INI-CET 2015 — Pediatrics

Q: Identify the congenital defect seen in this baby.

Craniorachischisis totalis. ***Craniorachischisis totalis*** - This is a severe form of **neural tube defect** characterized by complete failure of closure of the neural tube, involving both the **cranial and spinal regions**. - The image shows an extensive defect affecting the entire length of the neural axis, with exposed brain tissue and spinal cord, which is consistent with **craniorachischisis totalis**. *Cystic hygroma* - A **cystic hygroma** is a congenital malformation of the lymphatic system, typically appearing as a multicystic mass, most commonly in the **neck or axilla**. - It does not involve exposed brain or spinal cord tissue and has a different appearance than the extensive defect shown in the image. *Cervical meningocele* - A **cervical meningocele** is a type of spina bifida where only the **meninges** (membranes surrounding the spinal cord) protrude through a defect in the cervical spine. - While it involves the spine, it is usually a localized sac-like protrusion, and the brain itself is not extensively exposed as seen in the image. *Encephalocele* - An **encephalocele** is a neural tube defect where there is a protrusion of brain tissue and/or meninges through an opening in the **skull**, typically at the back of the head. - While it involves the brain, it is generally a localized defect of the skull, not extending the entire length of the spine and cranium as depicted in the image. [Practice more Pediatrics PYQs on OnCourse]

Q: A 4-year-old admitted in ward with pneumonia. He develops sudden onset of breathlessness. What is the next step in management?

Emergency needle thoracostomy. ***Emergency needle thoracostomy*** - This patient, a 4-year-old with pneumonia and sudden breathlessness, likely has a **tension pneumothorax**, which is a life-threatening emergency requiring immediate decompression. The chest X-ray shows a collapsed right lung and a mediastinal shift, consistent with tension pneumothorax. - An **emergency needle thoracostomy** (needle decompression) is the immediate life-saving procedure to relieve the pressure in a tension pneumothorax before more definitive treatment can be initiated. - Performed by inserting a large-bore needle (14-16G) into the **2nd intercostal space, mid-clavicular line** on the affected side. *Intercostal drainage tube insertion* - While an intercostal drainage tube (chest tube) is the definitive treatment for pneumothorax, it takes more time to insert and is not the immediate first step for a **tension pneumothorax** in an unstable patient. - The delay in performing needle decompression could be fatal in a rapidly deteriorating patient with tension pneumothorax. *Decrease mechanical ventilation setting* - Decreasing mechanical ventilation settings would not address the underlying pathology of a tension pneumothorax, which is trapped air causing lung collapse and mediastinal shift. - This action could further compromise the patient's respiratory status if the pneumothorax is severe and the patient is already hypoxemic. *Increase mechanical ventilation setting* - Increasing mechanical ventilation settings would likely worsen a **tension pneumothorax** by forcing more air into the pleural space and increasing intrathoracic pressure. - This would further compromise venous return to the heart and reduce cardiac output, rapidly leading to **cardiovascular collapse**. [Practice more Pediatrics PYQs on OnCourse]

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Identify the congenital defect seen in this baby.

A 4-year-old admitted in ward with pneumonia. He develops sudden onset of breathlessness. What is the next step in management?

INI-CET 2015 - Pediatrics INI-CET Practice Questions and MCQs

Question 1: Identify the congenital defect seen in this baby.

A. Craniorachischisis totalis (Correct Answer)
B. Cystic hygroma
C. Cervical meningocele
D. Encephalocele

Explanation: ***Craniorachischisis totalis*** - This is a severe form of **neural tube defect** characterized by complete failure of closure of the neural tube, involving both the **cranial and spinal regions**. - The image shows an extensive defect affecting the entire length of the neural axis, with exposed brain tissue and spinal cord, which is consistent with **craniorachischisis totalis**. *Cystic hygroma* - A **cystic hygroma** is a congenital malformation of the lymphatic system, typically appearing as a multicystic mass, most commonly in the **neck or axilla**. - It does not involve exposed brain or spinal cord tissue and has a different appearance than the extensive defect shown in the image. *Cervical meningocele* - A **cervical meningocele** is a type of spina bifida where only the **meninges** (membranes surrounding the spinal cord) protrude through a defect in the cervical spine. - While it involves the spine, it is usually a localized sac-like protrusion, and the brain itself is not extensively exposed as seen in the image. *Encephalocele* - An **encephalocele** is a neural tube defect where there is a protrusion of brain tissue and/or meninges through an opening in the **skull**, typically at the back of the head. - While it involves the brain, it is generally a localized defect of the skull, not extending the entire length of the spine and cranium as depicted in the image.

Question 2: A 4-year-old admitted in ward with pneumonia. He develops sudden onset of breathlessness. What is the next step in management?

A. Intercostal drainage tube insertion
B. Emergency needle thoracostomy (Correct Answer)
C. Decrease mechanical ventilation setting
D. Increase mechanical ventilation setting

Explanation: ***Emergency needle thoracostomy*** - This patient, a 4-year-old with pneumonia and sudden breathlessness, likely has a **tension pneumothorax**, which is a life-threatening emergency requiring immediate decompression. The chest X-ray shows a collapsed right lung and a mediastinal shift, consistent with tension pneumothorax. - An **emergency needle thoracostomy** (needle decompression) is the immediate life-saving procedure to relieve the pressure in a tension pneumothorax before more definitive treatment can be initiated. - Performed by inserting a large-bore needle (14-16G) into the **2nd intercostal space, mid-clavicular line** on the affected side. *Intercostal drainage tube insertion* - While an intercostal drainage tube (chest tube) is the definitive treatment for pneumothorax, it takes more time to insert and is not the immediate first step for a **tension pneumothorax** in an unstable patient. - The delay in performing needle decompression could be fatal in a rapidly deteriorating patient with tension pneumothorax. *Decrease mechanical ventilation setting* - Decreasing mechanical ventilation settings would not address the underlying pathology of a tension pneumothorax, which is trapped air causing lung collapse and mediastinal shift. - This action could further compromise the patient's respiratory status if the pneumothorax is severe and the patient is already hypoxemic. *Increase mechanical ventilation setting* - Increasing mechanical ventilation settings would likely worsen a **tension pneumothorax** by forcing more air into the pleural space and increasing intrathoracic pressure. - This would further compromise venous return to the heart and reduce cardiac output, rapidly leading to **cardiovascular collapse**.