Health-related quality of life outcomes in children after congenital heart disease surgery in low-middle-income countries: a systematic review and meta-analysis.

Introduction: Improved treatments for children with congenital heart disease (CHD) have led to a growing interest in long-term functional outcomes such as health-related quality of life (HRQOL). Studies on HRQOL in children with CHD have yielded contradictory results. In this study, we aimed to perform a systematic review and meta-analysis to analyze the effect of surgery on HRQOL outcomes in children with CHD in low-income and middle-income countries. Methods: A comprehensive search for articles was performed using the Medline (PubMed), Scopus, and Embase databases from their inception to September 5, 2023. Studies reporting QOL outcomes in children <18 years and published in English were included. Results: Of the 1239 records screened, 10 studies, including 1721 participants, were included in the study. The overall QOL was significantly better in the control group than in the children who underwent surgery for CHD (P=0.04, standard mean difference of -0.62, 95% CI: -1.2 to -0.04), and the overall QOL was significantly better in the children with CHD after surgery than before surgery (P=0.05, standard mean difference of -0.56, 95% CI: -1.11 to -0.01). Conclusion: The QOL of children from low-income and middle-income countries who undergo surgery for CHD is significantly poorer than that of controls in all dimensions except the emotional domain. Meanwhile, surgery has the greatest impact on improving the physical domain in children with CHD after surgery. Strategies to improve HRQOL in this subgroup of patients should be further investigated.

Acute mesenteric ischemia due to superior mesenteric artery thrombosis in a patient with protein S deficiency: A case report.

INTRODUCTION: Protein S deficiency resulting in mesenteric vein thrombosis has been reported in previous studies however those causing SMA thrombosis has been rarely reported. Multidisciplinary approach involving general surgeon, a vascular surgeon, an interventional radiologist, and an intensivist are crucial for management of SMA thrombosis. CASE PRESENTATION: A 39-year-old non-smoker hypertensive female who was diagnosed with partially occlusive thrombus in the superior mesenteric artery via Contrast-enhanced computed tomography (CECT) re-presented after 5 days and CECT revealed a partially occlusive thrombus in the superior mesenteric artery and Protein S deficiency (free protein S:15 %). She was managed by lysis of thrombus with streptokinase by interventional radiology team. The patient is on anticoagulants and without abdominal complaints on follow-up at 24 months. DISCUSSION: Computed tomography angiography should be done immediately in any patient suspected of AMI since delay in diagnosis accounts for high mortality rates of 30-70 %. The surgical treatment of the condition is well established and consists of revascularization and/or resection of nonviable bowel. Endovascular techniques have emerged as an alternative for occlusion of the SMA. Patients with protein C and/or S deficiency treated for AMI require lifelong anticoagulant/antiplatelet therapy to prevent relapse. CONCLUSION: Hereditary thrombophilia should be suspected in young people with unusual thrombotic presentations. Earlier diagnosis and aggressive antithrombotic therapy in individuals with hypercoagulable states can improve outcomes. Treatment involving a multidisciplinary approach improves outcomes.

De Garengeot hernia: A unique presentation of femoral hernia with perforated appendicitis.

INTRODUCTION: De Garengeot hernia is a rare subset of femoral hernias containing the vermiform appendix. It is more common in females. The presenting symptoms are non-specific hence diagnosis is challenging and there is no consensus on treatment of it. PRESENTATION OF CASE: An 85-year-old male patient who had previously undergone herniorrhaphy presented with a four-day history of pain and swelling in the right groin region. On a contrast-enhanced computed tomography scan it revealed incarceration of the appendix within femoral hernia. It was managed with appendectomy and closure of the defect using non-absorbable suture. DISCUSSION: Diagnosis of De Garengeot hernias remains challenging due to their non-specific presentation and attenuated clinical symptoms, often leading to intraoperative identification. It is crucial for physicians to recognize this rare presentation of appendicitis and be familiar with the available surgical interventions. However, the literature does not establish a consensus regarding the preferred surgical approach. CONCLUSION: De Garengeot hernia remains an uncommon and challenging presentation of femoral hernia, particularly when complicated by appendiceal incarceration leading to acute appendicitis. Surgical management should be tailored to each patient's unique circumstances.

Loco-regional recurrence of adrenocortical carcinoma: A case report.

INTRODUCTION: Adrenocortical carcinoma (ACC) is a rare and aggressive endocrine malignancy with a high recurrence rate. Approximately half of the patients are asymptomatic, while others experience symptoms due to the tumor's size or hormone secretion. Ro resection if possible is the best option for treatment of primary as well as locoregional recurrent ACC. CASE PRESENTATION: A 20-year-old female who previously underwent open left adrenalectomy for Stage III ACC presented with complaints of heaviness and vague discomfort in the left upper abdomen. Current hormonal assays were normal. Imaging revealed a lesion in the spleen suggestive of recurrence. She underwent elective surgery involving en bloc resection of the spleen, diaphragm, and associated structures. Postoperative recovery was uneventful, histopathology confirmed recurrence and subsequent PET-CT showed no recurrence. She is currently on mitotane and remains symptom-free with no signs of recurrence after initial surgery. CLINICAL DISCUSSION: Complete resection (Ro) if possible, for recurrent and metastatic disease has been linked to long-term survival and offers significant palliative benefits, particularly in cases involving symptomatic steroid production. CONCLUSION: ACC has a high frequency of local recurrence therefore management of recurrence should be considered from the initial diagnosis. Ro resection of recurrence is the best potential treatment. Follow-up protocols and improving integration between surgical, oncological, and supportive care departments are crucial for overcoming healthcare challenges in Nepal.

Correlation Between Radiological Grading and Clinical Scoring in Knee Osteoarthritis Patients.

BACKGROUND: Osteoarthritis is characterized by mechanical joint abnormalities, primarily involving articular cartilage and subchondral bone degradation. Diagnosis of knee osteoarthritis relies on American College of Rheumatology criteria, and severity assessment utilizes the Western Ontario and McMaster Universities Osteoarthritis Index score and Kellgren and Lawrence grading. Despite reported associations, discrepancies persist in the correlation between Western Ontario and McMaster Universities Osteoarthritis Index and Kellgren and Lawrence grade. METHODS: Descriptive cross-sectional study was conducted at the National Trauma Centre, over six months with consecutive convenience sampling. Demographic and clinico-radiological data were collected. Mean, SD and correlation coefficient was calculated. RESULTS: Consecutive convenient sampling yielded 80 participants meeting American College of Rheumatology criteria, aged 52-81 years, predominantly female (67.50%). Significant positive correlations were identified between age, Western Ontario and McMaster Universities Osteoarthritis Index score, and KL grade. Results showed most cases with Kellgren and Lawrence grade III and strong correlations (r=0.73, p=0.00) between total Western Ontario and McMaster Universities Osteoarthritis Index scores and Kellgren and Lawrence grading. Notably, pain, stiffness and physical functions individually exhibited a significant possitive correlation with Kellgren and Lawrence grading. The study affirms age-related influences on osteoarthritis, emphasizes female predilection, and underscores the importance of assessing both clinical and radiological parameters. CONCLUSIONS: In conclusion, this study supports Western Ontario and McMaster Universities Osteoarthritis Index efficacy in dynamic disease assessment and management, especially in settings where radiological examinations may be impractical, thus establishing Western Ontario and McMaster Universities Osteoarthritis Index as a versatile tool for systematic monitoring and intervention in knee osteoarthritis.

Concomitant intussusception and appendicitis "appendi-sception" in children: A case report and review of literature.

INTRODUCTION: Pediatric intussusception is the leading cause of bowel obstruction in children under 2 years of age. Concurrent intussusception and appendicitis, known as "appendi-sception" is exceptionally rare in the pediatric population. CASE PRESENTATION: A 37-month-old boy presented with periumbilical abdominal pain, vomiting, and red currant jelly stool for two weeks. Clinical examination and ultrasonography confirmed intussusception. Hydroreduction was attempted twice but failed, necessitating surgical intervention. During exploratory laparotomy, ileocolic intussusception and an inflamed appendix were discovered for which an appendectomy was performed. The postoperative course was uneventful, and histopathology confirmed suppurative appendicitis. The patient had no difficulty at the one-year follow-up. DISCUSSION: Intussusception with appendicitis as a lead point is rare and often challenging to diagnose preoperatively. The literature review revealed 11 pediatric cases, with concomitant intussusception and appendicitis highlighting diagnostic challenges due to symptom overlap. The overlap in symptoms between intussusception and appendicitis complicates diagnosis. Hydroreduction failure should prompt consideration of secondary causes, including appendicitis. CONCLUSION: Considering secondary causes in intussusception is crucial, especially when initial management fails. CT scans should be considered in such cases. Appendectomy and manual reduction can effectively manage concurrent intussusception and appendicitis. This case underscores the importance of considering multiple diagnoses in complex pediatric abdominal presentations.

Benign duodenojejunal junction stricture: a case report.

Introduction: Duodenojejunal stricture is a rare entity that has been attributed to peptic stricture, malignancy, chronic pancreatitis, Crohn's disease and other benign causes. Case presentation: The authors present a case of a 67-year-old male who presented with upper abdominal pain for 2 weeks, 2 episodes of bilious vomiting, and inability to pass stool and flatus for 1 day. He had a history of chronic upper abdominal pain over the last 40 years and pulmonary tuberculosis 50 years back.Computed tomography (CT) scan of the abdomen and pelvis showed short segment narrowing in the fourth segment of the duodenum with dilated first, second and third segment duodenal loops. Resection and end-to-end duodenojejunal anastomosis was performed and the outcome was normal. Discussion: Benign duodenojejunal can be treated with balloon dilatation, stenting, strictureplasty and resection anastomosis. Treatment should be offered considering efficacy, availability, complications of these modalities and aetiology. Conclusion: Anterograde push enteroscopy and CT scan can aid in preoperative diagnosis of duodenojejunal stricture. Even in older age groups without prior surgical history, benign duodenojejunal stricture can be the cause of intestinal obstruction. Resection and end-to-end duodenojejunal anastomosis can be safe and effective treatment modalities for duodenojejunal junction stricture.

Acute abdomen due to perforated jejunal gastrointestinal stromal tumor: A case report.

INTRODUCTION: Gastrointestinal stromal tumors (GISTs) are rare mesenchymal tumors, with jejunal GISTs being particularly uncommon. Jejunal GISTs causing perforation and acute diffuse peritonitis is rare. CASE PRESENTATION: A 53-year-old female with a history of hypertension presented with severe, acute abdominal pain and vomiting. Examination revealed abdominal distension, tenderness, and guarding, with imaging suggestive of gastrointestinal perforation. Emergency laparotomy revealed a 9 cm × 8 cm mass with perforation in the jejunum, which was resected which on histopathological examination confirmed a low-grade GIST. The postoperative course was complicated by a wound infection, managed with antibiotics and secondary suturing. At one-year follow-up, the patient remained disease-free without the need for adjuvant therapy. CLINICAL DISCUSSION: The most common symptoms of jejunal GISTs include vague abdominal pain or discomfort, early satiety, obstruction or hemorrhage. Preoperative diagnosis and confirmation of GIST is difficult due to nonspecific symptoms and none of the radiographic procedures can establish the diagnosis with certainty. The surgical excision of the tumor along with infiltrated tissues is the treatment of choice for GIST. CONCLUSION: This case underscores the necessity of considering GISTs in differential diagnoses of acute abdomen and the critical role of prompt surgical management and multidisciplinary care in achieving favorable outcomes.

Emergency surgery for acute rectal bleeding in a newly diagnosed case of fulminant ulcerative colitis.

INTRODUCTION: The incidence of ulcerative colitis is rising among Asian population. Massive bleeding per rectum is an uncommon, but serious, complication of UC accounting for 0.1-1.4 % of admissions. CASE PRESENTATION: A 22-year-old male, reported persistent abdominal pain, bloody diarrhea, and intermittent vomiting for one week. Physical examination revealed signs of dehydration and pallor. Laboratory tests showed elevated inflammatory markers. CT and colonoscopy confirmed ulcerative colitis, refractory to corticosteroids, leading to a subtotal colectomy followed by Ileal Pouch-Anal Anastomosis. The patient was symptom free at a 24-month follow-up. DISCUSSION: Bleeding that occurs during the initial stages of the disease or in cases where the diagnosis of ulcerative colitis hasn't been confirmed makes it challenging for patients to accept the need for extensive surgery and the creation of a stoma. Conservative procedures are advised in such settings. CONCLUSION: Acute severe bleeding in ulcerative colitis is rare but demands a multidisciplinary approach for timely diagnosis and treatment. In resource and expertise limited situation, where patients are noncompliant to regular follow-ups surgical treatment can still be intervention of choice for these cases.

Coexistence of cecal duplication cyst and Meckel's diverticulum presenting as intestinal obstruction: A case report.

INTRODUCTION: Enteric duplication cysts (EDC) are rare anomalies of the gastrointestinal tract, with only 0.4 % occurring in the cecum. Meckel's diverticulum (MD) is a common congenital anomaly affecting up to 2 % of the population. The simultaneous occurrence of these two conditions is rare with no existing guideline on treatment. CASE PRESENTATION: An 11-month-old boy presented with fever, vomiting, and abdominal distension. A contrast-enhanced computed tomography scan confirmed the diagnosis of an enteric duplication cyst causing intestinal obstruction. The patient underwent exploratory laparotomy, during which a cecal duplication cyst measuring 30 × 20 mm was found along with MD in the distal ileum. Right limited hemicolectomy was performed. Histopathological examination revealed features consistent with an enteric duplication cyst and the presence of gastric mucosal heterotopia. CLINICAL DISCUSSION: Differentiating EDC from MD is a significant challenge, as both can present with similar symptoms and be positive on a Tc-99 m radionuclide scan. The final diagnosis of EDC and MD can only be made by correlating the imaging findings with the surgical findings and pathological features. CONCLUSION: Cecal duplication cysts should be considered a differential diagnosis in infants who present with intestinal obstruction. Although their presentation may resemble that of Meckel's diverticulum, both conditions can coexist. Excision of duplication cysts in children is considered a safe and efficient treatment approach.

Richter supraumbilical hernia managed with invagination: a case report.

Introduction and importance: Richter's hernia is an incarceration of the anti-mesenteric border of a segment of bowel through an abdominal wall defect. It primarily affects elderly individuals but can occur at any age, with a slightly increased incidence in females. The increase in laparoscopic and robotic-assisted procedures has led to a rise in Richter's hernias. Case presentation: A 40-year-old male with a history of laparoscopic cholecystectomy and kidney transplantation presented with a 4-day history of supraumbilical swelling and abdominal pain. The swelling was irreducible and accompanied by mild tenderness, and local signs of inflammation were exhibited. Intraoperatively, a 1.5 cm hernia defect was found, with the sac containing omentum and a portion of bowel segment for which invagination with serosal closure with the Mayo double-breasting technique was done. Clinical discussion: Richter's hernia presents with abdominal discomfort, bloating, nausea, and vomiting, with a notable feature being the delayed onset of symptoms due to its partial involvement of the bowel wall. Diagnosis can be achieved through a computed tomography (CT) scan or intraoperative exploration. Management of Richter hernia is contingent upon the patient's clinical condition, physical examination, and suspicion of strangulation. Conclusion: Diagnosis of Richter's hernia demands higher suspicion, particularly in patients with predisposing factors like a history of minimally invasive surgery. Prompt surgical intervention is crucial for reducing mortality and enhancing prognosis, with invagination alone being adequate if ischaemia is confined and mesh placement is unnecessary.

Hepatic angiomyolipoma masquerading as abdominal pain: A case report.

INTRODUCTION AND IMPORTANCE: Hepatic angiomyolipoma (HAML) is a rare liver tumor composed of blood vessels, smooth muscle, and fat cells. HAML occurs across a wide age range, with symptoms including abdominal discomfort, bloating, and weight loss. Diagnosis is challenging due to varied imaging appearances, but histopathological examination supplemented by immunohistochemical analysis, particularly using HMB-45, is definitive. CASE PRESENTATION: A 33-year-old man presented with a two-year history of right upper quadrant abdominal pain, occasionally relieved with analgesics but worsening over the past month and a half. Examinations revealed a soft, non-distended abdomen with a palpable liver. Laboratory tests, including viral markers and tumor markers were normal. Contrast-enhanced CT revealed a well-defined oval mass in liver segment III with heterogeneous enhancement leading to provisional diagnosis of HAML. The patient underwent a successful en bloc excision with no intraoperative or postoperative complications. CLINICAL DISCUSSION: Surgical resection is recommended for symptomatic cases or inconclusive biopsies, with stringent follow-up necessary due to the potential for recurrence and association with other malignancies. CONCLUSION: HAML may present with prolonged nonspecific abdominal symptoms. CT imaging aids in diagnosing cases with abundant fatty tissue. En bloc tumor excision proves safe and effective in treating symptomatic presentations.

Incidentally discovered intestinal malrotation during evaluation for blunt abdominal trauma: A case report.

INTRODUCTION AND IMPORTANCE: Intestinal malrotation is a congenital abnormality predominantly diagnosed in children, with only a few cases reported in adults. Patients may be incidentally identified during unrelated surgical procedures or postmortem examinations. It is crucial to promptly recognize this condition to prevent severe complications such as bowel ischemia and potential fatality. CASE PRESENTATION: A 40-year-old male presented to the Emergency Department after a child jumped on his abdomen with complaints of acute left upper quadrant abdominal pain progressing to be generalized. Examination showed pallor, abdominal tenderness without guarding or rigidity, and intact bowel sounds. Preoperative diagnostic tools revealed intestinal malrotation confirmed during the laparotomy, prompting the performance of Ladd's procedure to address the malrotation. CLINICAL DISCUSSION: Disruption in the normal embryological development of bowel is the cause of intestinal malrotation. The role of additional surgery especially in patients with asymptomatic disease related to malrotation is debated. CONCLUSION: Intestinal malrotation is rare in adults and often found incidentally during evaluation for unrelated medical conditions. Timely identification and surgical intervention usually result in positive outcomes. Our case underscores the incidental discovery of malrotation during the evaluation of blunt abdominal trauma, treated with Ladd's procedure. This is particularly significant due to geographical constraints associated with the patient's rural origin, as untreated malrotation could lead to complications in future occurrences.

Maternal exposure to folate antagonists and susceptibility to congenital heart disease in offspring: A systematic review and meta-analysis.

AIMS: The objective of this meta-analysis was to determine whether maternal exposure to folate antagonists is associated with increased rates of congenital heart disease in offspring. METHODS: A comprehensive search for articles in the MEDLINE (PubMed) and EMBASE databases published up to 21 August 2023 was performed. The search strategy was not limited by study design but only for articles in the English language. RESULTS: Analysis of 6 cohort studies and 5 cross-sectional studies, published between 1976 and 2020, showed significant increase in rate of congenital heart disease (odds ratio 1.55, 95% confidence interval, 1.28-1.87) when exposed to folate antagonists compared with the control. Further subgroup analysis showed the increased rate for exposure to both dihydrofolate reductase inhibitors and antiepileptic drugs separately. No differences were observed when analyses were stratified by timing of study. CONCLUSION: Administration of folate antagonists within the 12-week period preceding conception and throughout the second and third months of gestation exhibited a statistically significant elevation in the susceptibility to congenital heart diseases. Notably, the protective effect of folic acid supplementation was reported in cases of congenital heart disease linked to dihydrofolate reductase inhibitors but not that associated with antiepileptic drugs.

De novo subgaleal abscess complicated by spontaneous osteomyelitis and epidural abscess: a case report and review of literature.

Introduction and importance: A subgaleal abscess is a collection of pus in a potential space between the galea aponeurotica and pericranium. De novo subgaleal abscesses are a subset of subgaleal abscesses that develop in the absence of identifiable risk factors such as head trauma or procedures. However, these have rarely been reported in the literature. Case presentation: We present the case of a 65-year-old woman who presented with a headache for two and a half months, followed by swelling of the right parieto-occipital scalp. She denied any history of trauma, procedures, or anticoagulant use. A diagnosis of subgaleal abscess complicated by osteomyelitis and epidural abscess was made after obtaining a computed tomography of the head. Surgical treatment consisting of drainage, debridement, and craniectomy was performed, and the disease was successfully treated with a 6-week course of antibiotics. Clinical discussion: It is uncommon to have a de novo subgaleal abscess with spontaneous osteomyelitis and an epidural abscess as concurrent complications. The symptoms can be subtle, such as chronic headaches which can lead to delayed hospital visits. Computed tomography of the head is sufficient to make a definitive diagnosis. Appropriate antibiotic therapy and surgical intervention are necessary, which may encompass incision, drainage, debridement, and occasionally a craniectomy in order to achieve full resolution. Conclusions: One should be vigilant when evaluating scalp swelling for possible underlying abscesses. Prompt diagnosis and appropriate surgical treatment with adequate antibiotics are preferred treatment options for de novo subgaleal abscesses.

Prevalence and Clinical Types of Tremor in Multiple Sclerosis and its Associated Disability: A Systematic Review.

Objective: To describe the state of literature regarding prevalence, clinical types of tremor in Multiple Sclerosis and associated disability. Background: Tremor has long been recognized as an important symptom of multiple sclerosis. This can be intention and postural tremor that affects the upper limbs. Patients with multiple sclerosis who experience tremor of any severity typically retire early or lose their jobs due to disability. Methods: This systematic review was performed up to September 9, 2022. Article selection was performed by searching the MEDLINE (PubMed) and EMBASE electronic bibliographic databases. The search strategy was not limited by study design but only for articles in the English language. Results: A total of nine full-text articles were included in the analysis. Six studies were cross-sectional studies; one each was a prospective observational study, a case-control study, a community-based cohort. The prevalence of tremor in the multiple sclerosis (MS) population among studies ranged widely, between 12.5% and 68.9%. The presence of severe tremor ranged from 3% to 33%. Younger age was a significant predictor of tremor in two studies. The most common tremor subtype was action tremor. Upper extremities were the most common site involved in the majority of our studies, followed by head and neck. Conclusions: Prevalence of tremor ranged from 12.5% to 68.9% in the MS population with severe tremor being an infrequent complication. Severity of tremor correlated with increasing disability. Upper limb action tremor was the most common with rare occurrences of resting and rubral tremor.

Mesenteric Ischemia and Its Need for Timely Recognition and Management.

Mesenteric ischemia is a fatal vascular emergency of the small intestine which, if not diagnosed and treated in time, has a very high mortality rate. Presenting with nonspecific symptoms such as abdominal pain, nausea, constipation, tachycardia, and gastrointestinal bleeds, it can masquerade as other causes of acute abdomen, particularly bowel obstruction. Ideal laboratory tests and markers are still lacking due to complexity in bowel's anatomy, physiology, blood supply, and drainage. We report 10 cases of mesenteric ischemia presented in our center with their demography, laboratory findings, approach to diagnosis, and treatment along with their outcomes at discharge. Out of the ten cases, six cases presented with signs and symptoms of acute bowel obstruction without passage of stool and one with passage of black stool. These seven patients underwent laparotomy, while the remaining three cases who either presented with milder symptoms or refused surgical interventions were managed conservatively. All patients were diagnosed with either acute or chronic mesenteric ischemia based on their operative and/or radiographic findings.

Guillain-Barre Syndrome following lower segment caesarean section under spinal anesthesia: A case report.

Symptoms of Guillain-Barre Syndrome (GBS) may be mistaken for typical puerperal changes, delaying diagnosis. Surgery and anesthesia may be triggers for GBS with an overall increase in pro-inflammatory cytokines in the postpartum period. We report a unique case of GBS in the postpartum period who made a good recovery with supportive measures.

Guillain-Barré syndrome following coronavirus disease vaccine: First report from Nepal.

ChAdOx1 nCoV-19 is an effective and well-tolerated coronavirus disease 2019 vaccine. However, rare cases of serious adverse events have been reported with it. We report a patient who did not have active or prior coronavirus disease 2019 infection, who developed Guillain-Barré syndrome 7 days following the first dose of ChAdOx1 nCoV-19 vaccination. He was treated with intravenous immunoglobulin, with stabilization of the disease. Proper monitoring and prompt reporting of such cases are required to ensure the safety of the vaccine.

Paraparetic Guillain-Barre syndrome: An uncommon diagnosis of acute flaccid paralysis of the lower limbs.

Apart from the usual differentials of transverse myelitis and cord compression, paraparetic GBS should be considered when sudden, flaccid paralysis of the lower limbs occurs, as prompt diagnosis and management can minimize sequel and unnecessary procedures. We do report a case wherein we managed a similar situation without the use of an immunomodulatory therapy.