A new technique in the treatment of intestinal malrotation. / Una nueva técnica en el tratamiento de la malrotación intestinal.

INTRODUCTION: Intestinal malrotation is a congenital pathology with potentially catastrophic complications, such as volvulus, whose treatment has barely not changed in nearly 100 years (Ladd's procedure). Dr. Abu-Elmagd recently described a new technique that was applied in one of our patients. CLINICAL CASE: 12-year-old boy who had undergone Ladd's procedure as a result of intestinal volvulus secondary to malrotation when he was 2 days old. He had subocclusion and eventually obstruction, with intestinal volvulus compatible imaging. Intraoperative findings: duodenal subocclusion, volvulus and lymphangiectasias. Kareem's procedure: bowel positioning in normal rotation, duodenopexy (duodenal C posterior to the mesenteric vessels), formation of neo-Treitz, and fixation of the cecum, the ascending colon, and the mesenteric root. The patient was discharged on postoperative day 6 and remains asymptomatic after 1 year of follow-up. DISCUSSION: Kareem's procedure is a safe and effective malrotation repair technique. It can replace Ladd's procedure as it reduces the risk of re-volvulation and improves digestive symptoms.

INTRODUCCION: La malrotación intestinal es una patología congénita con complicaciones potencialmente catastróficas, destacando el vólvulo, cuyo tratamiento no ha variado significativamente en casi 100 años (procedimiento de Ladd). Recientemente el Dr. Abu-Elmagd describió una técnica que hemos aplicado en un paciente. CASO CLINICO: Varón de 12 años, intervenido con 2 días de vida por vólvulo intestinal secundario a malrotación realizándose procedimiento de Ladd. Presenta cuadros suboclusivos y finalmente obstructivo con imágenes compatibles con vólvulo intestinal. Hallazgos intraoperatorios: suboclusión duodenal, vólvulo y linfangiectasias. Procedimiento de Kareem: posicionando intestino en normorotación, duodenopexia (C duodenal posterior a vasos mesentéricos), formación de neoTreitz, fijación de ciego, colon ascendente y raíz mesentérica. Alta hospitalaria al 6º día postoperatorio, asintomático en 1 año de seguimiento. COMENTARIOS: El procedimiento de Kareem es una técnica segura y efectiva que corrige la malrotación, pudiendo reemplazar al procedimiento de Ladd al disminuir el riesgo de revolvulación y mejorar síntomas digestivos.

A Prospective Management Strategy for Heterotaxy Syndrome with Intestinal Rotation Abnormalities: Imaging Does Not Predict Need for Surgery.

PURPOSE: Heterotaxy syndrome (HS) affects right-left anatomical development in 3% of children with congenital heart disease. Commonly, these patients have intestinal rotation abnormalities (IRA) that differ from typical malrotation. In this prospective study, we examine the development of a management pathway, imaging findings, and clinical course of patients with HS and IRA. METHODS: After literature review, a multispecialty focus group designed a pathway for HS. Participants from a single institution were prospectively enrolled from 2016 to 2019. They underwent an abdominal ultrasound and UGI, however timing was variable based on symptoms. Symptomatic IRA was defined as feeding intolerance, bilious or non-bilious vomiting, bloating, severe reflux, and intermittent pain or abdominal distention. Screening for symptoms occurred every three months for the first two years and then annually. RESULTS: 18 participants were followed for a median of 5.0 years. Three clinical categories were identified: 1) asymptomatic, not requiring intestinal surgery, 72.2%, 2) symptomatic with feeding intolerance or failure to thrive requiring gastrostomy placement and diagnostic laparoscopy with Ladd procedure, 16.7%, and 3) symptomatic requiring urgent exploration for suspicion of volvulus, 11.1%. Need for surgery did not necessarily correlate with US and/or UGI findings. CONCLUSIONS: In children with HS and IRA, postnatal imaging did not inform the need for intestinal surgery. Although rare, volvulus or other forms of proximal obstruction can be anticipated, and symptomatic patients should be offered surgical intervention. A multidisciplinary care pathway for patients with HS and IRA can coordinate care and help counsel families on the likelihood of needing intestinal surgery for IRA. LEVEL OF EVIDENCE: Level II. TYPE OF STUDY: Prospective Cohort Study.

Pediatric Gastrointestinal Histopathology in Patients With Tetratricopeptide Repeat Domain 7A (TTC7A) Germline Mutations: A Rare Condition Leading to Multiple Intestinal Atresias, Severe Combined Immunodeficiency, and Congenital Enteropathy.

Mutations in the tetratricopeptide repeat domain 7A (TTC7A) gene are a rare cause of congenital enteropathy that can result in significant morbidity. TTC7A deficiency leads to disruption of the intestinal epithelium. The histopathology of this condition has been partly described in case reports and clinical studies. This manuscript describes an in-depth investigation of the pediatric gastrointestinal pathology of the largest histologically examined cohort with confirmed TTC7A mutations reported to date and, for the first time, compared the findings to age-matched and sex-matched control patients with intestinal atresia not thought to be associated with TTC7A mutations. Hematoxylin and eosin-stained slides of endoscopically obtained mucosal biopsies and surgical resection specimens from 7 patients with known TTC7A mutations were examined retrospectively. The microscopic findings were found to be on a spectrum from atresia-predominant to those with predominantly epithelial abnormalities. Several unique histopathologic characteristics were observed when compared with controls. These included neutrophilic colitis and prominent lamina propria eosinophilia throughout the gastrointestinal tract. Striking architectural abnormalities of the epithelium were observed in 4 of the 7 patients. The 5 patients with intestinal atresia demonstrated hypertrophy and disorganization of the colonic muscularis mucosae accompanied by bland spindle cell nodules within the intestinal wall. The components of the latter were further elucidated using immunohistochemistry, and we subsequently hypothesize that they represent obliterated mucosa with remnants of the muscularis mucosae. Finally, atrophic gastritis was noted in 4 patients. In conclusion, the unique histopathologic characteristics of TTC7A mutation-associated enteropathy described herein more fully describe this novel disease entity in infants who present with congenital enteropathy or enterocolitis.

A Network Pharmacological Approach to Explore the Mechanisms of TongXieYaoFang in Inflammatory Bowel Disease

Abstract Inflammatory bowel disease (IBD) is a chronic inflammatory disorder of the intestine, demonstrating an increasing incidence every year. TongXieYaoFang (TXYF) has been used widely in China as a complementary therapy to relieve the symptoms of IBD for hundreds of years. In the present research, a network pharmacology-based approach was used to systematically explore the intrinsic mechanisms of TXYF in IBD at the molecular level. Network pharmacology-based methods, which mainly included database mining, screening of bioactive compounds, target prediction, collection of IBD-related targets, gene enrichment analysis, network construction, and molecular docking, were employed in the present study. Network analysis revealed a total of 108 potential targets derived from 22 component compounds of TXYF, among which 34 targets were common with the IBD-related targets. In the protein-protein interaction (PPI) network, 10 key targets were identified. The gene enrichment analysis suggested that anti-inflammatory processes, such as NF-kappa B signaling pathway and Toll-like receptor signaling pathway, could be the core processes involved in the action of TXYF in IBD. Molecular docking results revealed that three compounds present in TXYF exhibited strong binding affinity for PTGS2. The present study provides novel insights into the molecular mechanisms and network approaches of TXYF action in IBD from a systemic perspective. The potential targets and pathways identified in the present study would assist in further research on the clinical application of TXYF in IBD therapy.

Enhancement of dissolution rate of racecadotril by liquisolid compact technology

Abstract The current investigation was used to improve the rate of dissolution of an anti-diarrheal drug i.e., racecadotril (RT) at low pH conditions (i.e., in the stomach) by reducing the water secretion and electrolyte in to the intestine by liquisolid tablets. Different formulations (liquisolid) were prepared using Avicel PH 102 as a carrier. Aerosil 200 as a coating material and sodium starch glycolate used as a disintegrant. Polyethylene glycol 200 was used as a non-volatile vehicle to dissolve the drug. FTIR, DSC, XRD and dissolution studies were conducted to characterise liquisolid tablets. Characterisation studies indicated that no interactions between carrier and drug. Solid state characterization had shown a reduction in crystallinity that further supports increment in solubility and dissolution. The optimised formulation showed a significant increase in dissolution i.e., 99.54±0.62% in 30 min compared to directly compressible tablets (38.47±0.26%). The % dissolution efficiency of racecadotril liquisolid tablets 76.86% compared to marketed tablet (27.56%) and conventional direct compression tablet (17.11%). Significant reduction in mean dissolution time of racecadotril from liquisolid tablets (6.84 min) compared to direct compression tablet (44.57 min), indicating faster release of drug and faster onset of action. Formulation of liquisolid tablets could enhance solubility, dissolution and bioavailability of racecadotril

Vólvulo de intestino medio: a propósito de dos casos / Middle intestine volvulus: on the purpose of two cases / Volvulus do intestino médio: com propósito de dois casos

La malrotación intestinal es un espectro de malformaciones, que incluye una gran variedad de alteraciones en el proceso de rotación y fijación del intestino. La ausencia completa de rotación intestinal, uno de los tipos de malrotación intestinal más frecuentes, puede presentarse como hallazgo asintomático o manifestarse clínicamente por un vólvulo del intestino medio. Sin embargo, incluso entre aquellos que se presentan con vólvulo del intestino medio, la clínica puede ser muy diferente, según el grado de isquemia y eventual necrosis intestinal. Se presentan dos casos clínicos de malrotación intestinal con vólvulo del intestino medio con presentaciones, imágenes, tratamientos y evoluciones muy disímiles. Se analizan los mismos a la luz de una revisión bibliográfica relevante al tema tratado, se sacan aprendizajes del manejo realizado y la evolución que presentaron, y se enfatizan los elementos de mayor jerarquía para optimizar el manejo de estos pacientes.

Intestinal malrotation is a spectrum of malformations that includes a great variety of alterations in the rotation and fixation process of the intestines. The total absence of intestinal rotation, one of the most frequents types of intestinal malrotation could present as an asymptomatic find or appear clinically as a midgut volvulus. However, even those that appear as midgut volvulus, could show quite different signs and symptoms according to the degree of ischemic insult and possible intestinal necrosis. Two clinical cases of intestinal malrotation with midgut volvulus with quite different presentations, images, treatments, and evolutions, are informed. The two cases are analyzed under a relevant bibliographic revision, knowledge is derived from the carried-out management and evolution, and elements for the future optimization of management are underlined.

A má rotação intestinal é um espectro de malformações, que inclui uma grande variedade de alterações no processo de rotação e fixação do intestino. A ausência completa de rotação intestinal, um dos tipos mais comuns de má rotação intestinal, pode se apresentar como um achado assintomático ou manifestar-se clinicamente como um volvo de intestino médio. Porém, mesmo entre aqueles que apresentam volvo de intestino médio, os sintomas podem ser muito diferentes, dependendo do grau de isquemia e eventual necrose intestinal. Dois casos clínicos de má rotação intestinal com volvo de intestino médio são apresentados com apresentações, imagens, tratamentos e evoluções muito diferentes. São analisados ​​à luz de uma revisão bibliográfica pertinente ao tema em questão, lições aprendidas com o manejo realizado e a evolução que apresentaram, e os elementos de maior hierarquia são enfatizados para otimizar o manejo desses pacientes.

Communicating multiple tubular enteric duplication with toxic megacolon in an infant: A case report.

RATIONALE: Gastrointestinal tract duplication is a rare congenial anomaly which can be found anywhere along the gastrointestinal tract. While many patients are incidentally diagnosed during operation, in some cases it can present with severe gastrointestinal symptoms. In this case report, the patient presented with signs of toxic megacolon leading to rapid aggravation of inflammatory shock. PATIENT CONCERNS: A 49-day old male infant presented with fever, poor feeding, and severe abdominal distension. DIAGNOSIS: Abdominal ultrasonography was done. During the examination, a foley catheter was inserted through the anus to evaluate bowel patency and enable rectal decompression. The tip of the foley catheter was located in a separate narrower tubular lumen adjacent to the distended rectum. These findings suggested possibility of a tubular duplication cyst of the rectum as the culprit for the bowel obstruction. INTERVENTIONS: The patient underwent emergency laparotomy. Findings showed multiple tubular intestinal duplications involving the ileum, appendix, cecum, descending colon, sigmoid colon and rectum. The true lumen of the rectosigmoid colon was completely collapsed while the adjacent tubular cyst remained severely distended and stool passage was not possible. Decompression of the sigmoid colon was done with loop colostomy with both the wall of the true bowel and enteric cyst forming the colostomy orifice. OUTCOMES: After 40 days of postoperative care, the patient was discharged with no immediate complications. Four months after the initial operation, colostomy take-down and transanal rectal common wall division was done. No complications were observed. LESSONS: To our knowledge, this is the first case to be reported where a rare presentation of intestinal duplication resulted in an acute presentation toxic megacolon. Such emergency cases can be effectively treated with emergency surgical bowel decompression and elective common wall division.

[Inguinal hernia in children: easily incarcerated]. / Liesbreuk bij kinderen: snel beklemd.

Inguinal hernia in children is caused by an incomplete obliteration of the vaginal process during the embryological development. The vaginal process can thus become a hernia sac that often contains bowel and in girls, an ovary. The diagnosis of inguinal hernia is made by history and physical examination. According to current guidelines surgical repair should be performed without delay to avoid incarceration, which gives a high risk of complications, including testicular atrophy and ischemia of vital organs. However, patients are regularly not referred adequately. We present three cases of children who developed complications of a non-repaired inguinal hernia. Additionally, the data of all children with a congenital inguinal hernia, surgically treated from January 2018 until August 2019 show that out of 243 children 13.6% presented acutely with an incarcerated inguinal hernia. Another 6% received a wrong advice from their primary care doctor and was not referred to a (pediatric) surgeon.

Outcomes of Laparoscopic Versus Open Ladd Procedures and Risk Factors for Conversion.

Background: Malrotation is a common congenital anomaly that can lead to bowel obstruction and ischemia if not corrected with a Ladd procedure. Controversy exists between open and laparoscopic approaches. We sought to compare postoperative outcomes and determine risk factors for conversion to an open procedure. Methods: The National Surgical Quality Improvement Program (NSQIP)-Pediatric was used to identify patients undergoing Ladd procedures from 2013 to 2018. Propensity score matching was used to account for differences in patient characteristics between open and laparoscopically treated cohorts. Chi-square tests and adjusted logistic regression analysis were used to determine patient outcomes differences between treatment groups and factors associated with conversion. Results: A total of 2437 patients were identified, 1889 (77.5%) open, 548 (22.5%) laparoscopic, and 193 (35.2%) laparoscopic converted to open. Patients undergoing laparoscopic compared with open procedures had shorter length of stay (5 versus 7 days, P < .001) and lower overall complication rates (13.1% versus 18.1%, P = .025), despite longer operative times (108.9 versus 93.7 minutes, P < .001). Patients requiring conversion were more likely to be younger, have an urgent/emergent case, sepsis/septic shock, and nutritional support requirement. Conclusions: After risk adjustment, laparoscopic Ladd procedure is associated with decreased complications and minimal operative time increases compared with an open approach. Risk factors associated with conversion should be considered during operative planning.

Prevalence and Clinicopathologic Features of Intestinal Perforation Caused by Segmental Absence of the Intestinal Musculature in Adults.

Segmental absence of the intestinal musculature (SAIM) can cause intestinal perforation in adults. However, its prevalence and clinicopathologic features have not been well-described. This study aimed to determine the prevalence of SAIM-associated perforation and characterize its clinicopathologic features. We retrospectively examined 109 cases of intestinal perforation that underwent surgical resection from January 2009 to December 2019. SAIM was defined as the complete absence of the muscularis propria without extensive inflammation and fibrinous exudation around the perforation. SAIM was the second most frequent cause of perforation (26 cases: 24%), the most frequent cause being related to diverticulitis (39 cases: 36%). The most common site was the sigmoid colon (12 cases: 46.2%). The younger group (aged below 65 y) exhibited more frequent perforation of the upper segments of the gastrointestinal tract (from the duodenum to the descending colon) than the older group (65 y and above) (P=0.0018). No patients developed recurrence. The most common gross features were well-defined circular or small punched-out lesions, and the histologic features were complete absence of the muscularis propria and absence of hemorrhage and necrosis around the area of perforation. The characteristic features of SAIM were unique and their prevalence was higher than previously reported. The precise recognition of SAIM can aid in understanding the cause of perforation and avoiding further unnecessary examinations.

Laparoscopic Ladd Procedure for Malrotation in Newborns and Infants.

BACKGROUND: Laparoscopic approach for malrotation has become more popular for neonates and in cases with volvulus, but its safety and efficacy remains controversial. This study reviewed laparoscopy outcomes in neonate/infant malrotation. METHODS: Medline/PubMed and Lilacs databases were reviewed. Data from studies published in English/Spanish between 1995 and 2019 were collected. Results are presented as percentages and means/medians; logistic regression was used to study possible associations. RESULTS: Nineteen papers offered 99 neonates/infants with median age and weight of 10.5 days and 3.5 kg, respectively. Ladd's procedure was performed in 95 (96%) patients and bands' division in 4 (4%); appendectomy was not included in 16 (16.2%) patients, and cecopexy was not performed in all cases. Volvulus was reported in 39 (39.4%) patients. There were 11 conversions (11.1%) and 10 recurrences of symptoms (10.1%) that required reintervention. An association was found between volvulus and recurrence (P = .05) and the need for conversion (P < .01). There were 10 (10.1%) minor complications and no mortality. The median follow-up was 10 months. DISCUSSION: Laparoscopic approach to malrotation is feasible and safe in hemodynamically stable neonates/infants without intestinal necrosis and is associated with 11% conversion rate and 10% reinterventions. The presence of volvulus is associated with recurrence and conversion. Laparoscopic Ladd's procedure with appendectomy and without cecopexy is the commonly practiced approach that is associated with minor complications.

Malrotation Correction in the Adult Population.

BACKGROUND: Malrotation is a congenital anomaly most often affecting the pediatric population. The Ladd procedure is the standard treatment for this pathology. Well-studied in the pediatric population, large studies of the demographics and outcomes of patients who reach adulthood are lacking. METHODS: An analysis of the American College of Surgeons National Surgical Quality Improvement Program (ACS NSQIP) database (2015-2018) was performed, capturing patients with a post-operative diagnosis of malrotation and who underwent surgical correction with or without appendectomy, excluding those who underwent other major procedures such as colectomy. Baseline demographics and outcomes were compared. The primary outcome was mortality. Secondary outcomes such as length of stay and discharge destination were included. RESULTS: Two hundred twenty patients undergoing surgical correction of malrotation were captured, all of which were performed by a general surgeon under general anesthesia. One hundered and nine (49.55%) of these patients also underwent an appendectomy. Most of these patients were female (68.18%). Comorbidities and perioperative variables were clinically similar. Operative time was similar between the two groups (112 ± 86 vs. 98 ± 49 min, p = 0.1385). Thirty-day mortality (1.36%), length of stay (4.79 ± 6.21 days), readmission rate (13.64%), wound infection (2.27%) and discharge destination (95.00% to home) were statistically similar between groups. CONCLUSIONS: The data describes demographics and outcomes in adults undergoing Ladd procedures with and without appendectomy. Immediate outcomes may be equivalent regardless of incidental appendectomy. Further work is necessary to describe the population of adults with malrotation reaching adulthood.

Laparoscopic Roux-en-Y gastric bypass in intestinal non-rotation.

There are some incidental findings during bariatric surgery. Some of these findings may make the operation more challenging than routine surgery. Intestinal malrotation/non-rotation are rare congenital anomalies that may remain asymptomatic until bariatric surgery. Here we report a 30-year-old female patient with unknown intestinal congenital anomaly which was diagnosed during surgery and she underwent Roux-en-Y gastric bypass, which can be a challenging procedure in intestinal malrotation/non-rotation and conversion may be needed.

Malrotation of the midgut associated with horseshoe kidney presenting as gastric outlet obstruction in a 15-year-old boy.

Malrotation occurs in approximately 1 in 500 live births. However, the true incidence of malrotation is unknown since many asymptomatic patients fail to present. Approximately 90% of patients with malrotation are diagnosed within the 1st year of life. Eighty per cent of them are diagnosed within the 1st month of life. Nevertheless, there are recent reports of manifestations later in life both as emergency conditions and more chronic gastrointestinal symptoms. The relationship between malrotation and horseshoe kidney has not been fully understood, but few case reports have highlighted their occurrence in the same patient. The mode of presentation of this case and its association with a horseshoe kidney is the reason for this report. This was a case of malrotation associated with horseshoe kidney. He had exploratory laparotomy and Ladd's procedure. Malrotation is associated with horseshoe kidney which presented as gastric outlet obstruction. He responded well to treatment after Ladd's procedure.

A fatal twist in childhood: A post-mortem finding of intestinal volvulus with malrotation.

Gastrointestinal pathology leading to the death in paediatric age group is uncommon. The diseases that encountered were mostly intestinal obstruction, peritonitis and gastrointestinal bleeding. Due to the severe symptoms, most of the patients presented to hospital in time and were treated appropriately. However, with the presence of contributing factors, certain gastrointestinal pathology can progress rapidly leading to the death. We report a rare case of intestinal volvulus in a 3 years old girl where the deceased presented with one day short history of vomiting before her demise. The contributing factors were bronchopneumonia sepsis and underlying intestinal malrotation identified via post-mortem examination.

¿Está asociada la malrotación intestinal sintomática en el paciente adulto con el sobrecrecimiento bacteriano de intestino delgado? / Is there an association between adult symptomatic intestinal malrotation and small intestinal bacterial overgrowth?

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Congenital segmental intestinal anomalies in calves.

This focus article has been prepared by Alwyn Jones, veterinary investigation officer with SRUC Veterinary Services.