Pediatric Surgery Practice Questions

Q: What is not part of the curative therapy for medulloblastoma in a 1.5-year-old child?

Open surgery with leaving tumor margins. **Explanation:** Medulloblastoma is a highly malignant WHO Grade 4 embryonal tumor of the posterior fossa. The primary goal of surgery is **maximal safe resection**. **Why Option A is the correct answer:** In oncological surgery for medulloblastoma, the objective is "Gross Total Resection" (GTR). Leaving tumor margins intentionally is incorrect because the volume of residual tumor is a critical prognostic factor. A residual tumor area $>1.5 \text{ cm}^2$ classifies the patient as "High Risk," significantly worsening the prognosis. Therefore, leaving margins is not part of curative therapy. **Analysis of other options:** * **B. Chemotherapy:** This is a cornerstone of treatment, especially in children under 3 years of age, to delay or avoid the neurocognitive side effects of radiation. * **C. Total Craniospinal Irradiation (CSI):** Medulloblastoma has a high propensity for CSF seeding (drop metastases). CSI is standard for "sealing" the neuraxis, though it is often deferred in children $<3$ years old to prevent developmental delay. * **D. VP Shunt:** Approximately 80% of patients present with obstructive hydrocephalus. While many are managed with perioperative steroids or EVD, a permanent VP shunt is often required for symptomatic relief and stabilization. **High-Yield Clinical Pearls for NEET-PG:** * **Origin:** Arises from the **external granular layer** of the cerebellum; typically found in the **vermis** (midline) in children. * **Histology:** Characterized by **Homer-Wright Rosettes** and small round blue cells. * **Genetics:** Four molecular subgroups exist: **WNT** (best prognosis), **SHH**, Group 3 (worst prognosis), and Group 4. * **Classic Sign:** "Drop metastasis" to the cauda equina.

Q: What is the initial surgical treatment for Tetralogy of Fallot (TOF)?

Modified Blalock-Taussig shunt. **Explanation:** Tetralogy of Fallot (TOF) is a cyanotic congenital heart disease characterized by pulmonary stenosis, right ventricular hypertrophy, overriding of the aorta, and a VSD. The definitive treatment is total surgical correction; however, in neonates with severe cyanosis, hypercyanotic spells, or hypoplastic pulmonary arteries, an **initial palliative procedure** is required to increase pulmonary blood flow. **1. Why Modified Blalock-Taussig (mBT) Shunt is correct:** The mBT shunt is the preferred initial palliative surgery. It involves creating a connection between the **subclavian artery and the ipsilateral pulmonary artery** using a synthetic GORE-TEX graft. This mimics a Patent Ductus Arteriosus (PDA), ensuring adequate blood reaches the lungs for oxygenation, allowing the pulmonary arteries to grow before definitive repair. **2. Why other options are incorrect:** * **Fontan Procedure:** Used for "single ventricle" physiology (e.g., Tricuspid Atresia). It redirects systemic venous return directly to the pulmonary arteries, bypassing the right ventricle. * **Glenn Shunt:** A bidirectional superior vena cava-to-pulmonary artery anastomosis. It is a second-stage procedure for single ventricle physiology, not TOF. * **Rastelli Operation:** Used for TOF with Pulmonary Atresia or Transposition of Great Arteries (TGA) with VSD/PS. It involves using a valved conduit to connect the RV to the pulmonary artery. **High-Yield Clinical Pearls for NEET-PG:** * **Classic BT Shunt:** Direct anastomosis of the subclavian artery to the pulmonary artery (rarely done now due to limb ischemia). * **Boot-shaped heart (Coeur en sabot):** Classic X-ray finding in TOF due to RV hypertrophy and an upturned apex. * **Most important prognostic factor:** The severity of Right Ventricular Outflow Tract (RVOT) obstruction. * **Drug of choice for Tet Spells:** Morphine (reduces infundibular spasm) and IV Beta-blockers (Propranolol).

Q: What is the incidence of undescended testis in term infants?

<5%. **Explanation:** The descent of the testis into the scrotum is a complex process occurring in two phases: the transabdominal phase (7–15 weeks) and the inguinoscrotal phase (25–35 weeks). Because the final descent occurs late in the third trimester, the incidence of undescended testis (UDT) is highly dependent on gestational age. **1. Why Option A is correct:** In **full-term infants**, the incidence of undescended testis is approximately **3% to 4%** (fitting the **<5%** category). It is important to note that spontaneous descent often occurs during the first few months of life, particularly in the first 3 to 6 months, bringing the incidence down to about 1% by the age of one year. **2. Why the other options are incorrect:** * **Option B (<1%):** This represents the incidence of UDT in the general male population after the age of one year (post-spontaneous descent period). * **Options C & D (8% and 10%):** These values are too high for term infants. However, in **preterm infants**, the incidence is significantly higher, ranging from **20% to 30%**, as they are born before the inguinoscrotal phase of descent is complete. **Clinical Pearls for NEET-PG:** * **Most common site:** The most common location for an undescended testis is the **inguinal canal**. * **Spontaneous Descent:** If the testis does not descend by **6 months** of age, spontaneous descent is unlikely. * **Management:** The current gold standard is **Orchidopexy**, ideally performed between **6 to 12 months** of age to preserve fertility and allow for early detection of testicular cancer. * **Complications:** UDT increases the risk of infertility, inguinal hernia, testicular torsion, and **testicular germ cell tumors** (most commonly seminoma). Note that orchidopexy reduces the risk of malignancy but does not eliminate it.

Q: Cystic hygroma may be associated with which of the following?

All of the above. **Explanation:** **Cystic Hygroma** (also known as macrocystic lymphatic malformation) is a congenital malformation of the lymphatic system. It occurs due to the failure of the jugular lymph sacs to communicate with the internal jugular vein, leading to the sequestration of lymphatic tissue and subsequent cystic dilatation. **Why "All of the Above" is correct:** Cystic hygromas are frequently associated with chromosomal aneuploidies. While they are most classically linked to **Turner’s syndrome (45, XO)**, where they often present as fetal hydrops or nuchal translucency, they are also significantly associated with other trisomies and sex chromosome abnormalities: * **Turner’s Syndrome:** The most common association (up to 80% of fetuses with cystic hygroma). * **Down’s Syndrome (Trisomy 21):** A frequent association found during prenatal screening. * **Klinefelter’s Syndrome (47, XXY):** Though less common than Turner’s, it is a documented association in literature. * **Other associations:** Edward’s syndrome (Trisomy 18) and Patau syndrome (Trisomy 13). **Clinical Pearls for NEET-PG:** * **Most common site:** Posterior triangle of the neck (Left side > Right side). * **Clinical Feature:** It is a soft, painless, compressible, and **brilliantly transilluminant** swelling. * **Complications:** Sudden increase in size due to hemorrhage or infection; respiratory distress if it compresses the airway. * **Treatment of choice:** Surgical excision is the gold standard. For unresectable cases or as an adjunct, sclerotherapy (using **OK-432/Picibanil** or Bleomycin) is used. * **Note:** It is not a true neoplasm but a malformation; however, it can infiltrate tissue planes, making complete surgical removal challenging.

Q: Sacrococcygeal teratoma is associated with which of the following?

Meningocele. **Explanation:** Sacrococcygeal Teratoma (SCT) is the most common newborn tumor, arising from the **totipotent cells of Hensen’s node** (primitive streak). The correct association is **Meningocele** (Option A) because both SCT and neural tube defects occur due to developmental anomalies at the caudal end of the embryo. Specifically, SCT is a key component of the **Currarino Triad**, which consists of: 1. Anorectal malformation (usually anal stenosis) 2. Sacral bony abnormality (scimitar sacrum) 3. Presacral mass (teratoma, meningocele, or enteric cyst) **Analysis of Incorrect Options:** * **B, C, and D:** While SCT can be associated with other anomalies in about 10-15% of cases (such as imperforate anus or urinary tract abnormalities), there is no established or high-yield syndromic association with cardiac defects, hepatic cysts, or congenital cystic adenomatoid malformation (CCAM). **High-Yield Clinical Pearls for NEET-PG:** * **Epidemiology:** Most common in females (4:1 ratio), but malignancy is more common in males. * **Altman Classification:** * **Type I:** Predominantly external (most common). * **Type IV:** Entirely internal/presacral (highest risk of malignancy due to delayed diagnosis). * **Diagnosis:** Elevated **Alpha-fetoprotein (AFP)** is a marker for yolk sac components (malignancy) and is used for post-operative monitoring. * **Surgical Management:** Complete excision of the tumor **along with the coccyx** is mandatory to prevent recurrence. * **Complication:** Large tumors can cause "Maternal Mirror Syndrome" or fetal hydrops due to high-output cardiac failure (vascular steal).

Q: What is true about Bochdalek hernia?

It is a differential diagnosis for pleuropericardial cyst.. **Explanation:** **Bochdalek Hernia** is the most common type of congenital diaphragmatic hernia (CDH), resulting from the failure of the pleuroperitoneal canal to close during embryonic development. **Why Option C is correct:** A Bochdalek hernia typically presents as a mass in the posterior mediastinum or the cardiophrenic angle. On imaging (like a chest X-ray or CT), a fat-containing hernia or a hernia involving abdominal viscera can mimic other cystic or solid lesions in the lower mediastinum. Therefore, it is a recognized **differential diagnosis for a pleuropericardial cyst**, as both can appear as masses near the diaphragm/heart borders. **Why other options are incorrect:** * **Option A:** Bochdalek hernias are overwhelmingly **left-sided (approx. 80-85%)**. The right side is protected by the presence of the liver, which acts as a physical barrier during development. * **Option B:** It is located **posterolaterally**. In contrast, the **Morgagni hernia** is the one located in an anterior (retrosternal) position. **High-Yield Clinical Pearls for NEET-PG:** * **Mnemonic:** **B**ochdalek is **B**ack and **B**ig (Posterior and more common); **M**orgagni is **M**idline/Anterior and **M**ore rare. * **Clinical Triad:** Scaphoid abdomen, respiratory distress, and shifted heart sounds (dextrocardia if left-sided). * **Most Important Prognostic Factor:** The degree of **pulmonary hypoplasia** and associated pulmonary hypertension, rather than the defect size itself. * **Management:** It is a physiological emergency, not a surgical one. Stabilize the infant (intubation, avoid bag-mask ventilation) before considering surgical repair.

Q: A 9-month-old girl presents with perianal bleeding, vomiting, a right lumbar mass, and masked liver dullness. She is in a shock-like condition. Management should include all of the following EXCEPT?

Barium enema. **Explanation:** The clinical presentation of a 9-month-old with perianal bleeding (likely "red currant jelly" stools), vomiting, and a palpable right lumbar mass is classic for **Intussusception**. However, the presence of **masked liver dullness** and a **shock-like condition** indicates a critical complication: **bowel perforation and peritonitis.** **Why Barium Enema is the Correct Answer (Contraindicated):** In stable cases of intussusception, a barium or air enema is the gold standard for both diagnosis and non-surgical reduction. However, if there are signs of **peritonitis, perforation (pneumoperitoneum causing masked liver dullness), or profound shock**, any form of hydrostatic or pneumatic reduction is **strictly contraindicated**. Performing an enema in this state increases intra-luminal pressure, which can worsen the perforation and lead to tension pneumoperitoneum or fecal contamination of the peritoneal cavity. **Why the other options are incorrect (Necessary Management):** * **Intravenous fluids:** Essential for resuscitation to manage the shock-like state and correct dehydration/electrolyte imbalances. * **Oxygen administration:** Necessary to ensure adequate tissue perfusion in a patient in shock. * **Nasogastric tube insertion:** Crucial for gastric decompression to prevent aspiration and reduce abdominal distension prior to surgery. **NEET-PG High-Yield Pearls:** * **Triad of Intussusception:** Paroxysmal pain, sausage-shaped mass (usually right upper quadrant/lumbar), and red currant jelly stools. * **Dance’s Sign:** An "empty" right iliac fossa due to the migration of the cecum. * **Target/Donut Sign:** The classic appearance on ultrasonography (the investigation of choice). * **Management Rule:** If the patient is stable, attempt non-surgical reduction (Air/Hydrostatic). If the patient has peritonitis or shock, proceed directly to **emergency laparotomy**.

Q: A 4-month-old infant has undergone surgical treatment for meningomyeloencephalocele. An operation was carried out in the posterior cranial fossa to partially replace cerebellar contents to an intracranial position. In investigations for progressive hydrocephalus, it is noted that there is herniation of the cerebellar tonsils through the foramen magnum, and a diagnosis of Arnold-Chiari syndrome is established. This syndrome may also include which of the following?

Abnormal elongation of the medulla and lower cranial nerves. ### Explanation **Arnold-Chiari Malformation (Type II)** is a congenital anomaly characterized by the downward displacement of the cerebellum and brainstem through the foramen magnum. It is almost always associated with a **myelomeningocele**. #### Why Option C is Correct: In Chiari Type II malformation, the posterior fossa is abnormally small. This leads to the **herniation of the cerebellar tonsils, vermis, and the medulla oblongata** through the foramen magnum into the spinal canal. Consequently, the **medulla and lower cranial nerves (IX, X, XI, XII) become abnormally elongated** and stretched as they travel upward to exit their respective foramina. This stretching can lead to symptoms like stridor, swallowing difficulties, and apnea in infants. #### Why Other Options are Incorrect: * **Options A & B (Fusion of lobes):** These are features of **Holoprosencephaly**, a defect in the primary prosencephalic cleavage, not a posterior fossa defect. * **Option D (Absence of pituitary):** This is associated with **Septo-optic dysplasia** or panhypopituitarism, which is unrelated to the structural hindbrain herniation seen in Chiari malformations. #### NEET-PG High-Yield Pearls: * **Chiari Type I:** Downward displacement of cerebellar tonsils only (>5mm); often asymptomatic until adulthood; associated with **syringomyelia**. * **Chiari Type II:** Displacement of tonsils, vermis, and medulla; associated with **lumbar myelomeningocele** and **hydrocephalus** (due to aqueductal stenosis). * **Radiological Sign:** "Beaked midbrain" (tectal plate deformity) and "Banana sign" (curved cerebellum) on prenatal ultrasound. * **Management:** Ventriculoperitoneal (VP) shunting for hydrocephalus and posterior fossa decompression if symptomatic.

Question 1

What is not part of the curative therapy for medulloblastoma in a 1.5-year-old child?

Accepted Answer

Open surgery with leaving tumor margins

Answer

Chemotherapy

Answer

Total craniospinal irradiation

Answer

Ventricular-peritoneal shunt for obstructive hydrocephalus

Question 2

Currarino triad includes:

Accepted Answer

Anorectal malformations, sacrococcygeal osseous defect, and presacral mass

Answer

Pre-sacral meningocele, sacral defect, and tethered cord

Answer

Ectopia vesicae, anorectal malformation, and sacrococcygeal osseous defect

Answer

Tethered cord, anorectal malformations, and ectopia vesicae

Question 3

What is the initial surgical treatment for Tetralogy of Fallot (TOF)?

Accepted Answer

Modified Blalock-Taussig shunt

Answer

Fontan procedure

Answer

Glenn shunt

Answer

Rastelli operation

Question 4

Which electrolyte should be replenished in hypertrophic pyloric stenosis?

Accepted Answer

Chloride

Answer

Sodium

Answer

Hydrogen

Answer

Bicarbonate

Question 5

What is the incidence of undescended testis in term infants?

Accepted Answer

<5%

Answer

<1%

Answer

8%

Answer

10%

Question 6

Cystic hygroma may be associated with which of the following?

Accepted Answer

All of the above

Answer

Turner's syndrome

Answer

Klinefelter's syndrome

Answer

Down's syndrome

Question 7

Sacrococcygeal teratoma is associated with which of the following?

Accepted Answer

Meningocele

Answer

Cardiac defects

Answer

Hepatic cysts

Answer

Cystic adenoid malformation

Question 8

What is true about Bochdalek hernia?

Accepted Answer

It is a differential diagnosis for pleuropericardial cyst.

Answer

It is typically seen on the right side.

Answer

It is located in an anterior position.

Answer

All of the above.

Question 9

A 9-month-old girl presents with perianal bleeding, vomiting, a right lumbar mass, and masked liver dullness. She is in a shock-like condition. Management should include all of the following EXCEPT?

Accepted Answer

Barium enema

Answer

Intravenous fluids

Answer

Oxygen administration

Answer

Nasogastric tube insertion

Question 10

A 4-month-old infant has undergone surgical treatment for meningomyeloencephalocele. An operation was carried out in the posterior cranial fossa to partially replace cerebellar contents to an intracranial position. In investigations for progressive hydrocephalus, it is noted that there is herniation of the cerebellar tonsils through the foramen magnum, and a diagnosis of Arnold-Chiari syndrome is established. This syndrome may also include which of the following?

Accepted Answer

Abnormal elongation of the medulla and lower cranial nerves

Answer

Fusion of the frontal lobes

Answer

Fusion of the temporal, parietal, and occipital lobes

Answer

Partial or complete absence of the pituitary gland

Pediatric Surgery — MCQs

Pediatric Surgery — MCQs

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