Successful Treatment of Fibro-Adipose Vascular Anomaly with Sirolimus.

BACKGROUND: The purpose of this study was to present our initial experience in using sirolimus therapy to treat fibro-adipose vascular anomaly (FAVA). METHODS: We retrospectively reviewed the medical records of eight patients with FAVA who were treated with sirolimus at our hospital between July 2017 and October 2020. RESULTS: Six girls (75%) and two boys (25%) were included in the cohort; the average age was 8 years (range, 1-13 years). Vascular tumors developed mainly on the extremities, including the forearm (n = 2; 25.0%), calf (n = 4; 50.0%), and thigh (n = 2; 25.0%). The predominant symptoms included swelling of the lesion (n = 8; 100%), pain (n = 7; 87.5%), contracture (n = 3; 37.5%), and phlebectasia (n = 3; 37.5%). Magnetic resonance imaging was the primary method used for FAVA diagnosis, and all patients underwent enhanced MRI. All lesions were heterogeneous with hyperintense T1 signals. The fat-suppressed T2-weighted images also revealed heterogeneous hyperintense masses, thus indicating fibrofatty infiltration. All eight patients received a sirolimus treatment regimen after FAVA diagnosis. One patient underwent tumor resection but experienced recurrence, whereas the other six patients underwent biopsy. Histological examination revealed that the lesions consisted of fibrofatty tissue with abnormal venous channels and anomalous lymphatic vascular components. Sirolimus softened the masses and caused tumor shrinkage within 5.25 ± 2.6 weeks (range, 2-10 weeks) after treatment initiation. The tumors also involuted rapidly and became stable within 7.75 ± 2.25 months after treatment initiation (range, 6-12 months). All seven patients experiencing pain reported relief within 3.8 ± 1.8 weeks (range, 2-7 weeks) after initiation of sirolimus therapy. Sirolimus alleviated but did not fully resolve the contracture in three patients. Remarkably, five patients exhibited a complete response, and three patients exhibited a partial response. At the time of the last follow-up, three patients had begun to gradually taper off sirolimus after 24 months of treatment and maintained a low blood sirolimus concentration. No serious adverse effects were observed during treatment. CONCLUSION: FAVA is a complex vascular malformation that appears to respond well to sirolimus treatment. Thus, sirolimus may be an effective and safe treatment for FAVA. LEVEL OF EVIDENCE: LEVEL IV.

Treatment and Prevention of Periprosthetic Capsular Contracture in Breast Surgery With Prosthesis Using Leukotriene Receptor Antagonists: A Meta-Analysis.

BACKGROUND: Capsular contracture (CC) is the most common long-term complication of breast surgery with prosthesis. Leukotriene receptor antagonists (LRAs) have been tested as a potential treatment; however, mixed results have been observed. OBJECTIVES: The aim of this study was to undertake a meta-analysis to clarify the treatment and prophylactic capabilities of LRAs in the management of CC. METHODS: A systematic literature search of the most popular English-language databases was performed to identify relevant primary publications. We included all studies that used the Baker scale to evaluate the treatment and preventive capabilities of LRAs. RESULTS: Six eligible studies were included based on predefined inclusion and exclusion criteria, totalling 2276 breasts, of which 775 did not receive LRAs and 1501 did. Final pooled results showed that LRAs could help manage CC with a risk difference (RD) of -0.38 with a corresponding 95% CI of -0.69 to -0.08, showing statistical significance at a Z value of 2.48, P = 0.01. Subgroup analysis based on the type of drug showed that only montelukast yielded statistical significance (RD = -0.27, 95% CI = -0.51 to -0.03, Z = 2.20, P = 0.03). Zafirlukast did not seem to influence CC. Further subgroup analysis based on treatment timing showed that prophylaxis was ineffective and only treatment for ongoing CC yielded statistically significant improvements. CONCLUSIONS: The current meta-analysis proved that LRAs could be used in the management of CC. Only treatment for ongoing CC showed statistically significant improvements. Montelukast seemed to be more efficient with a safer profile for adverse effects, whereas zafirlukast yielded no statistically significant results.

Breast contracture and skin sclerosis following 20 years of polyacrylamide hydrogel migration in a patient with familial vitiligo: a case report.

BACKGROUND: Breast augmentation with polyacrylamide gel (PAAG) injection was approved in China in 1998 and later banned in 2006. The ban ensued numerous complaints from patients such as pain, induration, deformation, infection, displacement, and milk deposition associated with PAAG injection. To date, no study has investigated the long-term effect of PAAG migration on autoimmune diseases. CASE PRESENTATION: We report a rare case of a 49-year-old female patient with familial vitiligo who receiving PAAG injection for breast augmentation. The patient reported to have felt persistent movement of PAAG in her thoracoabdominal area for almost 20 years. Furthermore, the PAAG-induced chronic inflammation that aggravated vitiligo, which in turn promoted skin sclerosis. This damaged the breast contracture, increased chest tightness and induced mild breathing problems. CONCLUSION: Here, we present a rare case in which a patient with a family history of vitiligo experienced long-term complications after receiving PAAG injection for breast augmentation. This case highlights the relationship between vitiligo, migration of PAAG and tissue hardening and skin contraction. LEVEL OF EVIDENCE: Level V.

Impact of statin intake on malignant hyperthermia: an in vitro and in vivo swine study.

BACKGROUND: Statin intake is associated with muscular side effects, among which the unmasking of latent myopathies and of malignant hyperthermia (MH) susceptibility have been reported. These findings, together with experimental data in small animals, prompt speculation that statin therapy may compromise the performance of skeletal muscle during diagnostic in vitro contracture tests (IVCT). In addition, statins might reduce triggering thresholds in susceptible individuals (MHS), or exacerbate MH progression. We sought to obtain empirical data to address these questions. METHODS: We compared the responses of 3 different muscles from untreated or simvastatin treated MHS and non-susceptible (MHN) pigs. MHS animals were also invasively monitored for signs of impending MH during sevoflurane anesthesia. RESULTS: Muscles from statin treated MHS pigs responded with enhanced in vitro contractures to halothane, while responses to caffeine were unaltered by the treatment. Neither agent elicited contractures in muscles from statin treated MHN pigs. In vivo, end- tide pCO2, hemodynamic evolution, plasma pH, potassium and lactate concentrations consistently pointed to mild acceleration of MH development in statin-treated pigs, whereas masseter spasm and rigor faded compared to untreated MHS animals. CONCLUSIONS: The diagnostic sensitivity and specificity of the IVCT remains unchanged by a short-term simvastatin treatment in MHS swine. Evidence of modest enhancement in cardiovascular and metabolic signs of MH, as well as masked pathognomonic muscle rigor observed under simvastatin therapy suggest a potentially misleading influence on the clinical presentation of MH. The findings deserve further study to include other statins and therapeutic regimes.

Is there a link between exertional heat stroke and susceptibility to malignant hyperthermia?

OBJECTIVE: The identification of a predisposition toward malignant hyperthermia (MH) as a risk factor for exertional heat stroke (EHS) remains a matter of debate. Such a predisposition indicates a causal role for MH susceptibility (MHS) after EHS in certain national recommendations and has led to the use of an in vitro contracture test (IVCT) to identify the MHS trait in selected or unselected EHS patients. The aim of this study was to determine whether the MHS trait is associated with EHS. METHODS: EHS subjects in the French Armed Forces were routinely examined for MHS after experiencing an EHS episode. This retrospective study compared the features of IVCT-diagnosed MHS (iMHS) EHS subjects with those of MH-normal EHS patients and MH patients during the 2004-2010 period. MHS status was assessed using the European protocol. RESULTS: During the study period, 466 subjects (median age 25 years; 31 women) underwent MHS status investigation following an EHS episode. None of the subjects reported previous MH events. An IVCT was performed in 454 cases and was diagnostic of MHS in 45.6% of the study population, of MH susceptibility to halothane in 18.5%, of MH susceptibility to caffeine in 9.9%, and of MH susceptibility to halothane and caffeine in 17.2%. There were no differences in the clinical features, biological features or outcomes of iMHS EHS subjects compared with those of MH-normal or caffeine or halothane MHS subjects without known prior EHS episode. The recurrence rate was 12.7% and was not associated with MH status or any clinical or biological features. iMHS EHS patients exhibited a significantly less informative IVCT response than MH patients. CONCLUSIONS: The unexpected high prevalence of the MHS trait after EHS suggested a latent disturbance of calcium homeostasis that accounted for the positive IVCT results. This study did not determine whether EHS patients have an increased risk of MH, and it could not determine whether MH susceptibility is a risk factor for EHS.

Clinical outcomes of augmentation cystoplasty in patients suffering from ketamine-related bladder contractures.

OBJECTIVE: To evaluate the outcomes of augmentation cystoplasty in patients with bladder contractures secondary to chronic ketamine abuse. METHOD: Patients who had received augmentation cystoplasty to treat ketamine-related bladder contractures in two hospitals in our region were reviewed retrospectively. Their history of ketamine consumption, presenting symptoms, history of treatment, surgical information and post-operative conditions were retrieved from clinical records and then summarized. RESULTS: Between 2006 and 2011, four patients (three women and one man), aged 21-30 years (mean 27 years), underwent augmentation cystoplasty for ketamine-related bladder contractures. The duration of ketamine consumption ranged from 3 to 15 years, and all four patients resumed ketamine consumption after surgery. The mean maximal baseline and post-operative bladder capacity was 37.5 cc (range 25-50 cc) and up to 400-500 cc, respectively. Three patients experienced a further deterioration in renal function that was secondary to new-onset ureteral strictures in two cases and to sepsis in the other. At the time of the last follow-up, three patients could void spontaneously and one required regular intermittent catheterization. CONCLUSION: Ketamine cystitis is an emerging medical condition that requires a multi-disciplinary approach to manage the patients. Simple surgical management of the physical component of the contracted bladder may produce only suboptimal results, and could even cause further problems in some patients. The importance of compliance with post-operative care and abstinence from drug use should be stressed to the patients before surgery. In view of the high complication rate, the option of a simple ileal conduit should also be discussed prior to surgical intervention.

Use of the caffeine-halothane contracture test for the diagnosis of malignant hyperthermia in Brazil

Malignant hyperthermia (MH) is a pharmacogenetic disease triggered by volatile anesthetics and succinylcholine. Deaths due to MH have been reported in Brazil. The first Malignant Hyperthermia Diagnostic and Research Center in Latin America was inaugurated in 1993 at the Federal University of Rio de Janeiro, Brazil. The center followed the diagnostic protocols of the North America MH Group, in which the contractures of biopsies from the vastus lateralis muscle are analyzed after exposure to caffeine and halothane (CHCT). CHCT was performed in individuals who survived, their relatives and those with signs/symptoms somewhat related to MH susceptibility (MHS). Here, we report data from 194 patients collected over 16 years. The Southeast (N = 110) and South (N = 71) represented the majority of patients. Median age was 25 (4-70) years, with similar numbers of males (104) and females (90). MHS was found in 90 patients and 104 patients were normal. Abnormal responses to both caffeine and halothane were observed in 59 patients and to caffeine or halothane in 20 and 11 patients, respectively. The contracture of biopsies from MHS exposed to caffeine and halothane was 1.027 ± 0.075 g (N = 285) and 4.021 ± 0.255 g (N = 226), respectively. MHS was found in patients with either low or high blood creatine kinase and also, with a low score on the clinical grading scale. Thus, these parameters cannot be used with certainty to predict MHS. We conclude that the CHCT protocol described by the North America MH Group contributed to identification of MHS in suspected individuals at an MH center in Brazil with 100 percent sensitivity and 65.7 percent specificity.

Use of the caffeine-halothane contracture test for the diagnosis of malignant hyperthermia in Brazil.

Malignant hyperthermia (MH) is a pharmacogenetic disease triggered by volatile anesthetics and succinylcholine. Deaths due to MH have been reported in Brazil. The first Malignant Hyperthermia Diagnostic and Research Center in Latin America was inaugurated in 1993 at the Federal University of Rio de Janeiro, Brazil. The center followed the diagnostic protocols of the North America MH Group, in which the contractures of biopsies from the vastus lateralis muscle are analyzed after exposure to caffeine and halothane (CHCT). CHCT was performed in individuals who survived, their relatives and those with signs/symptoms somewhat related to MH susceptibility (MHS). Here, we report data from 194 patients collected over 16 years. The Southeast (N = 110) and South (N = 71) represented the majority of patients. Median age was 25 (4-70) years, with similar numbers of males (104) and females (90). MHS was found in 90 patients and 104 patients were normal. Abnormal responses to both caffeine and halothane were observed in 59 patients and to caffeine or halothane in 20 and 11 patients, respectively. The contracture of biopsies from MHS exposed to caffeine and halothane was 1.027 +/- 0.075 g (N = 285) and 4.021 +/- 0.255 g (N = 226), respectively. MHS was found in patients with either low or high blood creatine kinase and also, with a low score on the clinical grading scale. Thus, these parameters cannot be used with certainty to predict MHS. We conclude that the CHCT protocol described by the North America MH Group contributed to identification of MHS in suspected individuals at an MH center in Brazil with 100% sensitivity and 65.7% specificity.

Increased resistance to hydrogen peroxide-induced cardiac contracture is associated with decreased myocardial oxidative stress in hypothyroid rats.

The purpose of this study was to determine whether decreased oxidative stress would increase the resistance to cardiac contracture induced by H(2)O(2) in hypothyroid rats. Male Wistar rats were divided into two groups: control and hypothyroid. Hypothyroidism was induced via thyroidectomy. Four weeks post surgery, blood samples were collected to perform thyroid hormone assessments, and excised hearts were perfused at a constant flow with or without H(2)O(2) (1 mmol/L), being divided into two sub-groups: control, hypothyroid, control + H(2)O(2), hypothyroid + H(2)O(2). Lipid peroxidation (LPO) was evaluated by chemiluminescence (CL) and thiobarbituric acid reactive substances (TBARS) methods, and protein oxidation by carbonyls assay in heart homogenates. Cardiac tissue was also screened for superoxide dismutase (SOD), catalase (CAT), glutathione peroxidase (GPx) activities, and for total radical-trapping antioxidant potential (TRAP). Analyses of SOD and glutathione-S-transferase (GST) protein expression were also performed in heart homogenates. Hypothyroid hearts were found to be more resistant to H(2)O(2)-induced contracture (60% elevation in LVEDP) as compared to control. CL, TBARS, carbonyl, as well as SOD, CAT, GPx activities and TRAP levels were reduced (35, 30, 40, 30, 16, 25, and 33%, respectively) in the cardiac homogenates of the hypothyroid group as compared to controls. A decrease in SOD and GST protein levels by 20 and 16%, respectively, was also observed in the hypothyroid group. These results suggest that a hypometabolic state caused by thyroid hormone deficiency can lead to an improved response to H(2)O(2) challenge and is associated with decreased oxidative myocardial damage.

Effects of hyaluronan and iodine on wound contraction and granulation tissue formation in rat skin wounds.

BACKGROUND: Hyaluronan (HA) plays an important role in the repair of damaged skin and has been used for the treatment of wounds. Iodine is a mild topical antiseptic. AIM: A mixture of high molecular weight HA with the iodine complex KI(3) (hyiodine) was reported to accelerate wound healing in patients with diabetes and patients after surgery. We investigated how this mixture affects wound contraction, granulation tissue (GT) and wound edges in excision skin wounds in rats. METHODS: Hyiodine was applied to full-thickness wounds made on the back of rats. The areas of the contracting wounds were calculated from digital photographs. The moving edges of the wound were studied by histological methods. The properties of GT were studied in wounds in which contraction was prevented by the insertion of plastic rings. The effects of hyiodine were compared with those of high molecular weight (1200 kDa) HA, low molecular weight (11 kDa) HA and KI(3) solution. RESULTS: Hyiodine accelerated wound contraction significantly in the first 5 days of healing. On day 3, hyiodine-treated wounds had reduced to 63% of the original area, whereas the wound area in saline-treated animals was 75% of the original size. The proliferating epidermis was thicker in hyiodine-treated animals on day 7. In the wounds with inserted rings, hyiodine caused little change in GT, but the weight of the crust/exudate formed on the top of the wound was increased by 351% compared with only minor changes caused by the hyiodine components alone. CONCLUSIONS: Hyiodine supports wound healing by stimulating wound contraction and epidermal proliferation and by keeping the wound moist through increased exudation.

[Severe side effects after Octenisept irrigation of penetrating wounds in children]. / Schwere Nebenwirkungen nach Octenisept-Spülung von Perforationswunden im Kindesalter.

BACKGROUND: Deep penetrating wounds in children's hands are repeatedly treated in emergency wards conservatively through irrigation, antibiotic therapy and splint immobilisation. After we had seen severest phlegmonous reactions after irrigation with Octenisept followed by long troublesome histories we would like to warn against using this antiseptic agent for irrigation of wounds. We give an overview about the significance of antiseptics and the use of antibiotics in the treatment of deeper contaminated wounds. PATIENTS AND METHODS: Between 2003 and 2007, 5 children (aged 2 to 8 years) were treated for sequelae of local wound irrigation with Octenisept in perforating hand injuries. We describe the early and medium-term aspects after irrigation, the further development, therapeutic measures, long-term damages and necessary reconstructions. We present the results of bacteriological smear tests, laboratory reports and histological examinations as well as allergy tests. RESULTS: All children showed more or less identical hand appearances. Hands were swollen caused by an interstitial oedema, compartment pressures were increased and hand function was completely suspended. The oedemas persisted for weeks and were hardly controllable. Especially serious were injuries at thenar level and in the first web space. Long-term sequelae were contractures caused by fibrotic muscle changes. Neither through bacteriological nor histological analysis were hints of bacterial or viral infections found. An allergic reaction to Octenisept could be excluded in the 3 most heavily affected children by an ROAT test. CONCLUSION: To prevent damage, contaminated wounds should be operatively debrided and not be irrigated with an antiseptic liquid. Octenisept seems to have a toxic effect in non-distinguished tissue. Because of a slow resorption it remains for a long time in the tissue. For therapy we recommend fasciotomy of the mid-hand and probably finger compartments, followed by compression treatment, physiotherapy with lymphatic drainage, dynamic and static splints.

Multiple pulley rupture following corticosteroid injection for trigger digit: case report.

We report a case of pulley rupture following repeated local corticosteroid injections for trigger digit. The treatment involved exploration, tenolysis, and reconstruction using the palmaris longus tendon.

Flexion contracture of the elbow due to phlebosclerosis induced by anti-cancer drug infusion.

This case report describes a patient with elbow contracture due to phlebosclerosis induced by anti-cancer drug infusion. Limitation of elbow extension was completely relieved by surgical excision of the sclerotic vein.

Severe bladder contracture leading to cystectomy after intravesical mitoxantrone chemotherapy.

Since the early 1990s, mitoxantrone has been used as a chemotherapeutic agent for adjuvant intravesical treatment following transurethral resection of superficial transitional cell carcinomas of the bladder. Although its efficacy as adjuvant intravesical therapy remains questionable and its use has not gained wide acceptance, the safety profile of the drug has been reported as favorable. We report the first case of mitoxantrone-induced severe bladder contracture leading to a completely nonfunctional bladder after intravesical administration of the drug. Cystectomy and urinary diversion were the final consequences for the patient.

Imaging of the superior rectus in superior rectus overaction after retrobulbar anesthesia.

OBJECTIVE: Vertical diplopia after cataract surgery caused by an overaction of an extraocular muscle is more common when the superior rectus muscle is involved, whereas contracture is more common when the inferior rectus muscle is involved. However, no documented imaging has been presented. The aim of this report was to study the superior rectus in such a patient with magnetic resonance imaging (MRI). DESIGN: Observational case report. METHODS: Ophthalmologic examination and thin-sectioned MRI across the superior rectus muscle were performed in a patient with superior rectus overaction after cataract surgery. MAIN OUTCOME MEASURES: Ocular alignment, ocular movement, and the superior rectus muscle on MRI. RESULTS: Magnetic resonance imaging disclosed focal thickening of the superior rectus muscle near the orbital apex in a patient who showed superior rectus overaction after retrobulbar anesthesia for cataract extraction. CONCLUSIONS: The focal thickening of the superior rectus muscle in this patient is consistent with the theory that segmental contracture leads to overactive muscles after retrobulbar anesthesia.

Spontaneous recovery from inferior rectus contracture (consecutive hypotropia) following local anesthetic injury.

We present yet another case of apparent inferior rectus paralysis following retrobulbar anesthesia for cataract surgery. He initially had a typical course but, unusually, after developing hypotropia and presumed contracture, went on to spontaneously recover. We are unaware of any other such case described in the literature. Methods to enhance this more desirable course are considered.

Neuroleptic malignant syndrome in Kufs' disease.

A patient with adult neuronal ceroid lipofuscinosis (ANCL; Kufs' disease) is described in whom neuroleptic malignant syndrome occurred, initially presenting as catatonic syndrome. Comprehensive neuroimaging studies were conducted including FDG-PET, IBZM-SPECT, and beta-CIT-SPECT, electrophysiological examinations and an ex vivo contracture test exposing muscle biopsy specimens to neuroleptics. Collectively the results argued for an involvement of the muscle in neuroleptic malignant syndrome at least in ANCL.

Pseudoankylosis of the mandible as a result of methyl methacrylate-induced inflammatory cicatricial contracture of the temporal muscle after cranioplasty.

Pseudoankylosis of the mandible after intracranial surgical procedure has been widely reported, and is usually caused by fibrosis of the temporal muscle as a result of injury during the operation. We present an unusual case of mandibular pseudoankylosis as a result of methyl methacrylate-induced aseptic inflammatory cicatricial contracture of the temporal muscle after cranioplasty.