Pediatric Education and Research

Cerebral Palsy (CP), a non-progressive central motor disorder is characterized by deformities which are progressive and often merit corrective surgeries to limit these deformities for proper rehabilitation of the patient. Anaesthesia in such patients is challenging due to disabilities and altered pharmacokinetics of different drugs. Specific concerns include intraoperative hypothermia, slow emergence, increased risks of aspiration due to excessive Oro-pharyngeal secretions and gastro-esophageal reflux, recurrent Pneumonia, history of seizures and anti-seizure medications, respiratory depression, and post-operative pain and muscle spasms. We describe the anesthetic management of a five-year-male with spastic CP, who underwent corrective surgery for bilateral Congenital Telipes Equino varus (CTEV) and deformities in lower limb and emphasize upon these risks and their management. This case presented to the pediatric outpatients’ clinic as a diagnosed case of spastic cerebral palsy with seizures and bilateral congenital telipes equino varus (CTEV) and lower limb deformity. He also had a past history of recurrent pneumonia for which he received treatment in the pediatric medicine department. Child had been on Valproate for seizures, Baclofen for muscle spasms and Ranitidine for gastro-esophageal reflux disease (GERD). Surgery had been cancelled several times in the past due to the poor general condition of the child and the high risk involved. On examination, this patient weighed 15 KG and was pale, anicteric and tachycardic with a blood pressure of 96/64 mm Hg. He had delayed developmental milestones including language, social and mental milestones. Airway was normal with Mallampatti grade -1 on examination. Excessive oral and pharyngeal secretions were present and occasional drooling of saliva was noticed. There was also the presence of gastro-esophageal reflux. Patient had obvious Kypho-scoliosis and spinal deformity. He had bilateral CTEV with several contractures. Increased muscle tone, tense contracted muscles and exaggerated deep tendon reflexes were other significant findings. Examination of the respiratory system revealed bilateral equal air entry but there was crepitation in the right middle

Macrophage Activation Syndrome (MAS) is an unusual and severe complication in Mixed Connective Tissue Disease (MCTD). It involves multi-organ system and can be fatal if identified late.1,2 A 17-year-old female, diagnosed with MCTD one year prior and maintained on hydroxychloroquine, mycophenolate mofetil, and low-dose steroids, presented with 7 days of fever and facial puffiness with vitals stable. On admission child had pallor, skin thickening, digital pitting edema. On admission MAS workup was negative. After 3 days of admission, the patient developed neuropsychiatric symptoms and seizures suggestive of neurolupus and MAS that was confirmed on repeat workup. She was managed with intravenous immunoglobulin (2 g/kg over 5 days), broad-spectrum antibiotics, and pulse methylprednisolone therapy followed by high-dose oral prednisolone. Cyclosporine was added for disease activity control. Child developed concurrent renal involvement in the form of proteinuria and positive dsDNA antibodies, started on intravenous cyclophosphamide. This case is notable for MAS at any phase of disease along with disease flare in MCTD, complicated by Central nervous system and renal lupus activity. MAS is a rare but life-threatening hyperinflammatory syndrome, more frequently reported in systemic juvenile idiopathic arthritis than in MCTD. Prompt recognition and aggressive immunosuppression are essential to prevent irreversible organ damage. Our case underscores the importance of considering MAS in any patient with connective tissue disease presenting with unexplained fever, cytopenias, hyperferritinemia, and organ dysfunction. Early diagnosis and initiation of targeted immunosuppressive therapy can be lifesaving.3,4 Child is on monthly cyclophophamide and asymptomatic.

Background: Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart defect (1–2% of congenital heart diseases). The obstructed form, particularly infracardiac or mixed types, often presents as a neonatal emergency with hypoxemia and rapid deterioration, mimicking pneumonia or respiratory distress syndrome (RDS). Case Presentation: A late-preterm male neonate (35 + 5 weeks, 2.77 kg) developed early tachypnea and hypoxemia (SpO2 93% on room air). Chest X-ray showed a left upper-zone opacity, and congenital pneumonia was suspected. Despite oxygen, surfactant, and antibiotics, the infant progressed to severe mixed acidosis (pH 7.10, pCO2 139 mmHg, lactate 11 mmol/L), refractory shock, polycythemia (PCV 71%), and acute kidney injury. Echocardiography initially revealed only an atrial septal defect. The neonate died at 42 hours of life. Retrospective review suggested a missed obstructed TAPVC with ASD. Conclusion: Obstructed TAPVC should be suspected in neonates with persistent hypoxemia and acidosis unresponsive to routine management. Early repeat echocardiography focusing on pulmonary venous drainage is crucial. This case emphasises that clinical vigilance and timely reassessment remain vital for detecting critical congenital heart disease.

Gastric Duplication cysts form a very small subset of the rare Duplication anomalies involving the gastrointestinal tract. They have variable presentation and may present as an upper abdominal mass with obstructive or pressure symptoms. Excision of the lesion followed by histopathology clinches the diagnosis. We describe the case of an infant with gastric duplication cyst involving the greater curvature of the stomach with features of gastric outlet obstruction. He had upper abdominal distension with repeated episodes of non-bilious vomiting for one month. On examination, two conspicuous swellings with visible peristalsis from left to right side were found. Ultrasound and computed tomogram scan of the abdomen confirmed the diagnosis of cystic lesions in relation to the greater curvature of the stomach, suggesting a diagnosis of Gastric Duplication Cyst. There were no other associated anomalies in this patient. Excision of the lesion led to resolution of symptoms and histopathology confirmed the diagnosis.