Pediatric Surgery Practice Questions

Q: The image shows:

Bladder exstrophy. ***Bladder exstrophy*** - The image clearly displays an **exposed bladder mucosa** projecting from the lower abdominal wall, which is characteristic of bladder exstrophy. - This congenital anomaly results from a **failure of fusion of the anterior abdominal wall** and bladder during embryonic development. *Urachal anomalies* - Urachal anomalies involve persistent remnants of the **urachus**, connecting the bladder to the umbilicus, leading to cysts, sinuses, or fistulas. - They typically appear as lesions at or near the **umbilicus** and do not involve the direct exposure of the bladder. *Cloacal exstrophy* - Cloacal exstrophy is a **more severe** form of the exstrophy-epispadias complex, involving exposure of both the bladder and bowel through the abdominal wall. - It is distinguished from bladder exstrophy by the presence of an **omphalocele**, two hemibladders separated by exposed bowel, and associated spinal and hindgut anomalies. *Congenital adrenal hyperplasia* - This is an **endocrine disorder** affecting hormone production by the adrenal glands, leading to ambiguous genitalia in some cases. - It does not involve a **visible external defect of the bladder** or abdominal wall.

Q: The picture shows a newborn with esophageal atresia and distal tracheoesophageal fistula presenting with excessive salivation. Which of the following statements is NOT true regarding the condition shown?

Always associated with absence of gas shadows in abdomen. ***Always associated with absence of gas shadows in abdomen*** - In **esophageal atresia with distal tracheoesophageal fistula (Type C)**, air passes from the trachea through the distal fistula into the stomach and intestines, resulting in **gas shadows** in the abdomen. Therefore, absence of gas is **not always** the case. - A **gasless abdomen** is typically seen in **pure esophageal atresia** (without a distal TEF), where no air can reach the gastrointestinal tract. *Associated with polyhydramnios* - **Polyhydramnios** is a common association due to the inability of the fetus to **swallow amniotic fluid** because of the esophageal obstruction. - This leads to an excessive accumulation of amniotic fluid in the uterus. *Associated with VACTERL anomalies* - **Tracheoesophageal fistula** is a key component of the **VACTERL association**, which includes **Vertebral anomalies**, **Anal atresia**, **Cardiac defects**, **Tracheoesophageal fistula**, **Renal anomalies**, and **Limb abnormalities**. - These are often found together in affected newborns. *Surgical ligation of TEF and primary end-to-end anastomosis of esophagus is performed as definitive treatment* - The definitive treatment for **tracheoesophageal fistula (TEF)** with **esophageal atresia** is surgical repair. - This involves **ligation of the fistula** and a **primary end-to-end anastomosis** of the esophageal segments to restore continuity.

Q: What is shown in the image below?

Pectus excavatum. ***Pectus excavatum*** - The image clearly depicts **pectus excavatum**, which is the direct and accurate identification of the congenital chest wall deformity shown. - This represents a **depression of the sternum** and costal cartilages, creating the characteristic caved-in appearance of the anterior chest wall. *Pectus carinatum* - Pectus carinatum is a congenital chest wall deformity characterized by a **protrusion of the sternum** and costal cartilages, creating a "pigeon chest" appearance — the opposite of the caved-in deformity seen in the image. *Barrel chest* - Barrel chest refers to an **increased anterior-posterior diameter** of the chest, typically associated with chronic obstructive pulmonary disease (COPD) or emphysema, and does not show the focal sternal depression visible in the image. *Scoliosis* - Scoliosis is a **lateral curvature of the spine** and would present with asymmetry of the back and shoulders rather than the anterior sternal depression characteristic of pectus excavatum.

Q: A newborn male infant presents with the findings shown in the image. The clinical diagnosis is?

Bladder exstrophy. ***Bladder Exstrophy*** - The image depicts **bladder exstrophy** with associated **epispadias**, characterized by the exposed **bladder mucosa (exstrophy)** and a penile shaft that is short, dorsally curved, and has the urethral opening on the dorsal surface. - This congenital anomaly results from a failure of the abdominal wall and bladder to close properly during fetal development, often presenting with a **widened pubic symphysis**. *Epispadias* - **Epispadias** is characterized by the urethral opening on the **dorsal** surface of the penis and is a component of the exstrophy-epispadias complex, but the primary finding in the image is bladder exstrophy. - Isolated epispadias does not present with exposed bladder mucosa as seen in the image. *Hypospadias* - **Hypospadias** is a condition where the **urethral opening** is located on the **ventral** (underside) surface of the penis, not the dorsal surface as seen in the image. - While it can involve chordee (penile curvature), it typically does not present with bladder exstrophy. *Micro-penis* - **Micropenis** refers to a penis that is **abnormally small** in length, but otherwise structurally normal. - It describes the size of the penis, not an anomalous opening or exposed internal organs, and is not consistent with the image. *Mauriac syndrome* - **Mauriac syndrome** is a rare complication of poorly controlled **Type 1 diabetes** in children, characterized by growth retardation, delayed puberty, hepatomegaly, and Cushingoid features. - It is an **endocrine disorder** and has no direct relevance to the anatomical abnormality shown in the image.

Q: A 2-month-old girl was brought with swelling just above the gluteal area with progressive increase in size. Imaging reveals a midline mass attached to the coccyx with both external and internal presacral components. Which is the most probable diagnosis?

Sacrococcygeal teratoma. ***Sacrococcygeal teratoma*** - Sacrococcygeal teratomas are the **most common germ cell tumors in neonates and infants**, often presenting as a mass in the sacral region, as seen in the image. - They arise from pluripotent cells in the **Hensen's node** (primitive streak remnant) and can contain tissues derived from all three germ layers. *Rhabdomyosarcoma* - Rhabdomyosarcoma is a **malignant tumor of skeletal muscle origin** that can occur in various locations, including the gluteal area, but is less common in infants than SCT. - While it can present as a rapidly growing mass, a sacrococcygeal location in an infant points more strongly to teratoma. *Atypical Rhabdoid tumor* - Atypical rhabdoid tumor is a **highly malignant tumor** typically found in the brain and kidney, though it can occur in soft tissues. - It is rare and not typically the primary diagnosis for a large external gluteal mass in a 2-month-old. *Fibrosarcoma* - Fibrosarcoma is a **malignant tumor of fibroblasts**; infantile (congenital) fibrosarcoma characteristically presents in infancy and can appear as a rapidly enlarging soft tissue mass. - However, the midline coccygeal attachment with internal presacral extension seen in this case is characteristic of sacrococcygeal teratoma rather than fibrosarcoma, making teratoma the more likely diagnosis in this age group and location.

Q: The image shows presence of:

Gastroschisis. ***Gastroschisis*** - The image shows **exposed abdominal organs** (intestines) protruding through a defect in the abdominal wall, typically to the **right of the umbilical cord**. - In gastroschisis, there is **no covering membrane** over the protruding organs, which is characteristic of the condition seen in the image. *Omphalocele* - An omphalocele involves the **protrusion of abdominal organs** through the umbilical cord, but they are contained within a **peritoneal-amniotic sac**. - The image clearly shows **uncovered intestines**, ruling out an omphalocele. *Necrotizing enterocolitis* - This condition is an **inflammatory bowel disease** affecting premature infants, characterized by bowel necrosis, not by external protrusion of organs. - Clinical signs would include abdominal distension, feeding intolerance, and bloody stools, which are not directly depicted or implied here. *Malformation of vitello-intestinal duct* - Malformations of the vitello-intestinal duct (e.g., Meckel's diverticulum or persistent vitelline duct) involve remnants of the embryonic yolk sac connection to the intestine. - These malformations do not typically present as a large external defect with exposed bowel loops as seen in the image.

Q: All investigations are useful in work up of this condition except:

Barium enema. ***Barium enema*** - **Barium enema** is not a standard or useful investigation in the initial workup of **neonatal anorectal malformations**. In the neonatal period, the distal bowel cannot be accessed from below due to the malformation itself, making a routine barium enema technically unfeasible and uninformative for surgical planning in this condition. - Note: An appropriately performed **distal colostogram with water-soluble contrast** (performed after colostomy) is a distinct procedure from a routine neonatal barium enema and is used to delineate fistulous anatomy prior to definitive repair; it should not be confused with a routine barium enema. *Urine for meconium particles* - **Urine for meconium particles** is actually a valuable and standard investigation in **anorectal malformations** to detect **rectourinary fistulas**. - The presence of meconium in urine indicates a **communication between the rectum and urinary tract**, which is crucial information for surgical planning and management. *Prone cross-table lateral radiograph* - A **prone cross-table lateral radiograph** taken after sufficient time (at least 18–24 hours) for distal bowel gas accumulation allows determination of the **level of rectal pouch termination** relative to the **pubococcygeal line**. - This is the contemporary preferred method for distinguishing between high and low anorectal malformations, as it avoids the positional difficulties and inaccuracies associated with the older invertogram technique. *Ultrasound* - **Perineal ultrasound** can assess the **perineal body thickness**, presence of a **rectal pouch**, and help visualize **fistulous connections**. - It is particularly useful in **real-time assessment** of the anatomy and can identify associated **genitourinary anomalies** commonly seen with anorectal malformations.

Q: Which tumor is shown here?

Sacrococcygeal teratoma. **Sacrococcygeal teratoma** - The image shows a **large, complex mass** originating from the **sacrococcygeal region** of an infant, a classic presentation for a sacrococcygeal teratoma. - These tumors are typically **congenital germ cell tumors** and can be external, internal, or mixed, often presenting as a large visible mass at birth. *Meningocele* - A meningocele is a **protrusion of the meninges** through a defect in the spine, forming a sac filled with cerebrospinal fluid, but not containing neural tissue. - While it occurs in a similar anatomical region, a meningocele would appear as a **fluid-filled sac without the solid and sometimes hemorrhagic components** seen in the image. *Chloroma* - A chloroma (also known as a granulocytic sarcoma) is an **extramedullary tumor composed of myeloid blast cells**, typically associated with acute myeloid leukemia. - It usually presents as a discrete mass in various body sites, but its appearance is **rarely a large, malformed growth from the sacrococcygeal region** in an infant, and it usually has a greenish hue due to myeloperoxidase. *Infantile fibrosarcoma* - Infantile fibrosarcoma is a **malignant soft tissue tumor** that can occur in infants, often presenting as a rapidly growing mass. - While it can occur in various locations, the **distinctive appearance of a complex, mixed-tissue mass** in the sacrococcygeal area is more characteristic of a teratoma than a purely mesenchymal tumor like fibrosarcoma.

Q: A neonate presents with a doughy soft lump in the neck causing distortion of face and audible grunt, as shown in the image. Diagnosis is:

Cystic hygroma. ***Cystic hygroma*** - A **cystic hygroma** is a **congenital lymphatic malformation** typically presenting as a soft, doughy mass in the neck, which can be quite large and cause facial distortion and respiratory distress (indicated by an audible grunt). - Its presence at birth, large size, soft consistency, and impact on adjacent structures are classic features of a cystic hygroma. *Brachial cyst* - A **branchial cleft cyst** usually appears as a **well-defined, smooth lump** on one side of the neck, typically along the anterior border of the sternocleidomastoid muscle. - While congenital, these cysts are usually **smaller** and less likely to cause widespread facial distortion or immediate respiratory compromise in a neonate compared to a large cystic hygroma. *Sternocleidomastoid tumor* - A **sternocleidomastoid tumor** (fibromatosis colli) is a firm, non-tender mass within the sternocleidomastoid muscle, usually appearing a few weeks after birth. - It typically causes **torticollis** (head tilt) and is **firm to rubbery**, not doughy, and rarely causes facial distortion or respiratory grunting. *Birth injury* - **Birth injuries** to the neck might include hematomas or nerve damage, but they would typically present with **bruising**, swelling, or neurological deficits, rather than a soft, persistent, doughy lump causing facial distortion and grunting. - A large hematoma might be firm initially and would typically resolve over time, not present as a soft, fluid-filled mass.

Question 1

The image shows:

Accepted Answer

Bladder exstrophy

Answer

Congenital adrenal hyperplasia

Answer

Urachal anomalies

Answer

Cloacal exstrophy

Question 2

The picture shows a newborn with esophageal atresia and distal tracheoesophageal fistula presenting with excessive salivation. Which of the following statements is NOT true regarding the condition shown?

Accepted Answer

Always associated with absence of gas shadows in abdomen

Answer

Associated with VACTERL anomalies

Answer

Associated with polyhydramnios

Answer

Surgical ligation of TEF and primary end-to-end anastomosis of esophagus is performed as definitive treatment

Question 3

All are true about the condition shown in the figure except:

Accepted Answer

Surgical repair should be performed immediately at birth as an emergency procedure

Answer

Severe pulmonary hypoplasia and pulmonary hypertension are major determinants of survival

Answer

Scaphoid abdomen occurs due to herniation of contents into chest cavity

Answer

Posterolateral left sided defect is the most common site

Question 4

What is shown in the image below?

Accepted Answer

Pectus excavatum

Answer

Scoliosis

Answer

Pectus carinatum

Answer

Barrel chest

Question 5

A newborn male infant presents with the findings shown in the image. The clinical diagnosis is?

Accepted Answer

Bladder exstrophy

Answer

Mauriac syndrome

Answer

Epispadias

Answer

Hypospadias

Answer

Micro-penis

Question 6

A 2-month-old girl was brought with swelling just above the gluteal area with progressive increase in size. Imaging reveals a midline mass attached to the coccyx with both external and internal presacral components. Which is the most probable diagnosis?

Accepted Answer

Sacrococcygeal teratoma

Answer

Fibrosarcoma

Answer

Rhabdomyosarcoma

Answer

Atypical Rhabdoid tumor

Question 7

The image shows presence of:

Accepted Answer

Gastroschisis

Answer

Omphalocele

Answer

Necrotizing enterocolitis

Answer

Malformation of vitello-intestinal duct

Question 8

All investigations are useful in work up of this condition except:

Accepted Answer

Barium enema

Answer

Urine for meconium particles

Answer

Prone cross-table lateral radiograph

Answer

Ultrasound

Question 9

Which tumor is shown here?

Accepted Answer

Sacrococcygeal teratoma

Answer

Meningocele

Answer

Chloroma

Answer

Infantile fibrosarcoma

Question 10

A neonate presents with a doughy soft lump in the neck causing distortion of face and audible grunt, as shown in the image. Diagnosis is:

Accepted Answer

Cystic hygroma

Answer

Brachial cyst

Answer

Sternocleidomastoid tumor

Answer

Birth injury

Pediatric Surgery — MCQs

Pediatric Surgery — MCQs

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