Evaluation of the Resources and Inequities Among Pediatric Critical Care Facilities in Pakistan.

OBJECTIVES: To evaluate nationwide pediatric critical care facilities and resources in Pakistan. DESIGN: Cross-sectional observational study. SETTING: Accredited pediatric training facilities in Pakistan. PATIENTS: None. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A survey was conducted using the Partners in Health 4S (space, staff, stuff, systems) framework, via email or telephone correspondence. We used a scoring system in which each item in our checklist was given a score of 1, if available. Total scores were added up for each component. Additionally, we stratified and analyzed the data between the public and private healthcare sectors. Out of 114 hospitals (accredited for pediatric training), 76 (67%) responded. Fifty-three (70%) of these hospitals had a PICU, with a total of 667 specialized beds and 217 mechanical ventilators. There were 38 (72%) public hospitals and 15 (28%) private hospitals. There were 20 trained intensivists in 16 of 53 PICUs (30%), while 25 of 53 PICUs (47%) had a nurse-patient ratio less than 1:3. Overall, private hospitals were better resourced in many domains of our four Partners in Health framework. The Stuff component scored more than the other three components using analysis of variance testing ( p = 0.003). On cluster analysis, private hospitals ranked higher in Space and Stuff, along with the overall scoring. CONCLUSIONS: There is a general lack of resources, seen disproportionately in the public sector. The scarcity of qualified intensivists and nursing staff poses a challenge to Pakistan's PICU infrastructure.

Congenital diaphragmatic hernia in patient with 1p36 deletion.

Key Clinical Message: This case underscores the atypical presentation of late-onset congenital diaphragmatic hernia in a 9-old with 1p36 deletion syndrome. Recognition of respiratory distress and abdominal symptoms is crucial for intervention. Abstract: Congenital Diaphragmatic Hernia (CDH) is a condition characterized by the protrusion of abdominal contents into the thoracic cavity due to a defect in the diaphragm. While typically observed in the neonatal period, CDH can present in later life. This case report describes the presentation, diagnosis, and management of a nine-year-old boy with 1p36 deletion syndrome who presented with respiratory distress, abdominal pain, vomiting, and anorexia. The initial diagnosis was tension pneumothorax, and thus the patient underwent chest tube placement. However, a high-resolution CT scan revealed a left hemidiaphragmatic hernia, and the patient eventually underwent an emergency laparotomy due to acute-onset respiratory distress. Intraoperatively, a diagnosis of Bochdalek hernia with gastric perforation was made, and the CDH and gastric perforations were resolved successfully. This case highlights the importance of considering late-presenting CDH as a possible diagnosis in pediatric patients with similar symptoms and the radiological findings suggestive of tension pneumothorax. Early recognition and prompt surgical intervention can lead to successful management of such cases.

Tecto-cerebellar dysraphia manifesting as occipital meningocoele associated with congenital melanocytic nevi and pectus excavatum.

BACKGROUND: Only few reported cases of tectocerebellar dysraphia with occipital encephalocele have been reported in the literature. CASE PRESENTATION: Three month baby boy, the first child of healthy, consanguineous parents presented with a small swelling over the occipital region since birth. The child also used to have apneic spells without cyanosis and spontaneous recovery. CT scan showed absence of the cerebellar vermis, absence of tectum and the 4(th) ventricle communicating with the occipital menigocoele sac and an occipital bone defect. The excision of the encephalocoele sac was performed, however the child continued to have apneic spells and did not do well. CONCLUSION: In our child irregular respiration probably was the manifestation of the tecto-cerebellar dysraphia syndrome complex and associated shunt malfunction followed by seizures decompensated the physiology of the child leading to fatal outcome.