Association between types of abdominopelvic cancer in patients with situs inversus total: Systematic review.

BACKGROUND: Situs inversus is a rare congenital anatomical variant that involves a group of anomalies regarding the arrangement of intrathoracic and intraabdominal organs. Being able to find in the abdominal region the liver, gallbladder, inferior vena cava, and head of the pancreas and ascending colon on the left side of the abdomen, while on the right side there is the spleen, the stomach, the body of the pancreas, the ligament of Treitz, descending colon among others. In this same way, the thoracic organs, lungs and heart, are changed in their position in a mirror translocation. METHODS: We systematically searched MEDLINE, Web of Science, Google Scholar, CINAHL, Scopus, and LILACS; the search strategy included a combination of the following terms: "Situs inversus," "Situs inversus totalis," "Cancer," "Neoplasm," "Abdominopelvic regions," and "clinical anatomy." RESULTS: Within the 41 included studies, 46 patients with situs inversus who had cancer, in addition to being found in this organ and in these regions, we also found as a result that the majority of the studies in the research were in stage II; finally, no one study could assert the direct relationship between the situs inversus totalis and the cancer. CONCLUSION: If our hallmarks could make us think that more exhaustive follow-up of the stomach and other organs should be carried out in these patients, there could also be other predisposing factors for cancer, which is why more studies are suggested to give future diagnostic and treatment guidelines treatment.

Primary abdominal gas gangrene: a report of two autopsy cases

Primary hepatic gas gangrene is a form of primary abdominal gas gangrene. The condition is caused by Clostridium perfringens, other clostridia, and non-clostridia bacterial species producing gas. Unlike classical gas gangrene or myonecrosis, the disease develops without a wound or a port of entry. Instead, gas-producing bacteria in the gastrointestinal tract colonize an underlying pathological process with foci of necrosis, producing excessive gas and spreading hematogenously to other organs. Herein we present two autopsy cases of primary hepatic gas gangrene diagnosed on autopsy, with the gross and histological changes that can be considered specific for this rare condition. Both patients had severe underlying liver disease-prone for this entity development. The gross changes in the cases are postmortem subcutaneous emphysema, skin bullae with pooled blood, pneumothorax, pneumoabdomen, abundant gas in the circulatory system, porous structure of the internal organs (tissue gas bubbles), and advanced tissue lysis, not corresponding to the post mortem time. Histology showed optically empty areas of varying size in the internal organs, which weave the structure of the organs and rod-shaped bacteria with scarcity or complete absence of inflammatory reaction.

Incisional hernia after surgical correction of abdominal congenital anomalies in infants: a systematic review with meta-analysis.

Incisional hernia (IH) in children could result in life-threatening complications, including incarceration and bowel strangulation. The incidence and risk factors of IH in infants are scarcely reported. Since IH-correction may require extensive surgery and a long recovery program, identifying infants and birth defects at risk, may lead to a different approach during the primary surgery. Therefore, the aim of this review is to systematically review the available data on the incidence of IH following surgery for congenital anomalies in infants. All studies describing IH were considered eligible. PubMed and Embase were searched and risk of bias was assessed. Primary outcome was the incidence of IH, secondary outcomes were difference in IH occurrence between disease severity (complex vs simple) and closure method (SILO vs primary closure) in gastroschisis patients. A meta-analysis was performed to pool the reported incidences in total and per congenital anomaly separately. Subgroup analysis within gastroschisis articles was performed. The 50 included studies represent 3140 patients. The pooled proportion of IH was 0.03 (95% CI 0.02-0.05; I2 = 79%, p ≤ 0.01) all anomalies combined. Gastroschisis (GS) reported highest pooled proportion 0.10 (95% CI 0.06-0.17; n = 142/1273; I2 = 86%; p ≤ 0.01). SILO closure (OR 3.09) and simple gastroschisis, i.e. without additional anomalies, (OR 0.18) were of significant influence. This review reports the incidence of IH in infants with different congenital abdominal anomalies, of which gastroschisis reported the highest risk. In GS patients, complex GS and SILO closure are risk factors for IH development.

Anatomy-based diagnostic criteria for complex body wall anomalies (CBWA).

BACKGROUND: Precise diagnosis and classification of CBWA cases can be challenging. BSA are considered when there is a body wall anomaly, skeletal abnormalities, and the umbilical cord is anomalous, absent or rudimentary, and LBWC when there is a body wall and structural limb anomalies with or without craniofacial abnormalities. METHODS: PubMed was searched for body stalk anomalies, limb body wall complex, body stalk anomalies and amniotic band syndrome, and limb body wall complex and amniotic band syndrome. Sixty nine articles were selected and reviewed. This article systematically classifies the variants of CBWA in 218 cases, the study is based on the embryological and anatomical criteria established by Martín-Alguacil and Avedillo to study BSA in the pig. RESULTS: Eight different BSA presentation were defined. One hundred and eighty nine cases were classified as BSA, from which five were Type I, nine Type II, 20 Type III, 57 Type IV, 11Type V, 24 Type VI, 11 Type VII, and 52 Type VIII. Twenty six cases presented cranial phenotype, 114 abdominal phenotype, 42 cranio/abdominal overlapping phenotype, and five without defined phenotype. In addition, 52 BSA cases presented some kind of spinal dysraphism (SPDYS) and were classified as BSA/SPDYS, most of these cases did not show structural limb anomalies, except for three cases and were classified as LBWC/SPDYS. CONCLUSION: This morphology-based classification represents a useful tool for clinical diagnosis, it helps to quantify and to evaluate CBWA in a precise, objective manner.

Idiopathic cocoon abdomen with congenital colon malrotation: a case report and review of the literature.

BACKGROUND: Cocoon abdomen is a relatively rare abdominal disease characterized by the total or partial encasement of the small intestinal by a dense fibro-collagenous membrane. CASE PRESENTATION: We reported an unusual case of idiopathic cocoon abdomen with congenital colon malrotation. Laparotomy and sac release were performed on the patient. The patient was no recurrence 6 months after operation. A literature review was also performed. CONCLUSION: Preoperative diagnosis of abdominal cocoon is difficult. A careful history, physical examination and appropriate radiology may be helpful in making a definitive diagnosis. If conservative treatment can't relieve symptoms effectively, surgery is currently considered to be important in the management of this disease.

Body stalk anomalies in pig-Definition and classification.

The presence of body wall closing defects (abdominoschisis and thoracoabdominoschisis) in combination with other congenital malformations was studied in the pig (Sus scrofa domesticus). After clinical examination and literature review, body wall defects with multiple congenital anomalies in eight pigs were described, and classified using anatomical and embryological criteria. Several BSA presentations were identified and classified as follows: (a) BSA Type I: fetus with spinal and UC defects, thoracoabdominoschisis, anal atresia and/or other internal organs structural defects, and structural limb defects; (b) BSA Type II: fetus with spinal and UC defects, thoracoabdominoschisis, anal atresia and/or other internal organs structural defects, and nonstructural limb defects; (c) BSA Type III: fetus with spinal and UC defects, abdominoschisis, anal atresia and/or other internal organs structural defects, and structural limb defects; and (d) BSA Type IV: fetus with spinal and UC defects, abdominoschisis, anal atresia and/or other internal organs structural defects, and nonstructural limb defects. Two types of LBWC were differentiated: LBWC Type I: characterized by thoracoabdominoschisis and structural limb defects, and LBWC Type II: characterized by abdominoschisis and structural limb defects, corresponding to BSA type I and type III. This is the first report on BSA and LBWC in the pig.

Abdominal Actinomycosis misdiagnosed as liposarcoma

Actinomycosis is an uncommon, endogenous, and chronic infection with varied and nonspecific clinical features such as abdominal, pelvic or cervical masses, ulcerative lesions, abscesses, draining fistula, fibrosis, and constitutional symptoms. The disease ensues when the bacteria disrupt the mucosal barrier, invade, and spread throughout interfascial planes. Currently, the diagnosis of actinomycosis is challenging because of its very low frequency and depending on the clinical presentation it may masquerade malignancies. Therapy consists initially in intravenous penicillin, followed by an oral regimen that may be extended until a year of treatment. A timely diagnosis is crucial to avoid extensive therapeutic attempt as surgery. However, a biopsy or drainage of abscesses and fistula's tract may be required not only as a diagnostic procedure as part of the therapy. We report the case of a 72-year-old woman with an abdominal mass initially misdiagnosed as a liposarcoma. A second biopsy of a skin lesion of the abdominal wall made the diagnosis of actinomycosis, avoiding a major surgical procedure. The patient was treated with a long-term course of antibiotics with favorable outcome. Liposarcoma was ruled out after the patient's full recovery with antibiotics and the misdiagnosis was credit to the overconfidence on the immunohistochemical positivity to MDM2.

Management of delivery of a fetus with autosomal recessive polycystic kidney disease: a case report of abdominal dystocia and review of the literature.

BACKGROUND: Autosomal recessive renal polycystic kidney disease occurs in 1 in 20,000 live births. It is caused by mutations in both alleles of the PKHD1 gene. Management of delivery in cases of suspected autosomal recessive renal polycystic kidney disease is rarely discussed, and literature concerning abdominal dystocia is extremely scarce. We present a case of a patient with autosomal recessive renal polycystic kidney disease whose delivery was complicated by abdominal dystocia, and we discuss the factors that determined the route and timing of delivery. CASE PRESENTATION: A 23-year-old Caucasian woman, G2 P0, with a prior unremarkable pregnancy was referred to our tertiary center at 31 weeks of gestation because of severe oligoamnios (amniotic fluid index = 2) and hyperechogenic, dedifferentiated, and enlarged fetal kidneys. She had no other genitourinary anomaly. Fetal magnetic resonance imaging showed enlarged, hypersignal kidneys and severe pulmonary hypoplasia. We had a high suspicion of autosomal recessive renal polycystic kidney disease, and after discussion with our multidisciplinary team, the parents opted for conservative care. Ultrasound performed at 35 weeks of gestation showed a fetal estimated weight of 3550 g and an abdominal circumference of 377 mm, both above the 90th percentile. Because of the very rapid kidney growth and suspected risk of abdominal dystocia, we proposed induction of labor at 36 weeks of gestation after corticosteroid administration for fetal lung maturation. Vaginal delivery was complicated by abdominal dystocia, which resolved by continuing expulsive efforts and gentle fetal traction. A 3300-g (P50-90) male infant was born with Apgar scores of 1-7-7 at 1, 5, and 10 minutes, respectively, and arterial and venous umbilical cord pH values of 7.23-7.33. Continuous peritoneal dialysis was started at day 2 of life because of anuria. Currently, the infant is 1 year old and is waiting for kidney transplant that should be performed once he reaches 10 kg. Molecular analysis of PKHD1 performed on deoxyribonucleic acid (DNA) from the umbilical cord confirmed autosomal recessive renal polycystic kidney disease. CONCLUSIONS: Management of delivery in cases of suspected autosomal recessive renal polycystic kidney disease needs to be discussed because of the risk of abdominal dystocia. The route and timing of delivery depend on the size of the fetal abdominal circumference and the gestational age. The rate of kidney growth must also be taken into account.

Physiological parameters for Prognosis in Abdominal Sepsis (PIPAS) Study: a WSES observational study.

Background: Timing and adequacy of peritoneal source control are the most important pillars in the management of patients with acute peritonitis. Therefore, early prognostic evaluation of acute peritonitis is paramount to assess the severity and establish a prompt and appropriate treatment. The objectives of this study were to identify clinical and laboratory predictors for in-hospital mortality in patients with acute peritonitis and to develop a warning score system, based on easily recognizable and assessable variables, globally accepted. Methods: This worldwide multicentre observational study included 153 surgical departments across 56 countries over a 4-month study period between February 1, 2018, and May 31, 2018. Results: A total of 3137 patients were included, with 1815 (57.9%) men and 1322 (42.1%) women, with a median age of 47 years (interquartile range [IQR] 28-66). The overall in-hospital mortality rate was 8.9%, with a median length of stay of 6 days (IQR 4-10). Using multivariable logistic regression, independent variables associated with in-hospital mortality were identified: age > 80 years, malignancy, severe cardiovascular disease, severe chronic kidney disease, respiratory rate ≥ 22 breaths/min, systolic blood pressure < 100 mmHg, AVPU responsiveness scale (voice and unresponsive), blood oxygen saturation level (SpO2) < 90% in air, platelet count < 50,000 cells/mm3, and lactate > 4 mmol/l. These variables were used to create the PIPAS Severity Score, a bedside early warning score for patients with acute peritonitis. The overall mortality was 2.9% for patients who had scores of 0-1, 22.7% for those who had scores of 2-3, 46.8% for those who had scores of 4-5, and 86.7% for those who have scores of 7-8. Conclusions: The simple PIPAS Severity Score can be used on a global level and can help clinicians to identify patients at high risk for treatment failure and mortality.

Preoperative computerized tomography-guided blue dye localization for metastatic lymphadenopathy and peritoneal tumor implants during laparoscopic surgery: Two case reports.

RATIONALE: Computerized tomography (CT)-guided blue dye localization has been widely discussed for preoperative localization of pulmonary nodules. However, few studies have investigated this technique for intra-abdominal lesions. Although preoperative localization is not commonly required in laparotomy, it may assume importance with advancements in the field of laparoscopic surgery. PATIENT CONCERNS: Herein, we report the cases of 2 patients diagnosed with colon cancer who underwent hemicolectomy with extended lymphadenectomy and subsequent chemotherapy. DIAGNOSES: Follow-up CT scans showed newly developed metastatic lymphadenopathy and peritoneal tumor implants. INTERVENTIONS: Considering the difficulty in identification of and access to the target lesions during laparoscopic surgery, preoperative CT-guided blue dye localization was performed in both cases. OUTCOMES: All the target lesions were identified by the dye marker and removed successfully. The pathologic results revealed adenocarcinoma. LESSONS: We established the following strategy for preoperative CT-guided dye localization of intra-abdominal lesions:Intra-abdominal lesions that are hard to identify due to their size or morphology, and difficult to approach due to their location or surrounding structures, maybe the candidates for this procedure, especially in cases of laparoscopic surgery.Operators should adjust their localization planning based on the surgery method, cutting path, and location of port sites. The target dye marker should be clearly visible in the presumed intra-operative field of view.A second dye marker should be made to ensure surgical success when the target dye marker is obscured by the surrounding structures in the presumed intra-operative field of view.

Onfaloplastia em triângulo isósceles e com dupla fixação na abdominoplastia / Omphaloplasty based on an isosceles triangle with double fixation in abdominoplasty

Introdução: Na abdominoplastia convencional, a cicatriz do novo umbigo representa o ponto de maior desafio. Em sua execução, já foram descritas e utilizadas várias técnicas e táticas cirúrgicas, com resultados nem sempre satisfatórios, sob o ponto de vista do paciente e também do médico. O objetivo é demonstrar a aplicabilidade e satisfação com a onfaloplastia em triângulo isósceles e com dupla fixação na abdominoplastia. Métodos: Foram selecionadas 97 pacientes do sexo feminino, com idades entre 25 e 65 anos. Todas foram submetidas à dermolipectomia abdominal clássica associada à lipoaspiração moderada de todo abdome anterior e flancos e avaliadas com 90, 180 e 360 dias pós-operatórios, pelo mesmo cirurgião. Resultados: Observou-se um índice de resultados satisfatórios das cicatrizes umbilicais na maioria dos casos (92,8%). Algumas cicatrizes umbilicais apresentaram estenoses (3,1%) e outras, cicatrizes inestéticas (4,1%). Não se observaram necroses. Conclusão: A utilização desta técnica demonstrou ser eficaz, de fácil execução e com resultados muito satisfatórios na estética da cicatriz umbilical nas dermolipectomias abdominais.

Introduction: In conventional abdominoplasty, the creation of a new umbilical scar is challenging. Several surgical techniques and approaches have previously been described and applied, but not always with satisfactory results. The objective is to demonstrate the applicability and satisfaction with omphaloplasty based on an isosceles triangle with double fixation in abdominoplasty. Methods: The study included 97 female patients aged between 25 and 65 years. All underwent classic abdominal dermolipectomy with moderate abdominal liposuction of the entire anterior abdomen and flanks by the same surgeon and were evaluated at 90, 180, and 360 days postoperatively. Results: Patients were satisfied with the umbilicus in most cases (92.8%). Some umbilical scars had contracted (3.1%) and others appeared unsightly (4.1%). No necrosis was observed. Conclusion: This technique was effective and easy to perform, with satisfactory umbilical scar aesthetic outcomes in abdominal dermolipectomy.

Use of Magnetic Resonance Imaging in Evaluating Fetal Brain and Abdomen Malformations during Pregnancy.

Magnetic resonance imaging (MRI) is used as a clarifying technique after a high-resolution ultrasound examination during pregnancy. Combining ultrasound with MRI, additional diagnostic information is obtained or ultrasound diagnosis is frequently corrected. High spatial resolution provides accurate radiological imaging of internal organs and widens possibilities for detecting perinatal development disorders. The safety of MRI and the use of intravenous contrast agent gadolinium are discussed in this article. There is no currently available evidence that MRI is harmful to the fetus, although not enough research has been carried out to prove enduring safety. MRI should be performed when the benefit outweighs the potential side effects. The narrative review includes several clinical cases of fetal MRI performed in Vilnius University Hospital Santaros Clinics.

A novel ISLR2-linked autosomal recessive syndrome of congenital hydrocephalus, arthrogryposis and abdominal distension.

ISLR2 (immunoglobulin superfamily containing leucine-rich repeat 2), encodes a protein involved in axon guidance in brain development (hence the other name leucine-rich repeat domain- and immunoglobulin domain-containing axon extension proteins; LINX). A recently described mouse knockout displays hydrocephalus. However, the corresponding phenotype in humans is unknown. Here, we describe a multiplex consanguineous family in which a homozygous truncating variant in ISLR2 segregates with severe congenital hydrocephalus, arthrogryposis multiplex congenita and abdominal distension. We suggest this syndrome may represent the human "knockout" phenotype for ISLR2.

Brida amniótica abdominal, reporte de un caso / Precordial pain in pediatrics. Update

Existe un amplio espectro de deformidades causadas por bandas amnióticas, siendo las extremidades la ubicación más común. La banda de constricción en el abdomen es un lugar raro para el síndrome de brida amniótica. Presentamos un caso de banda de constricción circunferencial congénita del abdomen sin otras malformaciones. La condición no afectó la ventilación, la alimentación, las deposiciones o el crecimiento. Se planificaron múltiples Z-Plasties en el abdomen antes del primer mes de vida. Revisamos la literatura que hace hincapié en la presentación clínica y el tratamiento de esta rara entidad clínica.

There is a wide spectrum of deformities caused by amniotic bands, being the extremities the most common location. The constriction band in the abdomen is a rare location for constriction band syndrome. We report a case of congenital circumferential constriction band of abdomen without other malformations. The condition did not affect ventilation, feeding, bowel movements, or growth. Multiple Z-plasties were planned in the abdomen before the first month from birth. We reviewed the literature emphasizing on the clinical presentation and management of this rare clinical entity.

Percutaneous sonographically assisted endoscopic gastrostomy for difficult cases with interposed organs.

OBJECTIVES: The aim of this retrospective observational study was to clarify the usefulness and safety of percutaneous sonographically assisted endoscopic gastrostomy or duodenostomy (PSEGD) using the introduction method. METHODS: The information for the sequential 22 patients who could not undergo standard percutaneous endoscopic gastrostomy (PEG) and underwent PSEGD for 3 y was extracted and was reviewed. In standard PEG, we performed pushing out of the stomach from the mediastinum and full distention to adhere the gastric wall to the peritoneal wall without interposing of the intraperitoneal tissues by air inflation and a turning-over procedure of the endoscope, four-point square fixation of the stomach to the peritoneal wall by using a Funada-style gastric wall fixation kit under diaphanoscopy, extracorporeal thumb pushing, and in difficult cases extracorporeal ultrasound guidance, and if necessary confirmation of fixation of the gastric wall to the peritoneal wall and placement of the PEG tube without any interposed tissues by using ultrasound. RESULTS: Twenty-one patients (95.5%) successfully underwent PSEGD. Early complications (more than grade 2 in Clavien-Dindo classification) just after the procedure occurred in one case (active oozing). We did not encounter a case with mispuncture of the intraperitoneal organs and tissues. Delayed complications occurring within 1 mo were pneumonia in five patients, including death in three cases; bleeding from puncture site in two patients; and atrial fibrilation in one patient. CONCLUSION: PSEGD using the introduction method is a useful procedure for difficult patients in whom intraperitoneal organ or tissue is suspected to be interposed between the abdominal wall and stomach.

Value of Fetal MRI in the Era of Fetal Therapy for Management of Abnormalities Involving the Chest, Abdomen, or Pelvis.

OBJECTIVE: The purpose of this article is to present illustrative cases highlighting the value of fetal MRI as a problem-solving tool in evaluating anomalies of the fetal chest, abdomen, and pelvis that are being evaluated for fetal therapy. CONCLUSION: Fetal MRI is an increasingly valuable noninvasive tool for evaluating fetal anomalies in the age of fetal therapy. Although ultrasound remains the primary diagnostic imaging modality, MRI can provide additional information to assist parental counseling and patient care.