Proteus syndrome: report of intra-abdominal lipomatosis.
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ABSTRACT:
SUBMITTER: Erginel B
PROVIDER: S-EPMC4335946 | biostudies-other | 2013 Jun
REPOSITORIES: biostudies-other
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Project description:BackgroundVascular Ehlers-Danlos syndrome has a high mortality rate due to hemorrhagic complications.Case presentationWe report a case of vascular-type Ehlers-Danlos syndrome diagnosed due to rupture of multiple celiac aneurysms. The patient was a 25-year-old Japanese man with a history of a sigmoid perforation. He was admitted to a nearby hospital because of abdominal pain. On day 9 of hospitalization, the patient experienced shock. Enhanced abdominal computed tomography revealed a hepatic aneurysm and intra-abdominal bleeding, and the patient was transferred to our hospital. Emergency abdominal angiography revealed multiple aneurysms in the celiac, common, and right hepatic arteries. The right hepatic artery was considered responsible and was embolized. The patient had characteristic physical findings of the syndrome, aiding in confirming the genetic analysis of COL3A1 gene abnormality.ConclusionJuvenile-onset colonic perforation and rupture of the celiac arteries are key findings in the suspicion of vascular-type Ehlers-Danlos syndrome.
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Project description:BackgroundIntra-abdominal hypertension is frequently present in critically ill patients and is an independent predictor for mortality. Risk factors for intra-abdominal hypertension and abdominal compartment syndrome have been widely investigated. However, data are lacking on prevalence and outcome in high-risk patients. Our objectives in this study were to investigate prevalence and outcome of intra-abdominal hypertension and abdominal compartment syndrome in high-risk patients in a prospective, observational, single-center cohort study.ResultsBetween March 2014 and March 2016, we included 503 patients, 307 males (61%) and 196 females (39%). Patients admitted to the intensive care unit with a diagnosis of pancreatitis, elective or emergency open abdominal aorta surgery, orthotopic liver transplantation, other elective or emergency major abdominal surgery and trauma were enrolled. One hundred and sixty four (33%) patients developed intra-abdominal hypertension and 18 (3.6%) patients developed abdominal compartment syndrome. Highest prevalence of abdominal compartment syndrome occurred in pancreatitis (57%) followed by orthotopic liver transplantation (7%) and abdominal aorta surgery (5%). Length of intensive care stay increased by a factor 4 in patients with intra-abdominal hypertension and a factor 9 in abdominal compartment syndrome, compared to patients with normal intra-abdominal pressure. Rate of renal replacement therapy was higher in abdominal compartment syndrome (38.9%) and intra-abdominal hypertension (8.2%) compared to patients with normal intra-abdominal pressure (1.2%). Both intensive care mortality and 90-day mortality were significantly higher in intra-abdominal hypertension (4.8% and 15.2%) and abdominal compartment syndrome (16.7% and 38.9%) compared to normal intra-abdominal pressure (1.2% and 7.1%). Body mass index (odds ratio 1.08, 95% confidence interval 1.03-1.13), mechanical ventilation at admission (OR 3.52, 95% CI 2.08-5.96) and Apache IV score (OR 1.03, 95% CI 1.02-1.04) were independent risk factors for the development of intra-abdominal hypertension or abdominal compartment syndrome.ConclusionsThe prevalence of abdominal compartment syndrome was 3.6% and the prevalence of intra-abdominal hypertension was 33% in this cohort of high-risk patients. Morbidity and mortality increased when intra-abdominal hypertension or abdominal compartment syndrome was present. The patient most at risk of IAH or ACS in this high-risk cohort has a BMI > 30 kg/m2 and was admitted to the ICU after emergency abdominal surgery or with a diagnosis of pancreatitis.
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Project description:BackgroundGeneral pathophysiological mechanisms regarding associations between fluid administration and intra-abdominal hypertension (IAH) are evident, but specific effects of type, amount, and timing of fluids are less clear.ObjectivesThis review aims to summarize current knowledge on associations between fluid administration and intra-abdominal pressure (IAP) and fluid management in patients at risk of intra-abdominal hypertension and abdominal compartment syndrome (ACS).MethodsWe performed a structured literature search from 1950 until May 2021 to identify evidence of associations between fluid management and intra-abdominal pressure not limited to any specific study or patient population. Findings were summarized based on the following information: general concepts of fluid management, physiology of fluid movement in patients with intra-abdominal hypertension, and data on associations between fluid administration and IAH.ResultsWe identified three randomized controlled trials (RCTs), 38 prospective observational studies, 29 retrospective studies, 18 case reports in adults, two observational studies and 10 case reports in children, and three animal studies that addressed associations between fluid administration and IAH. Associations between fluid resuscitation and IAH were confirmed in most studies. Fluid resuscitation contributes to the development of IAH. However, patients with IAH receive more fluids to manage the effect of IAH on other organ systems, thereby causing a vicious cycle. Timing and approach to de-resuscitation are of utmost importance, but clear indicators to guide this decision-making process are lacking. In selected cases, only surgical decompression of the abdomen can stop deterioration and prevent further morbidity and mortality.ConclusionsCurrent evidence confirms an association between fluid resuscitation and secondary IAH, but optimal fluid management strategies for patients with IAH remain controversial.
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Project description:Proteus syndrome was originally described by Cohen and Hayden in 1979. The disorder was named Proteus syndrome by Wiedmann and colleagues in 1983 after Proteus, the giant Greek god of the sea. Proteus syndrome is a rare, sporadic, congenital polymorphic condition. Approximately 200 cases have been reported in the literature, but none has been associated with anal bleeding from hemorrhoids. We describe the case of a 21-year-old man with Proteus syndrome with severe anal bleeding. A hemorrhoidectomy was assumed to be too risky because of the massive venous abnormalities seen on CT. The patient was successfully treated by Doppler-guided haemorrhoidal artery ligation (DG-HAL). Six months after surgery, the patient has had no further episodes of anal bleeding.
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Project description:Abdomen, a closed compartment, is prone to raised intra-abdominal pressure (IAP) in the postoperative period. After a critical value of ? 15 cm of water, IAP produces abdominal compartment syndrome (ACS). ACS leads to reduced venous return, reduced cardiac output, and domino effect of organ dysfunction, leading to death. Hence, it is the need of hour to monitor IAP to pick up intra-abdominal hypertension (IAH) and ACS. This routine facilitates early institution of treatment measures.To study IAP in abdominal operations in neonates, infants, and older children and to promote concept of routine measurement of IAP as standard care.Intravesical route was used to measure IAP in this prospective observational study. Seventy-nine pediatric abdominal surgeries met with criteria of availability of complete data for analysis and formed the cohort of the study. All major, infective, traumatic, tumor-related abdominal surgeries were included in the study. Outcome, C-reactive protein (CRP), procalcitonin, platelet counts, Simplified Sequential Organ Failure Assessment Score, and Acute Physiology and Chronic Health Evaluation II (APACHE II) score were the parameters analyzed. The World Society of ACS grading was adopted in the study with subdivision of normal into low-normal and high-normal subgroups.Extended Mantel-Haenszel Chi-square statistical tool when applied for linear relationship showed a linear relationship with outcome (P < 0.05), CRP (P < 0.05), procalcitonin (P < 0.05), Simplified Sequential organ failure Assessment Score, and APACHE II. Platelet counts (P > 0.05) were not significantly correlated. Decision for laparotomy was delayed in cases of ACS.Routine measure of IAP facilitates early recognition of IAH. This facilitates therapeutic measures to be initiated to reduce IAP. Early decision to decompress by laparotomy/laparostomy saves lives. Hence, routine IAP measurement should be a part of standard care in pediatric abdominal surgery.
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Project description:RationaleAggressive fibromatosis is a rare and locally infiltrative monoclonal fibroblastic proliferation with lack of metastatic potential. We describe a rare case of intra-abdominal aggressive fibromatosis on young female with hyperemesis.Patient concernsA 23-year-old female was admitted with hyperemesis and loss of weight.DiagnosesAccording to imaging findings and immunohistology findings, a diagnosis of intra-abdominal aggressive fibromatosis was formulated.OutcomesAfter the surgery, no evidence of local recurrence was noted during the 6 months of follow-up.LessonsAF may explain why pregnant women may have severe hyperemesis.
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Project description:Proteus syndrome (PS) is an extremely rare and sporadic disorder characterized by asymmetric and/or disproportionate overgrowth of limbs, hamartomas, and vascular malformations. The onset of overgrowth usually involves the skin, bone, fat, and other connective tissues in a patchy or mosaic pattern. Partial gigantism of the affected limb or digit is a pathognomonic sign of PS. Thus far, only a few cases of PS have been recorded in the literature. In the present report, a case of PS in a 35-year old woman with classic cerebriform plantar hyperplasia and macrodactyly of the left foot was documented. The clinical and molecular characteristics and differential diagnosis of PS are also discussed in this report.
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Project description:Proteus syndrome is a rare syndrome characterized by disproportionate overgrowth of limbs, multiple hamartomas, and vascular malformations. The cerebriform connective tissue nevi, also called cerebriform plantar hyperplasia, are present in most patients, and is the main characteristic of the syndrome. If present, even alone, they can be considered as a pathognomonic sign. This article reports a classic case of Proteus syndrome in a 2-year-old male patient who began to show a discrete asymmetry of the right hemibody in relation to the left one after birth, which increased over the months. He also showed cerebriform plantar hyperplasia and Port-wine stains, among other alterations.
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Project description:PurposeTo update the World Society of the Abdominal Compartment Syndrome (WSACS) consensus definitions and management statements relating to intra-abdominal hypertension (IAH) and the abdominal compartment syndrome (ACS).MethodsWe conducted systematic or structured reviews to identify relevant studies relating to IAH or ACS. Updated consensus definitions and management statements were then derived using a modified Delphi method and the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) guidelines, respectively. Quality of evidence was graded from high (A) to very low (D) and management statements from strong RECOMMENDATIONS (desirable effects clearly outweigh potential undesirable ones) to weaker SUGGESTIONS (potential risks and benefits of the intervention are less clear).ResultsIn addition to reviewing the consensus definitions proposed in 2006, the WSACS defined the open abdomen, lateralization of the abdominal musculature, polycompartment syndrome, and abdominal compliance, and proposed an open abdomen classification system. RECOMMENDATIONS included intra-abdominal pressure (IAP) measurement, avoidance of sustained IAH, protocolized IAP monitoring and management, decompressive laparotomy for overt ACS, and negative pressure wound therapy and efforts to achieve same-hospital-stay fascial closure among patients with an open abdomen. SUGGESTIONS included use of medical therapies and percutaneous catheter drainage for treatment of IAH/ACS, considering the association between body position and IAP, attempts to avoid a positive fluid balance after initial patient resuscitation, use of enhanced ratios of plasma to red blood cells and prophylactic open abdominal strategies, and avoidance of routine early biologic mesh use among patients with open abdominal wounds. NO RECOMMENDATIONS were possible regarding monitoring of abdominal perfusion pressure or the use of diuretics, renal replacement therapies, albumin, or acute component-parts separation.ConclusionAlthough IAH and ACS are common and frequently associated with poor outcomes, the overall quality of evidence available to guide development of RECOMMENDATIONS was generally low. Appropriately designed intervention trials are urgently needed for patients with IAH and ACS.
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