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.

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.

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.

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.

Subclinical thiamine deficiency in patients with abdominal cancer.

OBJECTIVE: Thiamine is an essential coenzyme for oxidative metabolisms; however, it is not synthesized in the human body, and the average thiamine storage capacity is approximately 18 days. Therefore, thiamine deficiency (TD) can occur in any condition of unbalanced nutrition. If TD is left untreated, it causes the neuropsychiatric disorder Wernicke encephalopathy (WE). Although WE is a medical emergency, it is sometimes overlooked because most patients with WE do not exhibit all of the typical symptoms, including delirium, ataxia, and ophthalmoplegia. If all of the typical clinical symptoms of WE are absent, diagnosis of TD or WE becomes more difficult. METHOD: From a series of cancer patients, we reported three patients who developed TD without the typical clinical symptoms of WE.ResultA 69-year-old woman with pancreatic body cancer receiving chemotherapy with paclitaxel and gemcitabine for six months. Her performance status (PS) was 1. A detailed interview revealed that she had appetite loss for six months. Another 69-year-old woman with ovarian cancer received nedaplatin; her PS was 0. A detailed interview revealed that she had appetite loss for three months. A 67-year-old woman with colon cancer receiving ramucirumab in combination with second-line fluorouracil with folinic acid and irinotecan. Her PS was 1. A detailed interview revealed that she had appetite loss for three weeks. None exhibited typical clinical signs of WE, but they developed appetite loss for six months, three months, and three weeks, respectively. The diagnosis of TD was supported by abnormally low serum thiamine levels.Significance of the resultsThis report emphasizes the possibility of TD in cancer patients even when patients do not develop typical clinical signs of WE. The presence of appetite loss for more than two weeks may aid in diagnosing TD. Patients receiving chemotherapy may be at greater risk for developing TD.

Co-existence of the double inferior vena cava with complex interiliac venous communication and aberrant common hepatic artery arising from superior mesenteric artery: a case report.

Variations of the arterial and venous system of the abdomen and pelvis have important clinical significance in hepatobiliary surgery, abdominal laparoscopy, and radiological intervention. A case of double inferior vena cava (IVC) with complex interiliac communication and variation of the common hepatic artery (CHA) arising from superior mesenteric artery (SMA) in a 79-year-old male cadaver is presented. Both IVCs ascended on either side of the abdominal aorta. The left-sided IVC crossed anterior to the aorta at the level of the left renal vein. The union of both IVCs was at the level just above the right renal vein. The diameter of right-sided IVC, left-sided IVC and the common IVC were 16.73 mm, 21.57 mm and 28.75 mm, respectively. In the pelvic cavity, the right common iliac vein was formed by a union of right external and internal iliac veins while the formation of left common iliac vein was from the external iliac vein and two internal iliac veins. An interiliac vein ran from right internal iliac vein to left common iliac vein with an additional communicating vein running from the middle of this interiliac vein to the right common iliac vein. Another co-existence variation in this case was the origin of the CHA arising from the SMA with a suprapancreatic retroportal course. Clinical importance of double IVC are observed in retroperitoneal surgery, whole organ transplantation or radical nephrectomy, surgical ligation of the IVC or the placement of an IVC filter for thromboembolic disease. The variation of CHA has an important clinical significance in liver transplantation, abdominal laparoscopy and radiological abdominal intervention. (Folia Morphol 2018; 77, 1: 151-155).

Knockdown of a putative argininosuccinate lyase gene reduces arginine content and impairs nymphal development in Nilaparvata lugens.

Nilaparvata lugens is a typical phloem feeder. Rice phloem is high in simple sugars and very low in essential amino acids. Nilaparvata lugens harbors an ascomycete Entomomyces delphacidicola that hypothetically biosynthesizes several amino acids to meet the nutrition requirement of the planthopper. Among these amino acids, here, we focused on arginine biosynthesis. A complete cDNA of an E. delphacidicola gene, arginine-succinate lyase, EdArg4, the last step in arginine biosynthesis, was obtained. RNAi-mediated suppression of EdArg4 reduced arginine content in the hemolymph, and decreased the expression of several arginine biosynthesis genes. Silencing of EdArg4 delayed nymphal development and led to nymphal lethality. About 20% of the EdArg4 RNAi surviving adults were deformed. The most obvious defect was wider and larger abdomen. The EdArg4 RNAi-treated planthoppers had thickened wings and enlarged antennae, legs, and anal tubes and a few adults did not normally emerge. Arginine deficiency in the EdArg4 RNAi planthoppers repressed nitric oxide signaling, determined at the transcriptional level. We infer that E. delphacidicola biosynthesizes essential arginine to compensate for nutrition deficiency in N. lugens.

Incidental Findings on Pediatric Abdominal Computed Tomography At A Pediatric Trauma Center.

BACKGROUND: The increasing availability and use of computed tomography (CT) in pediatric abdominal trauma has increased the detection of incidental findings. While some of these findings are benign, others may require further evaluation for possible clinical importance. OBJECTIVES: This study aimed to identify the frequency and type of incidental findings and their need for follow-up on abdominal CT in patients at a pediatric trauma center. METHODS: This was a retrospective, observational study on trauma patients ≤21 years of age who presented to the emergency department between January 1, 2004 and July 31, 2016 and underwent CT scans of the abdomen and pelvis. Findings were classified as benign anatomic variants, benign pathologic lesions, and pathologic lesions requiring additional work-up. RESULTS: There were 1073 patients included in the study population, with a mean age of 15.5 years; 707 (66%) were males. A total of 418 incidental findings were identified in 345 patients. Of these, 290 (69%) were benign and 60 (14%) were likely benign pathologic that required possible outpatient monitoring. Of those requiring additional evaluation, 5 (1%) patients warranted further evaluation before discharge. CONCLUSIONS: Nearly one-third of patients had at least one radiographic finding unrelated to their injury. Of these, more than two-thirds did not require additional evaluation, but nearly one-third of patients required some form of further work-up.

Aplasia cutis congenita in a setting of fetus papyraceus associated with small fetal abdominal circumference and high alpha-fetoprotein and amniotic acetylcholinesterase.

Fetus papyraceus is the fetal death of one or more fetuses in a multiparous pregnancy. The surviving infants can experience extensive aplasia cutis in an H-shaped distribution over the flanks and abdomen as a consequence of the loss of their fetal sibling. We report the case of a monochorionic, diamniotic pregnancy complicated by a single fetal death at 13 weeks of gestational age. Aplasia cutis of the surviving twin was suggested in utero by three criteria: high amniotic and maternal alpha-fetoprotein, detectable acetylcholinesterase, and small abdominal circumference on prenatal ultrasound. This constellation of findings in the setting of fetus papyraceus can be an indicator of aplasia cutis in the surviving fetus.

A comprehensive newborn exam: part II. Skin, trunk, extremities, neurologic.

Skin findings are common during the newborn examination. Although these findings are often benign, it is important to visualize the entire skin surface to distinguish these findings and appropriately reassure parents. The chest should be observed for symmetric movement, pectus excavatum, pectus carinatum, prominent xiphoid, or breast tissue. The infant should be as relaxed as possible so that the physician can more easily detect any abdominal masses, which are often renal in origin. A single umbilical artery may be associated with another congenital abnormality, especially renal anomalies, and intrauterine growth restriction and prematurity. Signs of ambiguous genitalia include clitoromegaly and fused labia in girls, and bilateral undescended testes, a micropenis, or a bifid scrotum in boys. Sacral dimples do not warrant further evaluation if they are less than 0.5 cm in diameter, are located within 2.5 cm of the anal verge, and are not associated with cutaneous markers; dimples that do not fit these criteria require ultrasonography to evaluate for spinal dysraphism. Brachial plexus injuries are most common in newborns who are large for gestational age, and physical therapy may be required to achieve normal function. Patients with abnormal findings on Ortolani and Barlow maneuvers should be evaluated further for hip dysplasia. It is also important to assess newborns for tone and confirm the presence of normal primitive reflexes.

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.

Fetus in fetu in a neonate: report of a case.

Fetus in fetu (FIF) is a rare condition in which a malformed fetus resides in the body of its host. The presence of a well-formed vertebral column secures the diagnosis of FIF and differentiates this entity from a teratoma. Although more than 100 cases of FIF have been reported in the literature, the presence of a well-formed vertebral column is not documented in many cases. We herein report the case of a 19-day-old female who presented with a lump in the abdomen. Plain radiograph of the abdomen showed the presence of a well-formed vertebral column with long bones, and the results were confirmed with ultrasonogram and CT scans. Therefore, a preoperative diagnosis of FIF was made, and the mass was successfully excised. Upon opening the sac of the mass, a malformed anencephalic fetus was found.

[Fetal magnetic resonance imaging of thoracic and abdominal malformations]. / Fetale Magnetresonanztomographie thorakaler und abdomineller Malformationen.

CLINICAL/METHODICAL ISSUE: Diagnosis and differential diagnosis of fetal thoracic and abdominal malformations. STANDARD RADIOLOGICAL METHODS: Ultrasound and magnetic resonance imaging (MRI). METHODICAL INNOVATIONS: In cases of suspected pathologies based on fetal ultrasound MRI can be used for more detailed examinations and can be of assistance in the differential diagnostic process. PERFORMANCE: Improved imaging of anatomical structures and of the composition of different tissues by the use of different MRI sequences. ACHIEVEMENTS: Fetal MRI has become a part of clinical routine in thoracic and abdominal malformations and is the basis for scientific research in this field. PRACTICAL RECOMMENDATIONS: In cases of thoracic or abdominal malformations fetal MRI provides important information additional to ultrasound to improve diagnostic accuracy, prognostic evaluation and surgical planning.

[Intra-abdominal hypertension as a consequence of plasty in the abdominal wall defects, the methods of its determination and prophylaxis].

The results of various methods of investigation of intraabdominal hypertension (IAH) in 186 patients were analyzed. In 134 (27.1%) patients IAH was measured via bladder, in 46 (8.9%)--the investigation using nasogastric probe was added and in 8 (1.6%), in whom the abdominal wall defect coincided with adhesive disease, the measurement was performed immediately via drainages. In 44 (8.9%) patients the IAH measurement while abdominal wall defect closure was performed, using Stryker Intra Compartmental Pressure Monitor apparatus. The data obtained using this apparatus were considered the most informative. For the IAH prophylaxis it is mandatory to prognosticate the intraabdominal pressure raising, so on the stage of the hernia gates edges approximation it must not be bigger than a second degree level.

First-trimester diagnosis of body stalk anomaly using 2- and 3-dimensional sonography.

To determine the sonographic features of body stalk anomaly in the first trimester using 2-dimensional (2D) and 3-dimensional (3D) sonography, we conducted a retrospective analysis of all nuchal translucency sonographic examinations performed between January 1, 2006, and January 1, 2010, at our institution. From a total of 6952 nuchal translucency sonographic examinations, 4 cases of body stalk anomaly were identified. All cases were characterized by an absent umbilical cord and a large ventral wall defect with herniation of the abdominal contents into the extraembryonic coelom. Associated features included kyphoscoliosis, limb defects, and enlarged nuchal translucency measurements. Three-dimensional sonography was a useful adjunct to 2D techniques in determining the precise relationship of fetal structures to the amniotic cavity. Our case series emphasizes the importance of a thorough anatomic survey at the time of nuchal translucency screening and the value of 3D sonography in the delineation of first-trimester anomalies.

Foetus-in-Foetu.

Foetus-in-foetu is a rare malformation in which a monozygotic diamniotic parasitic twin is incorporated into the body of its fellow twin and grows inside it. Less than 100 cases have been reported in literature. One day old newborn male was admitted with antenatal diagnosis of abdominal mass. It was cystic/solid in nature with pressure effects on left ureter and urinary bladder, most likely to be a germ cell tumour. Birth history was uneventful. Exploratory laparotomy was performed and the mass was excised. Physical examination confirmed the diagnosis of foetus-in-foetu. Although it is a rare condition, imaging may play an important role in the correct prospective diagnosis of foetus-in-foetu. Surgical excision is the recommended treatment.

Multiple abdominal vascular anomalies in a patient with Alagille syndrome.

Alagille syndrome affects multiple organ systems. The most common vascular manifestation of Alagille syndrome is peripheral pulmonary artery stenosis. A few cases of abdominal vasculature involvement have been reported, particularly in the pediatric age group. Herein, the authors describe an adult patient with Alagille syndrome who presented with multiple visceral vascular abnormalities, including a high-grade stenosis of the celiac artery, superior mesenteric artery (SMA), aneurysms of the distal common hepatic artery, and distal SMA detected with computed tomographic angiography.