Search

Objectives: Acute gastrointestinal graft-versus-host disease (GI-aGVHD) is a severe complication of allogeneic hematopoietic stem cell transplantation (HSCT). Diagnosis relies on clinical, endoscopic, and pathological investigations. Our purpose is to assess the value of magnetic resonance imaging (MRI) in the diagnosis, staging, and prediction of GI-aGVHD-related mortality., Methods: Twenty-one hematological patients who underwent MRI for clinical suspicion of acute GI-GVHD were retrospectively selected. Three independent radiologists, blinded to the clinical findings, reanalyzed MRI images. The GI tract was evaluated from stomach to rectum by analyzing fifteen MRI signs suggestive of intestinal and peritoneal inflammation. All selected patients underwent colonoscopy with biopsies. Disease severity was determined on the basis of clinical criteria, identifying 4 stages of increasing severity. Disease-related mortality was also assessed., Results: The diagnosis of GI-aGVHD was histologically confirmed with biopsy in 13 patients (61.9%). Using 6 major signs (diagnostic score), MRI showed 84.6% sensitivity and 100% specificity in identifying GI-aGVHD (AUC = 0.962; 95% confidence interval 0.891-1). The proximal, middle, and distal ileum were the segments most frequently affected by the disease (84.6%). Using all 15 signs of inflammation (severity score), MRI showed 100% sensitivity and 90% specificity for 1-month related mortality. No correlation with the clinical score was found., Conclusion: MRI has proved to be an effective tool for diagnosing and scoring GI-aGVHD, with a high prognostic value. If larger studies will confirm these results, MRI could partly replace endoscopy, thus becoming the primary diagnostic tool for GI-aGVHD, being more complete, less invasive, and more easily repeatable., Key Points: • We have developed a new promising MRI diagnostic score for GI-aGVHD with a sensitivity of 84.6% and specificity of 100%; results are to be confirmed by larger multicentric studies. • This MRI diagnostic score is based on the six MRI signs most frequently associated with GI-aGVHD: small-bowel inflammatory involvement, bowel wall stratification on T2-w images, wall stratification on post-contrast T1-w images, ascites, and edema of retroperitoneal fat and declivous soft tissues. • A broader MRI severity score based on 15 MRI signs showed no correlation with clinical staging but high prognostic value (100% sensitivity, 90% specificity for 1-month related mortality); these results also need to be confirmed by larger studies., (© 2023. The Author(s).)

This review focuses on the role of magnetic resonance imaging (MRI) in the evaluation of the gastrointestinal tract (GI MRI), analyzing the major technical advances achieved in this field, such as diffusion-weighted imaging, molecular imaging, motility studies, and artificial intelligence. Today, MRI performed with the more advanced imaging techniques allows accurate assessment of many bowel diseases, particularly inflammatory bowel disease and rectal cancer; in most of these diseases, MRI is invaluable for diagnosis, staging, and disease monitoring under treatment. Several MRI parameters are currently considered activity biomarkers for inflammation and neoplastic disease. Furthermore, in younger patients with acute or chronic GI disease, MRI can be safely used for short-term follow-up studies in many critical clinical situations because it is radiation-free. MRI assessment of functional gastro-esophageal and small bowel disorders is still in its infancy but very promising, while it is well established and widely used for dynamic assessment of anorectal and pelvic floor dysfunction; MRI motility biomarkers have also been described. There are still some limitations to GI MRI related to high cost and limited accessibility. However, technical advances are expected, such as faster sequences, more specific intestinal contrast agents, AI analysis of MRI data, and possibly increased accessibility to GI MRI studies. Clinical interest in the evaluation of bowel disease using MRI is already very high, but is expected to increase significantly in the coming years.

Background: FES certification is required to sit for the ABS Qualifying Exam. Previous work demonstrated a 40% FES pass rate for residents with standard clinical endoscopy training. After implementing a proficiency-based simulation curriculum, our FES pass rate increased to 87%. The purpose of this study was to monitor the success of our curriculum in its second year. We also hypothesized that residents who took the FES exam within 30 days of their clinical endoscopy rotation would have superior pass rates to residents who waited longer., Methods: PGY4 residents (N = 12) underwent flexible endoscopy training including a 1 month clinical rotation plus proficiency-based simulation training using bench-top models (Trus, Operation Targeting Task) and a virtual reality task on the GI Mentor. Residents that passed FES on their first attempt were compared to residents that did not pass based on number of endoscopies logged, hours spent practicing on simulators, and time elapsed between completing their endoscopy rotation and taking the FES exam. FES total scores and section scores were compared to historical controls., Results: Nine residents (75%) passed FES on their first attempt. Overall, 80% of residents who tested within 30 days of their endoscopy rotation (n = 5) passed FES while 71% of residents who waited longer (n = 7) passed FES (p = non-significant). Residents that passed FES were not significantly different from residents who did not pass based on number of endoscopies logged or hours spent practicing on simulators. Compared to historical controls, scores on loop reduction improved significantly with the new curriculum., Conclusions: FES pass rates decreased during the second year of our curriculum. Based on other literature, our trainees would benefit from higher volumes of endoscopy and/or a more robust proficiency-based simulation curriculum. Scheduling the FES exam in the month following the endoscopy rotation did not significantly improve pass rates.

The pathogenesis and progression of several lung disorders is propagated by inflammatory and oxidative processes, which can be controlled by adjunctive inhaled therapies. The present study aimed to develop an inhalable dry powder formulation consisting of co-spray-dried urea-crosslinked hyaluronic acid and sodium ascorbyl phosphate (SD HA-CL-SAP), a novel combination which was recently shown to possess anti-inflammatory, antioxidant, and wound healing properties. Native HA and SAP were co-spray dried (SD HA-SAP) and evaluated as control formulation. Yield (Y%) and encapsulation efficiency (EE%) were 67.0 ± 4.8% and 75.5 ± 7.2% for SD HA-SAP, 70.0 ± 1.5% and 66.5 ± 5.7% for SD HA-CL-SAP, respectively. Both formulations were shown to be suitable for lung delivery in terms of morphology, particle size (median volumetric diameter ∼ 3.4 μm), physical and thermal stability, in vitro aerosol performance - respirable fraction: 30.5 ± 0.7% for SD HA-SAP and 35.3 ± 0.3% for SD HA-CL-SAP. SAP release was investigated using Franz cells and air-interface Calu-3 cell model (>90% of SAP transported within 4 h). The innovative SD HA-CL-SAP formulation holds potential as inhalable dry powder for the treatment of inflammatory lung disorders., (Copyright © 2019 American Pharmacists Association®. Published by Elsevier Inc. All rights reserved.)

An innovative lyophilized dry powder formulation consisting of urea-crosslinked hyaluronic acid (HA-CL) and sodium ascorbyl phosphate (SAP) - LYO HA-CL - SAP- was prepared and characterized in vitro for physico-chemical and biological properties. The aim was to understand if LYO HA-CL - SAP could be used as adjuvant treatment for nasal inflammatory diseases. LYO HA-CL - SAP was suitable for nasal delivery and showed to be not toxic on human nasal septum carcinoma-derived cells (RPMI 2650 cells) at the investigated concentrations. It displayed porous, polygonal particles with unimodal, narrow size distribution, mean geometric diameter of 328.3 ± 27.5 µm, that is appropriate for nasal deposition with no respirable fraction and 88.7% of particles with aerodynamic diameter >14.1 µm. Additionally, the formulation showed wound healing ability on RPMI 2650 cells, and reduced interleukin-8 (IL-8) level in primary nasal epithelial cells pre-induced with lipopolysaccharide (LPS). Transport study across RPMI 2650 cells showed that HA-CL could act not only as carrier for SAP and active ingredient itself, but potentially also as mucoadhesive agent. In conclusion, these results suggest that HA-CL and SAP had anti-inflammatory activity and acted in combination to accelerate wound healing. Therefore, LYO HA-CL - SAP could be a potential adjuvant in nasal anti-inflammatory formulations., (Crown Copyright © 2019. Published by Elsevier B.V. All rights reserved.)

The hyporheic and riparian zones are critical domains in a river ecosystem since they mediate the interactions between surface water and groundwater. These domains are generally strongly heterogeneous and difficult to access; yet their characterization and monitoring still rely mostly on hard-to-perform invasive surveys that provide only point information. These well-known issues, however, can be overcome thanks to the application of minimally invasive methods. In this paper, we present the results of the hydrogeophysical characterization of the Vermigliana Creek's hyporheic and riparian zones, performed at an experimental site in the Adige catchment, northern Italy, by means of electrical resistivity tomography (ERT), distributed temperature sensing (DTS), and hydrological modeling. A major advancement is given by the placement of electrodes and of an optical fiber in horizontal boreholes at some depth below the river bed, put in place via directional drilling. The results of this static and dynamic (time-lapse) geophysical characterization identify the presence of two subdomains (the sub-riverbed and the left and right banks) and define the water flow and solute dynamics. The ERT information is then used, together with other hydrological data, to build a 3D subsurface hydrological model (driven mainly by the watercourse stage variations) that is calibrated against local piezometric information. A solute transport model is then developed to reproduce the variations observed in the dynamic geophysical monitoring. The results show good agreement between ERT data and the model outcome. In addition, the transport model is also consistent with the temperature data derived from DTS, even though some slight discrepancies show that the heat capacity of the solid matrix and heat conduction cannot be totally neglected., (Copyright © 2018 Elsevier B.V. All rights reserved.)

This in vitro study evaluated, for the first time, the safety and the biological activity of a novel urea-crosslinked hyaluronic acid component and sodium ascorbyl phosphate (HA-CL - SAP), singularly and/or in combination, intended for the treatment of inflammatory lung diseases. The aim was to understand if the combination HA-CL - SAP had an enhanced activity with respect to the combination native hyaluronic acid (HA) - SAP and the single SAP, HA and HA-CL components. Sample solutions displayed pH, osmolality and viscosity values suitable for lung delivery and showed to be not toxic on epithelial Calu-3 cells at the concentrations used in this study. The HA-CL - SAP displayed the most significant reduction in interleukin-6 (IL-6) and reactive oxygen species (ROS) levels, due to the combined action of HA-CL and SAP. Moreover, this combination showed improved cellular healing (wound closure) with respect to HA - SAP, SAP and HA, although at a lower rate than HA-CL alone. These preliminary results showed that the combination HA-CL - SAP could be suitable to reduce inflammation and oxidative stress in lung disorders like acute respiratory distress syndrome, asthma, emphysema and chronic obstructive pulmonary disease, where inflammation is prominent., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: Hemifacial microsomia presents with abnormalities including short ramus, absence of condyle, abnormal canting, deviated chin, and facial asymmetry. Many studies about distraction osteogenesis have been published over the last 20 years, but without long-term follow-up. The aim of this study was to evaluate patients with unilateral craniofacial microsomia who were treated with mandible distraction and with follow-up of more than 5 years., Methods: The following retrospective study was evaluated and approved by the Assistance Center for Cleft Lip and Palate. Data were compiled from the charts of 33 patients with hemifacial microsomia who underwent unilateral mandible distraction., Results: Average age at time of procedure was 7.3 years, with an average degree of distraction of 20 mm. Seventy percent of cases were treated with internal distraction, 30% external. Follow-up varied between 5 and 15 years, with a mean follow-up of 9 years. Ninety percent of the 33 patients in the study had recurrence of their asymmetry. Mean time to postsurgical recurrence was 44 months. Thirty patients were referred for orthognathic surgery. Six patients have already undergone corrective bimaxillary surgery. One patient underwent genioplasty only, and 1 patient underwent genioplasty with orthognathic jaw surgery. Twenty-two patients are awaiting orthognathic surgery, including one with temporomandibular joint ankylosis. Only 3 subjects had good outcomes, without signs of recurrence., Conclusions: Bone distraction once seemed a promising long-term option for treatment of craniofacial microsomia. However, this has not proven effective for all cases, and most patients needed subsequent orthognathic surgery.

Introduction: Number 3 cleft or oral-nasal-ocular cleft is a well-known entity that was described by Morian over a century ago. This malformation is a paranasal-medial orbitomaxillary cleft running across the lacrimal segment of the lower eyelid and over the lacrimal groove. The Tessier number 3 naso-ocular cleft represents one of the most difficult and challenging malformations to correct for the reconstructive surgeon. We have conducted a retrospective analysis of our series consisting of 21 cases., Objective: The objective was to review the functional outcome and aesthetic results of the different techniques applied for each case., Materials and Methods: From 1997 to 2007, 21 patients with a Tessier number 3 cleft were treated in our craniofacial units. The clinical findings, tomographic studies, and surgical procedures were reviewed and analyzed. We have discussed our protocol of the treatment., Results: We have treated facial malformation in 2 craniofacial centers. Fourteen patients were evaluated in the first year of their life, with an average age at presentation of 3 years. Twelve patients were female, and 9 were male; 6 patients had amniotic bands in limbs, 5 patients had an association with Tessier number 11 cleft, 3 patients with number 9 cleft, and 1 with number 7 cleft. Related to cleft lip, 10 patients had bilateral cleft lip, and 8 patients had unilateral cleft lip. Three patients did not have any involvement of the upper lip. The alar base was deviated upward in 19 patients, 11 cases had severe anatomic alteration with the lateral border of the ala above the medial canthus, and 8 cases had a mild dislocation. Nine cases of lacrimal duct obstruction and 8 cases of lacrimal duct extrophy were identified. Twelve patients had a lower eyelid coloboma of varying grades, and there were 2 cases of microblepharia. Aiming the soft tissue reconstruction, eyelid, nose, and upper lip were evaluated regarding their position, absence of tissue, and position of medial canthus and ala. Twelve of our patients underwent correction in the same moment, their medial canthus rotated upward and the ala downward, using the contralateral side as the reference. The lip was treated using a Millard-like technique. Neo-conjunctivorhinostomy was performed in the same moment in 2 patients or later in 1 case. Four patients had plagiocephaly due to the cranial involvement, and they were submitted to cranioplasty. Three had neurosurgical approach and advancement of the frontal bandeau. One adult patient received an acrylic plate to reshape the frontal area., Conclusions: Tessier number 3 cleft is one of the most difficult and challenging malformations to correct for the reconstructive surgeon. Besides the difficulties of its treatment, patients with Tessier number 3 cleft may achieve good results when the team has good skills.

Absence of half-nose is an extremely rare congenital malformation, which has a devastating impact on the patient and the family. A review of indexed English-language literature found 91 cases of half-nose, including 50 patients with proboscis lateralis. Pathogenesis is not clear, and the reported cases have sporadically occurred. Many aspects must be considered when reconstructing a congenital half-nose, such as timing of surgery, type of tissue to be used and the need to reconstruct nasal airway. The aim of this article is to present personal experience in seven cases of half-nose reconstruction, in order to review the literature regarding to this rare entity, highlighting aspects of incidence, pathogenesis and surgical treatment. Nasal reconstruction was performed at ages of 5-7 years to minimise psychological trauma. Forehead skin demonstrated to be an excellent donor site to re-surface the nose. For the inner lining, contralateral cutaneous nasal flap was our preference. Concerning the nasal framework reconstruction, alar contour was restored using a cartilage graft from the lower portion of ear tragus and concha., (Copyright (c) 2008 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

The Tessier no. 5 facial cleft is an extremely rare congenital malformation. Only 26 cases have been described in the English-language literature. The cleft begins in the upper lip just medial to the oral commissure, extending across the cheek as a groove ending at the junction of the middle and lateral thirds of the lower eyelid. The bone involvement usually includes an alveolar cleft in the premolar region, extends across the maxilla lateral to the infraorbital nerve, up to the infraorbital rim and orbital floor. The goals of the surgical procedure include reconstructing the lower eyelid, repositioning the lateral canthus, closure of the labiomaxillary cleft, and restoration of the skeletal continuity (including the orbital floor defect) with bone grafts. We present six patients with the Tessier no. 5 facial cleft who have been treated in our combined centers and discuss the surgical options and difficulties faced in the reconstruction of this rare and challenging craniofacial malformation. To date, we have treated six patients (two with bilateral and four with unilateral clefts). Three of the patients with unilateral clefting had an associated no. 4 cleft and one patient with a bilateral cleft had an associated no. 3 cleft. This paper represents the largest series to date documenting surgery for patients with the Tessier no. 5 facial cleft.

The no. 0-14 cleft involves the midline of the face and cranium. It may include both a true and a false median cleft lip, with or without associated hypotelorism or hypertelorism. The no. 0 cleft is the most common of the craniofacial clefts. The objective of this study was to review the functional outcome and aesthetic results of the different techniques applied for each case. We have conducted a retrospective analysis of our series consisting of 32 cases of Tessier no. 0 cleft, in the period between 1997 and 2007. The patients were divided into 2 groups: those with the true median cleft and those with the false median cleft. The clinical findings, lip malformation, alveolar cleft, nasal appearance, septal involvement, associated deformities, and surgical procedures, were all reviewed. Holoprosencephaly was present in 9 cases, with a false median cleft upper lip and an absence of the premaxilla, septum, and columella (only 1 patient underwent lip and columella reconstruction at 2 years of age). Nine patients had an incomplete median cleft lip. Seven of these cases had associated median alveolar cleft, and 1 had an intranasal tumor, associated with lipoma of corpus callosum, characteristic of the Pai syndrome. Six cases of a bifid nose were seen, 2 of which were associated with an alveolar median cleft and hypertelorism. An isolated median alveolar cleft was present in 7 cases, 2 of them associated with a no. 30 cleft. This article presents a large series of Tessier no. 0 cleft, describing the differences between the false and the true median cleft. The surgical procedures may vary in relation to the type of involvement.

The absence of an eyebrow, either partial or total, has been observed in patients with craniofacial clefts, such as the Tessier 9 to 13 cleft. Several techniques have been used to improve the appearance of the region, such as island scalp flaps and scalp strip grafting, with limited or marginally satisfactory aesthetic results. The authors report 2 patients with craniofacial clefts in whom a novel technique combining 2 separate surgical approaches, micrografting and tattooing, was used. The use of micrografting with single or double hair units, properly angulated, produces natural-looking and satisfactory results with a minimum of morbidity. Excellent volume and appearance of the eyebrow may be achieved in a single session using this technique. Tattooing performed subsequently over the microimplanted hairs provides the illusion of greater density to the eyebrow, resulting in an appearance closer to normal.

Epignathus is a rare congenital nasopharyngeal tumour that is derived from the upper jaw, palate, and sphenoid bone. It usually protrudes through the mouth, leading to an appreciable risk of obstruction of the upper airway and death soon after birth. We report two cases of unusual presentations that illustrated some uncommon and similar characteristics. Although these tumours are not consistent in origin, number, and differentiation of tissues, both contained structures that were derived from all three layers of germ cells, including different anomalous tissues internally (dental bud, fat and muscular tissues, mucosal epithelium). Externally, they contained normal epidermis (skin with fine hairs). Imaging studies and operative findings showed that both tumours originated from the anterior portion of the sphenoid bone causing a sphenoidal sinus cleft without intracranial communication.

Background: Despite all benefits offered by mandible distraction, complications and long-term consequences need to be evaluated to define its safety and morbidity. Forty mandible distractions were studied. Panoramic mandible radiographs obtained preoperatively, during distraction, and during the postoperative period were reviewed, with the intention of evaluating development and complications of molar buds and teeth in the distraction area., Methods: The mean patient age was 8.1 years. Twenty-five patients had craniofacial microsomia (one associated with a no. 10 facial cleft), five had temporomandibular joint ankylosis, two had familiar cases of auriculocondylar syndrome, one had a Tessier no. 30 facial cleft, and one had Treacher Collins syndrome. The severity of mandible hypoplasia was Pruzansky grade I in four cases, grade IIA in eight cases, grade IIB in 16 cases, and grade III in one case. Mean radiographic follow-up was 44.8 months., Results: Molar buds located in the distraction area erupted without any deformity or displacement in 18 sides (45 percent). Fourteen cases presented distalization of a dental bud to a superior position in the mandibular ramus (four migrated back to the original position). Six molar buds presented perforations, four had shape deformities (two caused by dental fracture), and two had dental root injuries followed by root absorption lately. One case developed a dentigerous cyst., Conclusions: Almost half of the patients did not have any molar bud or tooth alterations after mandible distraction, and more than 20 percent presented only bud distalization. Therefore, preventive bud enucleation or tooth extraction should be avoided before mandible distraction.

For many years, surgeons have sought a method to treat severe facial deformities without using bone grafts and extensive surgery. Distraction osteogenesis offers this promise. The technique used in mandibular hypoplasia follows the basic principles proposed by Ilizarov which states that the device must be elongated 1 mm per day to create optimal bone production. Despite the widespread implementation of this recommendation to include the 1-mm/day separation, doubt still exists as to whether this is the optimal treatment regimen. Intraoral devices with percutaneous activator pins were used in 16 patients with hypoplastic mandibles. The results of distraction were documented by panorex and cephalogram of the mandible. The length of the ramus as well as multiple mandible dimensions and facial angles were measured. The panorex and cephalogram of the mandible were effective in demonstrating the significant increase in length of the mandible and ramus, as well as the entire mandible, but there was no correlation between the stretching obtained by the distraction device and that measured by the radiographic studies. The S-N-B angle was the only facial angle in which there is a statistically significant increase measured and this appeared to be related to a mandible rotation. It is concluded that the mandible distraction (using an intraoral device and an external activator pin) was effective in increasing the ramus length and both the panorex and the cephalogram were effective in demonstrating this morphologic change. There was no correlation between the clinical result and the radiographic studies demonstrating that the clinical judgment still has a significant role in controlling mandible distraction.