tip Flap on Only the Columellar Attachment.

The nose has a robust blood supply derived from both internal and external carotid arteries. The main blood supply of the nasal tip and skin is derived primarily from the lateral nasal arteries, with a variable contribution from the columellar arteries. We present a patient with cut nose resulting from a homicidal attack in which the lower two-thirds of the nose was severed starting at the bony-cartilaginous junction of the dorsum of the nose down to the tip of the nose. The skin-cartilage flap was attached only to the columella and to the caudalmost 3 mm of the left ala nasi. The detached skin-cartilage flap was replaced and sutured anatomically, and the flap completely survived in spite of cutting of both lateral and dorsal nasal arteries.

Reliability and Safety of Cross-Leg Free Latissmus Dorsi Muscle Flap in Reconstruction of Mutilating Leg Injuries Using End-to-Side Anastomosis.

Background Free tissue transfer is considered the gold standard option for the reconstruction of distal leg defects. Free tissue transfer using recipient vessels in the contralateral leg (cross-leg bridge) is a potential option to supply the flap if there are no suitable recipient vessels in the injured leg. Most studies have described this technique using end-to-end anastomosis which sacrifices the main vessel in the uninjured leg. This study evaluated the use of a cross-leg free latissimus dorsi muscle flap for the reconstruction of defects in single-vessel legs, using end-to-side anastomosis to recipient vessels in the contralateral leg without sacrificing any vessel in the uninjured leg. Methods This is a retrospective study that included 22 consecutive patients with soft tissue defects over the lower leg. All the reconstructed legs had a single artery as documented by CT angiography. All patients underwent cross-leg free latissimus dorsi muscle flap using end-to-side anastomosis to the posterior tibial vessels of the contralateral leg. Results The age at surgery ranged from 12 to 31 years and the mean defect size was 86 cm 2 . Complete flap survival occurred in 20 cases (91%). One patient had total flap ischemia. Another patient had distal flap ischemia. Conclusion Cross-leg free latissimus dorsi muscle flap is a reliable and safe technique for the reconstruction and salvage of mutilating leg injuries, especially in cases of leg injuries with a single artery. As far as preservation of the donor limb circulation is concerned, end-to-side anastomosis is a reasonable option as it maintains the continuity of the donor leg vessels.

Serial, Visually-Evoked Potentials for the Assessment of Visual Function in Patients with Craniosynostosis.

This study aimed to evaluate the effect of craniofacial surgical intervention on the visual pathway's function by comparing pre- to post-operative patterned, visually-evoked potentials (pVEP). A retrospective review was conducted on craniosynostosis patients who had pre- and post-craniofacial surgery pVEP testing. The pVEP measured grade in terms of amplitude latency and morphology of the waveforms. The pre- and post-operative results were compared. The study identified 63 patients (mean age at preoperative pVEP of 16.9 months). Preoperatively, 33 patients (52.4%) had abnormal pVEP. Nine patients had evidence of intracranial hypertension, and of those, eight (88.9%) had abnormal pVEP. Within 6 months postoperatively, 24 of 33 patients (72.7%) with abnormal preoperative pVEP developed normal postoperative pVEP, while all 30 patients with normal preoperative VEP maintained their normal results postoperatively. Significant improvements in pVEP latency in patients with broad or delayed latency waveforms was evident for subjects with preoperative grades 2-4 (grade 2, p = 0.015; grade 3, p = 0.029; grade 4; p = 0.007), while significant postoperative increase in amplitude was significant for patients with abnormally low amplitude grade 3 and 5 waveforms (grade 3, p = 0.011; grade 5, p = 0.029). Serial pVEP testing represents a useful tool for the early detection of visual pathway dysfunction and follow up visual pathway function in craniosynostosis. Surgical intervention for craniosynostosis can result in the reversal of preoperative pVEP abnormalities seen in these patients, resulting in the normalization of the pVEP waveform, amplitude and latency, depending on the preoperative pVEP abnormality.

Serial Visual Evoked Potentials in Patients with Craniosynostosis and Invasive Intracranial Pressure Monitoring.

This study aimed to detect the ability of pattern visual evoked potentials to detect visual pathway dysfunction in a cohort of patients with craniosynostosis who also had invasive intracranial pressure measurement. A retrospective review was conducted on craniosynostosis patients who had invasive intracranial pressure measurement and at least one pattern visual evoked potentials test. Reversal pattern visual evoked potentials were performed with both eyes open. Thirteen patients met the inclusion criteria (mean age at intracranial pressure measurement, 5.7 years). Seven patients had raised intracranial pressure, and of these, five (71.4 percent) had abnormal or deteriorated pattern visual evoked potentials parameters on serial testing, whereas all patients (100 percent) with normal intracranial pressure had normal pattern visual evoked potentials amplitude and latency. Four of the five patients (80 percent) with raised intracranial pressure and abnormal pattern visual evoked potentials did not show evidence of papilledema. The mean latency in patients with raised intracranial pressure (118.7 msec) was longer than in those with normal intracranial pressure (108.1 msec), although it did not reach statistical significance (p = 0.09), whereas the mean amplitude in patients with raised intracranial pressure (12.4 µV) was significantly lower than in patients with normal intracranial pressure (23.3 µV) (p = 0.03). The authors' results showed that serial pattern visual evoked potentials testing was able to detect visual pathway dysfunction resulting from raised intracranial pressure in five of seven craniosynostosis patients, and of these five patients, 80 percent had no evidence of papilledema, demonstrating the utility of serial pattern visual evoked potentials in follow-up of the visual function in craniosynostosis patients. CLINICAL QUESTION/LEVEL OF EVIDENCE:: Diagnostic, II.

Syndromic and Systemic Diagnoses Associated With Isolated Sagittal Synostosis.

Reports of systemic associations in patients with Isolated Sagittal Synostosis (ISS) are sparse. Craniofacial surgeons, and other providers, should be aware that a significant proportion of patients with ISS may have syndromic or systemic involvement. This study investigates the incidence of systemic disease and syndromic diagnosis in a cohort of patients presenting with ISS (ie, patients with sagittal synostosis without other sutural involvement). METHODS: This study consists of a retrospective review of patients diagnosed with ISS between 2007 and 2017 at a single institution. Patients were divided according to onset (early <1 year, late >1 year) of ISS. Patient notes were examined for congenital anomalies, systemic conditions, and molecular testing. Only patients with isolated sagittal fusion-meaning, patients with sagittal synostosis and no other sutural involvement-were included. RESULTS: Three hundred seventy-seven patients met the inclusion criteria: systemic conditions were identified in 188/377 (50%) of them. One hundred sixty-one patients with early onset (Group A), and 216 patients with late onset ISS (Group B) were identified. Systemic involvement was identified in 38% of Group A and 60% of Group B, which was statistically significant (P < 0.001). Forty-eight of 377 (13%) of patients had a syndromic diagnosis, and 79% of these were confirmed via genetic testing. Thirty-five percent of patients were diagnosed with central nervous system anomalies and 16% had craniofacial anomalies. CONCLUSIONS: Nearly 50% of the patients initially diagnosed with ISS were found to have some form of systemic involvement. This supports affording full pediatric and genetic evaluation with molecular testing to these children.

The Incidence of Chiari Malformations in Patients with Isolated Sagittal Synostosis.

BACKGROUND: We report the incidence of Chiari malformation I (CMI) in a cohort of 377 patients with isolated sagittal synostosis (ISS), which is to the best of our knowledge the largest such series reported to date. METHODS: A retrospective review of patients seen at a single institution from 2007 to 2017 was completed. ISS, Chiari malformations (CMI and CMII) and hydrocephalus were diagnosed by a senior neuroradiologist (G.Z.). Patients who met the inclusion criteria were divided into early (group A) and late (group B) presenting groups, as well as operated (group I) and unoperated (group II) groups. The patients were further subdivided into group AI (early operated), group AII (early unoperated), group BI (late operated), and group BII (late unoperated). Once identified, patient notes were examined for the following data sets: date of birth, age of presentation, age at last follow-up, other systemic conditions as well as molecular testing results. Surgical interventions, ophthalmological, and other relevant data were recorded. Statistical analysis was run in the form of a chi-square test to identify a significant difference between each subgroup. A literature review of the incidence of Chiari malformations in patients with ISS was conducted. RESULTS: Three hundred seventy-seven patients constitute the study's total cohort (272 were males and 105 females). This cohort was divided into patients who underwent surgical repair of ISS (group 1: n = 200), and patients who did not (group 2: n = 177). The entire cohort was also divided into early (group A: n = 161) and late (group B: n = 216) presenting craniosynostosis. In the total cohort, 22/377 (5.8%) patients with CMI were identified. CMI was found in 14/200 (7.0%) patients in group I, and 8/177 (4.5%) patients in group II. CMI was found in 2/161 (1%) patients in group A, and 20/216 (9.2%) patients in group B. The incidence of CMI in group AI (early operated) was 2/151 (1.3%), in group AII (early unoperated) was 0/10, in group BI (late operated) was 11/49 (21%), and in group BII (late unoperated) was 9/167 (5.4%). Chi-square analysis revealed a significant difference between the incidence of CMI in the early-presenting (group A) and late-presenting (group B) groups (P = 0.001) and between the late-presenting operated (BI) and late-presenting unoperated (BII) groups (P = 0.001). The incidence of hydrocephalus was 1.6% (6/377) in the total cohort. However, all patients diagnosed with hydrocephalus came from group II (no surgical ISS correction). The incidence of hydrocephalus in group II was 3.3% (6/177). The incidence of hydrocephalus in group BII (late unoperated ISS) was 3.0% (5/167). The incidence of hydrocephalus in group AII (early unoperated ISS) was 9.0% (1/11). CONCLUSIONS: We noted the highest incidence of CMI-21%-in group BI (late-presenting operated). We noted hydrocephalus in group II (nonoperated), with the highest incidence of hydrocephalus found in the group BII (late-presenting unoperated) subgroup. We therefore recommend patients with ISS receive funduscopic examination to screen for raised intracranial pressure (ICP) associated with CMI and hydrocephalus, especially patients with late-presenting ISS.

RASopathy in Patients With Isolated Sagittal Synostosis.

RASopathy is caused by dysfunction in the MAPK pathway, and include syndromes like Noonan syndrome (NS), NS with multiple lentigines (formerly known as Leopard syndrome), cardiofaciocutaneous (CFC), Legius syndrome, capillary malformation-arteriovenous malformation, neurofibromatosis type 1, and Costello syndrome. When counted together, RASopathies affect 1/1000 live births, and are characterized by cardiovascular manifestations, short stature, developmental delay, renal, urogenital, skin/skeletal abnormalities, and dysmorphic appearance. NS-one of the most common RASopathies-occurs in 1/1000 to 1/2500 live births. On the other hand, the frequency of CFC is unknown, but it is one of the rarest RASopathies, with estimates of only a few hundred cases worldwide. However, its phenotype overlaps with that of NS. In this case series, we describe 5 patients with a clinical and genetic diagnosis of RASopathy-either NS or CFC-all of whom were also diagnosed with isolated sagittal synostosis (ISS). Medical records from ophthalmology, cardiology, plastic surgery, medical genetics, cleft craniofacial, and neurosurgery were used to determine patient history. In our cohort, late presentation of ISS was the predominant form of ISS presentation. We hope this report further characterizes the burgeoning relationship between RASopathy and ISS. Furthermore, these findings support including sagittal synostosis among the presenting features in the clinical phenotype of RASopathies. Ethical approval was obtained from the university's institutional review board.