Gastrointestinal Malformations Research Articles

Opioid analgesic exposure during the first trimester of pregnancy and the risk of major congenital malformations in infants: a systematic review and meta-analysis†.

Prescribed opioid analgesics are frequently used to manage pain in pregnancy. However, the available literature regarding the teratogenic potential of opioid use during pregnancy has not been systematically summarised. This systematic review and meta-analysis aimed to assess the quality of the evidence on these potential risks and calculate a pooled estimate of risk for any opioid analgesic and individual opioids. We searched PubMed, Embase and CINAHL for published studies assessing the risk of major congenital malformations in infants following first-trimester exposure to opioid analgesics compared with a reference group, excluding studies examining opioid agonist therapy or illicit opioid use. We assessed the risk of bias using the Risk of Bias in Non-Randomised Studies of Intervention tool. We pooled adjusted risk estimates from studies rated at serious risk of bias or better in a random-effects meta-analysis. Of 12 identified studies, 11 were at high risk of bias (eight serious; three critical). Relative to unexposed infants, those exposed to any opioid use during the first trimester of pregnancy were not at an increased risk of major congenital malformations overall (relative risk 1.04, 95%CI 0.98-1.11); cardiovascular malformations (relative risk 1.07, 95%CI 0.96-1.20); or central nervous system malformations (relative risk 1.06, 95%CI 0.92-1.21). Raised risk estimates were observed for gastrointestinal malformations (relative risk 1.40, 95%CI 0.38-5.16) and cleft palate (relative risk 1.57, 95%CI 0.48-5.13) following any opioid exposure and atrial septal defects (relative risk 1.20, 95%CI 1.05-1.36) following codeine exposure. Although the meta-analysis did not indicate substantial increased risk for most malformations examined, this risk remains uncertain due to the methodological limitations of the included studies. Healthcare professionals and pharmaceutical regulators should be aware of the issues related to the quality of research in this field.

A retrospective study of congenital anomalies and associated risk factors among children admitted at a tertiary hospital in northwestern Tanzania.

Congenital anomalies in Sub-Sahara Africa (SSA) are understudied despite the significant pediatric health burden. This retrospective longitudinal hospital-based study evaluated the records of 326 inpatient children under the age of two years with congenital anomalies at Bugando Medical Centre, a tertiary referral hospital in northwestern Tanzania. Classical logistic regression was used in the analysis of congenital malformation of muscles, gastrointestinal malformation, oral facial clefts, neural tube defects, and skeletal malformations. A modified poisson regression was used to model risk factors for Central Nervous System (CNS) hydrocephalus and congenital heart disease (CHD). A majority (78.8%) of children included in the study were less than six months of age. Nearly half (48.8%) were diagnosed with CHD followed by CNS hydrocephalus (10.4%) and congenital malformation of muscles (8.9%). Babies whose mothers missed periconceptual folic acid supplementation had 83% higher risk of hydrocephalus (aPR = 1.83, 95% CI = 1.11-1.96) and 78% higher for CHD (aPR = 1.78, 95% CI = 1.31-1.94). Male children had 1.67 higher odds of muscular congenital malformations (aOR = 1.67, 95% CI = 1.23-1.89). Less than 37 gestational age had a 1.86 higher odds of muscular congenital malformations (aOR = 1.86, 95% CI = 1.53-3.66). Our study highlights the critical need for folic acid supplementation and establishes a need for a registry and the potential for mapping.

A case of young male with recurrent acute pancreatitis caused by an intrapancreatic gastric duplication cyst.

Gastric duplication cyst (GDC) is a rare gastrointestinal malformation that frequently occurs in the greater curvature of the gastric antrum or corpus. Herein, we reported a case of intrapancreatic GDC found as a result of recurring pancreatitis. A 15-year-old man experienced repeated episodes of acute pancreatitis and was found to have a cystic lesion in the pancreatic tail. Contrast-enhanced computed tomography revealed a 20-mm cystic lesion with an enhanced thick wall. Endoscopic ultrasonography revealed an anechoic cyst with a three-layered wall. Magnetic resonance cholangiopancreatography and endoscopic retrograde pancreatography (ERP) revealed a connection between the cyst and the main pancreatic duct (MPD), and the duplication of the MPD. ERP showed the pancreatic duct stenosis downstream of the cyst. Although preoperative diagnosis was difficult, distal pancreatectomy was performed to prevent recurrence of pancreatitis. Pathological examination revealed that the cystic lesion was circumferentially surrounded by the pancreatic parenchyma. The epithelial lining of the cyst was crypt epithelium containing the fundic or pyloric glands and surrounded by a smooth muscle layer. The final diagnosis was intrapancreatic GDC.

Percutaneous nephrolithotomy in case of crossed fused renal ectopia: a case report

Crossed renal ectopia is a rare congenital anomaly that is frequently associated with gastrointestinal, cardiovascular, genital, and bone malformations. To best of our knowledge, there aren't many cases of inferior crossed fused renal ectopia of the right kidney documented in the literature at this time. A case of crossed-fused renal ectopia is presented here. A 32-year-old man arrived at our hospital complaining of pain in the right flank. Diagnosis: We suspected a single right kidney based on abdominal ultrasonography. An inferior left to right crossed-fused ectopic kidney with calculus in the right (normal) renal pelvis was the patient's diagnosis when combined with 3D computed tomography. Interventions: percutaneous nephrolithotomy with DJ stenting. Because the management of renal stone in this uncommon malformation is complicated, a preoperative assessment with contrast computed tomography is required before moving forward with surgery in order to explore a single kidney with calculi and avoid missing the diagnosis of crossed fused renal ectopia.

BILATERAL CONGENITAL MYDRIASIS WITH ACCOMPANYING MIXED DEVELOPMENTAL DELAY

Introduction: Congenital bilateral mydriasis is a rare condition characterized by fixed dilated pupils unresponsive to light. It may occur associated with cardiovascular syndrome, gastrointestinal malformation, or developmental delay. Referred causes in comprehensive differential diagnosis for congenital mydriasis include alterations in ocular structures, ocular innervation alterations and others secondary to trauma, infection, or topical medication.
 Case Illustration: A 10 months-old boy presented to the hospital with large pupils in both eyes since birth. He has a persistent pupillary membrane with 9 mm diameter of pupil and unable to respond to light in both eyes. The pupillary response test using pilocarpine solution 0.1% showed no difference in diameter of pupils. Comprehensive pediatric examination revealed developmental delay. Computed tomography and laboratory result (laboratory blood count, toxoplasmosis, and cytomegalovirus) within normal limit. After eight months follow up, no significant change in pupil size or light responsiveness was found.
 Discussion: Comprehensive examination of anterior to posterior segments of the eyes is imperative on the lookout for underdiagnosed diseases. Clinical assessment remains vital for guiding investigations. Observation is required to detect the presence of certain systemic abnormalities that frequently do not manifest at birth.
 Conclusion: Congenital mydriasis can be diagnosed quickly in ordinary conditions, but it can be quite challenging when it presents as a coincidental finding of other spectrum diseases. It is important to perform a comprehensive general and eye examination to rule out any serious condition related to it.
 Keyword: Bilateral congenital mydriasis, dilated pupil, mixed developmental delay, pediatric ophthalmology

First Trimester Use of Buprenorphine or Methadone and the Risk of Congenital Malformations

Use of buprenorphine or methadone to treat opioid use disorder is recommended in pregnancy; however, their teratogenic potential is largely unknown. To compare the risk of congenital malformations following in utero exposure to buprenorphine vs methadone. This population-based cohort study used health care utilization data from publicly insured Medicaid beneficiaries in the US from 2000 to 2018. A total of 13 360 pregnancies with enrollment from 90 days prior to pregnancy start through 1 month after delivery and first trimester use of buprenorphine or methadone were included and linked to infants. Data were analyzed from July to December 2022. A pharmacy dispensing of buprenorphine or a code for administration of methadone in the first trimester. Primary outcomes included major malformations overall and malformations previously associated with opioids (any cardiac malformations, ventricular septal defect, secundum atrial septal defect/nonprematurity-related patent foramen ovale, neural tube defects, clubfoot, and oral clefts). Secondary outcomes included other organ system-specific malformations. Risk differences and risk ratios (RRs) were estimated comparing buprenorphine with methadone, adjusting for confounders with propensity score overlap weights. The cohort included 9514 pregnancies with first-trimester buprenorphine exposure (mean [SD] maternal age, 28.4 [4.6] years) and 3846 with methadone exposure (mean [SD] maternal age, 28.8 [4.7] years). The risk of malformations overall was 50.9 (95% CI, 46.5-55.3) per 1000 pregnancies for buprenorphine and 60.6 (95% CI, 53.0-68.1) per 1000 pregnancies for methadone. After confounding adjustment, buprenorphine was associated with a lower risk of malformations compared with methadone (RR, 0.82; 95% CI, 0.69-0.97). Risk was lower with buprenorphine for cardiac malformations (RR, 0.63; 95% CI, 0.47-0.85), including both ventricular septal defect (RR, 0.62; 95% CI, 0.39-0.98) and secundum atrial septal defect/nonprematurity-related patent foramen ovale (RR, 0.54; 95% CI, 0.30-0.97), oral clefts (RR, 0.65; 95% CI, 0.35-1.19), and clubfoot (RR, 0.55; 95% CI, 0.32-0.94). Results for neural tube defects were uncertain given low event counts. In secondary analyses, buprenorphine was associated with a decreased risk of central nervous system, urinary, and limb malformations but a greater risk of gastrointestinal malformations compared with methadone. These findings were consistent in sensitivity and bias analyses. In this cohort study, the risk of most malformations previously associated with opioid exposure was lower in buprenorphine-exposed infants compared with methadone-exposed infants, independent of measured confounders. Malformation risk is one factor that informs the individualized patient decision regarding medications for opioid use disorder in pregnancy.

Enteric Duplication Cyst –A Rare Congenital Anomaly in Adolescence Presenting as Recurrent Pain Abdomen

Case Report: Presented by Dr. Harleen Bawa and Dr. Parthasarathi Hota, this case involves an 18-year-old male with recurrent abdominal pain, nausea, and fever. Initially suspected of congenital gastrointestinal malformation at 1 year, the patient underwent conservative management until symptoms reappeared. Imaging revealed blind-ended tubular structures attached to the small bowel, prompting further investigation for a definitive diagnosis. Management: An exploratory laparotomy revealed an enteric duplication cyst in the ileal region, 50 cm from the ileocecal junction. Resection of the involved ileum with end-to-end anastomosis was performed. Mesenteric lymphadenopathy and an inflamed appendix were also addressed. Biopsy results indicated changes consistent with chronic appendicitis. In Radiological findings CT images displayed well-defined blind-ended tubular structures arising from the distal ileum, suggesting Meckel’s diverticulum. Fluid density cystic lesions near the duodenum and pancreas raised the possibility of an enteric duplication cyst. Discussion: Enteric duplication cysts, though rare, can manifest with varied symptoms, including recurrent abdominal pain and intestinal obstruction. Imaging techniques such as CT, USG, and technetium pertechnetate scanning aid in diagnosis. Resection of the cyst and adjacent intestine with end-to-end anastomosis is a common surgical approach. The prognosis is excellent when complete excision is possible without compromising intestinal length. Conclusion: Enteric duplication cysts, located along the gastrointestinal tract, may present challenges in diagnosis and management. Surgical intervention, guided by imaging and clinical findings, often leads to favorable outcomes. Awareness of potential complications, such as bowel obstruction and bleeding, is crucial for timely intervention.

The outcomes of specific surgically correctable congenital gastro-intestinal malformations at a tertiary level neonatal intensive care unit in South Africa

Background: The outcome of neonates with congenital surgically correctable gastro-intestinal (GIT) malformations is poorly described in low middle income countries. Methods: A 5-year retrospective descriptive analysis of neonates admitted to a tertiary level neonatal intensive care unit (NICU), with congenital, surgically correctable GIT malformations, was performed. The primary outcome was the 30-day postoperative mortality as well as survival to 1 year. Secondary outcomes included patient demographics, clinical presentation as well as the burden of disease in our study population. Results: Eighty-four neonates met study criteria. The mean gestational age was 35 weeks (SD 3.19) and birthweight 2518g (SD 789.3). The most common congenital malformations were intestinal atresia (39%) followed by omphaloceles (21%). Associated systemic malformations were common (39%). The majority of neonates (88%) underwent surgery at a median age of 2 days (IQR 1.5-5). Ventilation was required in 19% of neonates preoperatively and 65% postoperatively. Full feeds were achieved at a median age of 13 days (IQR 9-18) after surgery. The 30 day-postoperative survival rate was 97% with a survival to discharge or transfer of 86%. Data for one year survival was available for 80% of neonates with a one-year survival rate of 75%. Conclusion: The 30-day postoperative survival was high in neonates with congenital, surgically correctable GIT malformations. Outcomes and burden on the healthcare system was dependent on the type of lesion. With early diagnosis and referral to a tertiary centre, good outcomes can be achieved. Neonates with congenital GIT malformations should have long term follow up to monitor growth and neurodevelopment as well as to address the high mortality post discharge.

Concurrence of Hirschsprung disease and annular pancreas: a rare case report

Hirschsprung’s disease (HD) and annular pancreas are both uncommon congenital gastrointestinal malformations. Although additional abnormalities are associated with 18% of HD cases, there is limited evidence of concomitant HD and annular pancreas. We present a full-term, two-day-old male with abdominal distended, and failure to pass first stool after 3 days of observation and rectal washout the abdominal X-rays showed a double bubble appearance. Intraoperative finding, the patient had pancreas annulare (PA). Colonic aganglionosis was discovered using full-thickness rectal biopsies (FTRB). At the age of seven months, a transanal endorectal pull-through was performed without complications. Although the diagnosis of these conditions might be difficult due to their clinical similarities, appropriate diagnosis, and surgical management are possible and can dramatically minimize morbidity and death in the newborn.

A comparative evaluation between Santulli-Type Ileostomy and Alternative Ileostomy Techniques in neonates

Introduction: Neonates often encounter various gastrointestinal malformations, necessitating surgical interventions. However, specific guidelines for choosing the ileostomy technique in these cases are lacking. The Santulli-type ileostomy has gained recognition for its benefits, including reduced complications and early restoration of intestinal transit. The aim of this study is to compare the outcomes of the Santulli-type ileostomy with alternative ileostomy techniques. Materials and methods: This is a case-control study. Statistical tests, such as chi-square (chi2), relative risk, and confidence interval, were used for comparisons, with p-values indicating the significance of observed differences. Complications during ileostomy permanence, restoration surgery, and nutritional status were analyzed. Results: A total of 58 patients who had undergone ileostomies were included in the analysis (Santulli group: 27, Control group: 31). Santulli ileostomies demonstrated significantly lower prolapse rates (p = 0.03) and fewer readmissions due to dehydration (p = 0.008). Santulli ileostomies showed a trend towards more frequent restoration surgeries (p = 0.2) with shorter ileostomy duration. Caliber differences, stoma closure materials, and complications after restoration surgery favored Santulli ileostomies. Conclusion: Our findings emphasize the benefits of Santulli-type ileostomies in neonates, highlighting their efficacy in reducing complications, enabling early restoration surgery, and facilitating a quicker recovery.

Identification of Choroidal Vascular Abnormalities in Patients with Hereditary Hemorrhagic Telangiectasia (HHT)

Purpose: To evaluate the frequency of ocular imaging in patients with HHT at a large academic medical institution; characterize the choroidal vascular changes on indocyanine green angiography (ICGA) and optical coherence tomography (OCT); and explore the relationship of these ocular features with HHT genotypes and systemic manifestations. Methods: Retrospective review of patients with HHT who were seen in the Mayo Clinic ophthalmology department between 2011 to 2021. Results: Of the 71 patients with HHT identified, 7 (10%) individuals had qualifying imaging defined as radial or raster spectral-domain OCT scans and/or ICGA. 4 (57%) patients had enlarged choroidal vessels detected on OCT and/or ICGA. 3 (43%) patients had retinal telangiectasias. Patients with choroidal changes (mean age 67 years) had the endoglin gene mutation, gastrointestinal arteriovenous malformations (AVM), and facial and mucocutaneous telangiectasias. In contrast, patients without choroidal changes (mean age 45 years) included the activin A receptor type-like kinase 1 gene mutation. Conclusion: Only a small proportion of patients with HHT undergo ophthalmic imaging at our institution. The presence of choroidal abnormalities is associated with the endoglin gene mutation, older age, corroborating retinal vascular changes, and increased systemic manifestations.

Influence and Role of the Body Mass Index and Its Development on the Course of Pediatric HSCT - a Retrospective Analysis

T Stetter and M Döring contributed equally to this work. Background Children and adolescents are at increased risk of malnutrition during the course of hematopoietic stem cell transplantation (HSCT). Increased endocrine activity, a catabolic metabolic state and inflammatory processes increase the energy metabolism and therapy-associated side effects impair nutritional intake. Studies have shown that the nutritional status before HSCT in adults has a decisive influence on the time afterwards. The role of nutritional status in children and adolescents before, during, and after HSCT remains poorly understood. Patients and Methods In this monocentric, retrospective study, data from patients who underwent autologous or allogeneic HSCT at the University Children's Hospital Tübingen between November 2012 and November 2021, were analyzed. Exclusion criteria were known gastrointestinal malformations, permanent enteral/parenteral nutrition, and known chronic inflammatory bowel disease before HSCT. On three observation days (baseline: day of in-patient admission, day +30, and day +200 after HSCT) data on age-adjusted BMI percentiles, incidence of transplant-associated adverse events (TRAE; e.g., graft-versus-host disease (GvHD), veno-occlusive disease (VOD), mucositis, infection), and survival (up to 10 years after HSCT; last note in file). were collected. Patients were categorized into alloHSCT and autoHSCT Patients as well as BMI-percentile groups depending on baseline BMI percentile: <3rd percentile, 3rd-<10th percentile, 10th-<25th percentile, 25th-<50th percentile, 50th-<75th percentile, 75th-97th percentile, and >97th percentile. Results A total of 365 patients with a median age of 9 years (0-23 years), 56% of whom were female, were studied. Of these, 307 (84%) underwent allogeneic and 58 (16%) autologous HSCT. The underlying disease was malignant in 262 cases (72%) and non-malignant in 103 cases (28%). The proportions of patients fell into the corresponding BMI percentile groups at the respective observation days (see Table). During the course of time until day +200 after HSCT, the BMI percentile category remained the same in 28% of the cases, in 17%, 10%, and 3% of cases, respectively, patients moved up one, two, and >2 categories, respectively; in 20%, 12%, and 9% of cases, patients dropped one, two, and >2 categories, respectively, compared to baseline. However, comparing the alloHSCT and autoHSCT patients, a different distribution in the development until day +200 is noticeable. Overall, 27% of the alloHSCT and 44% of autoHSCT patients ascend one or more percentile groups, whereas 46% of the alloHSCT and 22% of the autoHSCT patients descend one or more BMI percentile categories. Higher-grade intestinal GvHD stage 3-4 occurred statistically more frequently in the percentile group >75th-97th ( p=0.0028), but when all grades were considered together, no significant clustering in any group was observed. A significant accumulation ( p=0.0413) of VOD, BKV cystitis ( p=0.0021), and Viremia ( p=0.0081) was found in the group >75th-97th percentile. A total of 115 patients (32%) died during the observation period. In the alloHSCT group, 35 deaths (12% of alloHSCT patients) fell under TRM. Here, of the 35 deceased, 12 (34% of deceased, 4% of total alloHSCT patients) were ascribed to the >75th-97th percentile group; the median proportion of deceased patients was 9% (1% of alloHSCT group) in the other percentile groups. In the autoHSCT group, a total of 23 patients (41%) died; of these, all were due to progression or recurrence of the underlying disease. The <3rd percentile and >25th-50th percentile groups each recorded the most deaths, with 26% of those who died and 11% of patients in the autoHSCT group, respectively. Conclusions Contrary to the expectation that particularly underweight pediatric HSCT patients might be more affected by complications, this data collection showed statistically clustered complications and TRM in the group of patients between the 75th-97th BMI percentiles. Although this is only a status description of body weight and length during follow-up, and not a specific survey of nutritional status, the data analysis suggests that HSCT patients in the overweight range should be focused on more closely. A prospective survey of differential nutritional status in pediatric HSCT patients is currently being conducted at our study center.

Does COVID-19 Infection during Pregnancy Increase the Appearance of Congenital Gastrointestinal Malformations in Neonates?

COVID-19 was an infection that was capable of bringing the entire world to a standstill position within a period of days to months. Despite the advancements in the medical sector, the contagion was difficult to control and costed the lives of millions of people worldwide. Many short- and long-term effects are witnessed even to date in people that contracted the disease. Pregnant females had to suffer not only the devastating effects of the virus, but also the psycho-social impact of the lockdown. The impact of COVID-19 infection during pregnancy causing decreased antenatal care or hypoxemic episodes due to severe respiratory distress and whether it could lead to the appearance of congenital gastrointestinal malformation in neonates is still unclear. The aim of our study was to analyze if COVID-19 infection during pregnancy could increase the incidence of gastric malformations in neonates born from these women. We sifted the files of all neonates admitted into our hospital between January 2022 and December 2022, and based on inclusion and exclusion criteria, we included the cases having gastrointestinal congenital malformations during the COVID-19 pandemic. We performed a single-center, retrospective, observational descriptive study. We further divided the patients based on the anatomical location of the malformation. We also took down details of the evolution of pregnancy and whether the mother had contracted a SARS-CoV-2 infection during the pregnancy. Details regarding the Apgar score, days of intensive care admission, sex, and nutrition were the key findings studied. A total of 47 neonates were found to have digestive anomalies, among which, based on the anatomical locations, the number of malformation cases found at the level of the esophagus were 15, while 16 occurred at the level of the pylorus; we found 12 cases of malformation of the duodenum, and four cases had malformation of the rectum. Out of these 47 neonates, 38.3% were females and 61.7% were males. A total of 58% were preemies, among which 9% had intra-uterine growth retardation (IUGR), and 42% were full-term newborns, among which 4% had intra-uterine growth retardation (IUGR). A total of 45% of the births were primiparous pregnancies and 55% were from multiparous females. A total of 14 mothers were found to have tested positive for COVID-19 during the course of pregnancy (p-value = 0.23); many had mild symptoms but were not tested. COVID-19 can affect the wellbeing of the pregnant female and their fetus. Larger studies can help gain extensive knowledge as to whether COVID-19 also has the potential to result in congenital gastrointestinal anomalies in children born from COVID-19 positive mothers. In our study, only a few infants born with this pathology were found to be born from COVID-19 positive mothers. Hence, it is difficult to conclude or exclude a direct correlation between the infection and the congenital malformations.

Predictive models for sepsis in children with Staphylococcus aureus bloodstream infections: a retrospective cohort study

BackgroundThe presence of Staphylococcus aureus in the bloodstream can lead to the development of sepsis; however, the severity and risk factors of the systemic inflammatory response to Staphylococcus aureus bloodstream infections were unclear. This study is aimed to build a model to predict the risk of sepsis in children with Staphylococcus aureus bloodstream infections.MethodsA retrospective analysis of hospitalized pediatric patients diagnosed with Staphylococcus aureus bloodstream infections was performed between January 2013 and December 2019. Each patient was assessed using the pediatric version of the Sequential Organ Failure Assessment score (pSOFA) within 24 h of blood culture collection. A nomogram based on logistic regression models was constructed to predict the risk factors for sepsis in children with Staphylococcus aureus bloodstream infections. It was validated using the area under the receiver-operating characteristic curve (AUC).ResultsOf the 94 patients included in the study, 35 cases (37.2%) developed sepsis. The pSOFA scores ranged from 0 to 8, with 35 patients having a pSOFA score of ≥ 2. Six children (6.4%) died within 30 days, who were all from the sepsis group and had different pSOFA scores. The most common organs involved in sepsis in children with staphylococcal bloodstream infections were the neurologic system (68.6%), respiratory system (48.6%), and coagulation system (45.7%). Hospital-acquired infections (adjusted odds ratio [aOR], 3.0; 95% confidence interval [CI], 1.3–7.2), implanted catheters (aOR, 10.4; 95% CI, 3.8–28.4), procalcitonin level ≥ 1.7 ng/mL (aOR, 15.4; 95% CI, 2.7–87.1), and underlying diseases, especially gastrointestinal malformations (aOR, 14.0; 95% CI, 2.9–66.7) were associated with Staphylococcus aureus sepsis. However, methicillin-resistant Staphylococcus aureus infection was not a risk factor for sepsis. The nomogram had high predictive accuracy for the estimation of sepsis risk, with an AUC of 0.85.ConclusionsWe developed a predictive model for sepsis in children with Staphylococcus aureus infection.

Complex gastrointestinal vascular malformation treated with interpositional mesocaval shunt (Drapanas procedure).

Complex gastrointestinal vascular malformation treated with interpositional mesocaval shunt (Drapanas procedure).

Research progress on risk factors of postoperative functional defecation disorder in anorectal malformation

AbstractAnorectal malformation (ARM) is a common congenital gastrointestinal malformation in neonates, which has complex pathological changes and unclear etiology. The only treatment modality currently available for ARMs is through surgical treatment to correct the deformity of the anal morphology and restore normal defecation function. In addition, the mortality rates of children with ARMs have significantly decreased as a result of recent advances in medical technology. It is important to note that despite the treatment of perianal anatomical deformities, approximately one‐third of ARM patients continue to experience functional defecation disorder (FDD), which includes fecal incontinence and constipation and negatively impacts the patient's quality of life. There are still numerous questions regarding the exact cause of FDD in ARM patients following surgery. It is generally accepted that the occurrence of FDD may be related to clinical staging, structural changes in the perianal rectal muscles, anomalies of the enteric nervous system, surgical injury, etc. However, some of these factors are still controversial and require additional study to be confirmed. In this paper, we review the risk factors for FDD following surgery in ARMs, which will help us to subsequently develop appropriate interventions to enhance the anal function and improve quality of life of children with ARMs.

A systematic review of in utero cannabis exposure and risk for structural birth defects.

Cannabis use among pregnant women has increased over time. Therefore, there is a great public health need to understand the consequences of in utero cannabis exposure. While several meta-analyses and reviews have summarized the evidence of in utero cannabis exposure on adverse obstetric outcomes (e.g., low birth weight and preterm birth) and long-term offspring development, there has not been a focus on in utero cannabis exposure and risk for structural birth defects. We conducted a systematic review using PRISMA guidelines to evaluate the association between in utero cannabis exposure and structural birth defects. We identified 20 articles to include in our review and focused on interpreting findings from the 12 that adjusted for potential confounders. We report findings by seven organ systems. Within the 12 articles, four reported on cardiac malformations, three reported on central nervous system malformations, one reported on eye malformations, three reported on gastrointestinal malformations, one reported on genitourinary malformations, one reported on musculoskeletal malformations, and two reported on orofacial malformations. Findings on associations between in utero cannabis exposure and birth defects reported in more than two articles were mixed (i.e., findings for cardiac, gastrointestinal, central nervous system malformations). Findings for associations between in utero cannabis exposure and birth defects reported in two articles (i.e., orofacial malformations) or in a single article (eye, genitourinary, and musculoskeletal) suggested that cannabis exposure was not associated with these types of malformations, but strong conclusions cannot be drawn from such sparce research. We review the limitations and gaps in the existing literature and call for more research to rigorously evaluate associations between in utero cannabis exposure and structural birth defects. identifier CRD42022308130.

Complex Gastrointestinal Vascular Malformation Treated with Interpositional Mesocaval Shunt (Drapanas Procedure)

This video shows an open superior mesenteric vein to inferior cava vein shunt (Drapanas procedure) to treat severe recalcitrant gastrointestinal bleeding. This patient had previously has extensive inferior vena cava and superior mesenteric vein thrombosis that progressed to a massive complex portal vascular malformation. The patient survived for 10 months free of symptoms and with no recurrent gastrointestinal bleeding after the procedure, before dying from unrelated causes. eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiI4NTFiMjU5M2JkMmMzMzBmZWU3ZWE2ZWE0NDZiOWU3OSIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjg0ODU2MDA1fQ.CmDw7kksZLkcK06tdS_ZdZfB0hwVA2lcH2YUVHNa_RAx8nuCi3qlJOETRtq25NNDP23Sle_PT2mCGATsWHY570IwqchjhgvKlsrxd2kp8R03ljnSc5iOiTdfWGa7G2nB_7f1t8nojjbbBi7Wk5pKLnXBcvfLvPO7fU4mo_qbEy0aooZgtT2pD4I4JckacwDmJ67K9ktLtvJyMnOiVwNwdN07PeFl-6VBtYy4CHv8oASWbqmMV53LxQDy0Gcz2vewjFu9NZVtwM9b0EbcZzHRbRhWgr5i5n9tXiQsq3StQYyW1xtv0ka2ZjAJ1_fLHrx_XRP4QzcykrSrsXnOyFSdHQ Download .mp4 (79.86 MB) Help with .mp4 files SUBMISSION 1.11.2023.mp4

Advances in surgical treatment of gastrointestinal malformations

Advances in surgical treatment of gastrointestinal malformations

Evaluation of the regional distribution of the pediatric surgery workforce and surgical load in Brazil

ObjectiveThe purpose of this study is to examine the regional distribution of the pediatric surgery workforce and the expected local demand for pediatric surgical procedures in Brazil.MethodsWe collected data on...