Chylothorax: A Stepwise Approach to Diagnosis and Treatment

Chylothorax, a lymphatic flow disorder characterized by an abnormal circulation of lymph fluid into the pleural cavity, is the most common cause of pleural effusions during the neonatal period. This condition affects 1/15,000 neonates every year. Affected neonates often manifest with respiratory distress, electrolyte imbalances, sepsis, and even immunodeficiencies. Mortality risk is highest among neonates undergoing cardiac surgery as well as those with associated hydrops fetalis. Conservative treatment options include bowel rest with administration of parenteral nutrition, followed with medium-chain triglyceride enteral feedings, and octreotide therapy. Severe or persistent cases require surgical intervention. This can involve a unilateral or bilateral pleurectomy and thoracic duct ligation, with or without pleurodesis. Early identification and successful treatment of this condition is contingent upon awareness of the most current evidence and a timely cross-disciplinary approach to care.

TO STUDY THE EFFECTIVENESS OF CONSERVATIVE MANAGEMENT OF ACUTE PANCREATITIS

Background: Acute pancreatitis is the leading cause of hospitalization for gastrointestinal disorders in the United States, with more than 280,000 hospitalizations each year. Methods: This study was conducted in the department of surgery, RNT Medical College and Maharana Bhupal Govt. Hospital, Udaipur. A total of 35 cases of acute pancreatitis were studied Results: Out of 35 cases of acute pancreatitis, 26(74.28%) patient were given Octreotide therapy. 9(25.7%) patient out of 35 were not given Octreotide therapy. Conclusion: Thus it may be concluded that most of these cases can be managed conservatively and the use of Octreotide therapy helps in the favourable outcome. Keywords: Conservative, pancreatitis, Managed

TO STUDY THE MORBIDITY AND MORTALITY OF ACUTE PANCREATITIS

Background: Acute pancreatitis is a protean disease capable of wide clinical variation ranging from mild discomfort to apocalyptic prostration Methods: This study was conducted in the department of surgery, RNT Medical College and Maharana Bhupal Govt. Hospital, Udaipur. A total of 35 cases of acute pancreatitis were studied Results: Out of these 35 patients, 25(71.42%) had favourable outcome, while 4(15.4%) patient had complication (pancreatic pseudocyst in 3, and recurrence after 7 months in 1 patient), 3(8.57%) patient died and 3 (8.57%) patient could not be followed up due to non-compliance of patient. Conclusion: We concluded that history, clinical examination, radiological and blood investigations are helpful to come to a conclusive diagnosis and severity of acute pancreatitis and most of these cases can be managed conservatively and the use of Octreotide therapy helps in the favourable outcome. Keywords: Conservative, Pancreatitis, Outcome

Successful use of long-acting octreotide for protracted gastrointestinal bleeding related to protein-losing enteropathy after the Fontan procedure: a case report

Gastrointestinal bleeding complicated with protein-losing enteropathy after the Fontan procedure has been often reported in recent years, but there is no established therapy for it. We report the case of an 18-year-old boy who suffered from abdominal pain, melena, and anaemia due to intractable haemorrhagic protein-losing enteropathy after the Fontan procedure. He was successfully treated with octreotide therapy.

Treatment of Congenital Chylothorax Using High-Dose Octreotide

Introduction: Neonatal chylothorax is a rare and potentially life-threatening condition with a reported incidence of 1 in 5,800 - 24,000 and a high mortality rate of up to 64%. Octreotide is one of the treatment options available to reduce both the splanchnic blood flow and the intestinal secretion of electrolytes and water. However, there is no uniform guideline for the optimal dosage of octreotide in the treatment of congenital chylothorax. Case Presentation: We present the case of a neonate diagnosed with congenital chylothorax. At birth, the neonate manifested severe respiratory distress warranting cardiopulmonary resuscitation for 25 min. During admission, intravenous octreotide treatment was initiated and feeding was changed from breast milk to a medium-chain triglyceride-enriched formula. Bilateral hilar enlargement and diffusely increased opacities waxed and waned, and we gradually increased the octreotide dosage daily by 1 ~ 2 mcg/kg/h. After 3 days of octreotide administration at the rate of 20 mcg/kg/h, chest x-ray showed regression of pleural effusion. The patient was discharged with full bottle feeding achieved. Conclusions: High-dose octreotide therapy (with doses up to 20 mcg/kg/h) may be recommended for patients with idiopathic congenital chylothorax. However, patients should be carefully monitored for side-effects such as hyperglycemia, necrotizing enterocolitis, transient hypothyroidism, and ileus.

Management of large congenital chylous ascites in a preterm infant: fetal and neonatal interventions

Congenital chylous ascites is a rare cause of ascites in newborn infants. Its aetiology varies from localised leaky lymphatic duct to genetic syndromes. Most of these cases have transient ascites resolving over time with conservative management but some may progress needing medical as well as surgical treatment. We describe a case of antenatally detected large fetal ascites necessitating abdominal paracentesis and amnioreduction. Marked respiratory distress at birth required urgent abdominal paracentesis to relieve symptoms. The infant initially showed a good response to medium chain triglyceride (MCT) based formula milk feeds. Feeds were discontinued for 3 weeks due to sepsis with ileus. On recovery, recommencement of feeds resulted in reaccumulation of ascites. As the response to MCT-based formula was inadequate, octreotide therapy was initiated. Ascites showed remarkable resolution over the next 2 weeks and was discharged home. Follow-up at 5 years of age revealed normal growth and neurodevelopment.

Octreotide-related exocrine pancreatic insufficiency (EPI) in congenital hyperinsulinism

BackgroundCongenital hyperinsulinism (CH) is the most frequent cause of persistent hypoglycemia in the newborn. Octreotide, a long-acting somatostatin receptor analog (SSRA), is a second line treatment for diazoxide unresponsive CH patients. Although it has been found to be a safe and effective treatment, long-term benefits and side effects, have not been thoroughly evaluated.Case presentationSome authors have indicated that exocrine pancreatic insufficiency (EPI) is a common but under-recognized adverse reaction in adults treated with octreotide. However, no pediatric patient with SSRA-induced EPI has been reported to date. Here we report a case of an infant with diazoxide unresponsive, diffuse CH, caused by a heterozygous pathogenic paternally inherited mutation in the ABCC8 gene (NM_000352.4:c.357del), that developed exocrine pancreatic insufficiency and secondary vitamin K deficiency associated to chronic octreotide therapy.ConclusionsWe point out the atypical clinical onset with a cutaneous hemorrhagic syndrome, emphasizing the clinical relevance of this potential side effect.

Clinical Efficacy Evaluation of Sirolimus in Congenital Hyperinsulinism

Background. Congenital hyperinsulinism (CHI) is a rare and life-threatening genetic disorder. Sirolimus as a mammalian target of rapamycin inhibitor may be helpful in patients with CHI who do not respond well to other treatments including diazoxide and octreotide. However, the safety and efficacy of this therapy are still unclear. This study aimed to evaluate the potential therapeutic effects of sirolimus in CHI patients with mutations in the ABCC8 and KCNJ11 genes. Methods. During the period of this follow-up study, every child with a confirmed diagnosis of unresponsive CHI underwent genetic evaluation. Among those who had positive genetic testing, six families agreed to participate in this study. The participants were evaluated for ABCC8, KCNJ11, or HNF4α gene mutations by polymerase chain reaction (PCR) sequencing. The participants who were unresponsive to diazoxide and octreotide therapy received 0.5 mg/m2/d of sirolimus, and the dose was gradually increased until a serum concentration of 5–15 ng/ml was achieved. Then, the participants were followed up for any possible complications. Results. Among the study participants, only one neonate was completely free of hypoglycemia after one year of follow-up, whereas three others experienced a partial reduction in hypoglycemic episodes over six months. One neonate underwent pancreatectomy despite receiving sirolimus. The oldest participant with a mutation in the ABCC8 gene responded well to sirolimus therapy after surgery and remained asymptomatic for 18 months. Conclusion. This study suggested that sirolimus therapy needs further evaluation to determine which patients will benefit the most. The genetic basis of CHI may have possible implications for determining the patient’s response.