Clinical Quiz

The “EJPS Reports Clinical Quiz” is a new regular feature in which you can test your medical knowledge on the topics covered in this issue of the journal. Each quiz is a multi-choice challenge which will test your understanding and recall of the cases and resolutions described in each report.

1. Which statement on Caudal Duplication Syndrome (CDS) is incorrect?

   • CDS represents a rare entity of congenital anomalies that involve the gastrointestinal and urogenital tracts and neural tube

   • CDS describes a spectrum of structures derived from the embryonic cloaca and notochord which are duplicated to various degrees

   • The clinical presentation of each patient varies considerably

   • Colonic duplication is a common malformation that usually presents in adolescence

   • When the duplicated colons share a common wall, a septotomy is a practical option

2. Which description of Microgastria is incorrect?

   • Microgastria is almost always associated with other congenital anomalies

   • Total Esophageal Gastric Dissociation (TEGD) may be surgical option

   • The Hunt-Lawrence jejunal pouch to augment the stomach usually achieves age-appropriate catch up of growth without additional feeding tubes

   • Patients with microgastria have normal swallowing abilities

   • Neurologically normal patients who underwent TEGD with microgastria may tolerate full oral feeds without symptoms of dumping

3. Which statement on Congenital Lung Lesions is correct?

   • The coincidence of pulmonary sequestration (PS) in addition to a congenital diaphragmatic hernia (CDH) may improve the prognosis of patients as PS may act as an anatomical barrier to the rise of the abdominal viscera

   • Malignancy has been reported to be associated with cystic lesions, in particular with type I congenital cystic adenomatoid malformation in 10% of cases

   • Hypercapnia and acidosis during thoracoscopy in newborns frequently leads to neurologic impairment

   • The feeding vessel of pulmonary sequestration (PS) often rises from a pulmonary artery

   • If the feeding vessel of PS not properly closed it usually results in only minor postoperative bleeding

4. Which statement on Persistent Urogenital Sinus (UGS) is incorrect?

   • The anterior sagittal approach usually requires splitting of the rectum

   • UGS may be associated with external genitalia virilization (congenital adrenal hyperplasia [CAH])

   • The length of the common channel between the urethra and vagina has an impact on the choice of surgical approach

   • Simple flap vaginoplasties are usually sufficient to treat low persistent urogenital sinus (UGS)

   • Most patients with UGS are identified at birth

5. Which answer on Neonatal Gardner Fibroma (GF) and Familial Adenomatous Polyposis (FAP) is correct?

   • The diagnosis of GF in the neonatal period requires the exclusion of FAP in the child but not the parents

   • The growth behavior of GF can be well predicted, also the onset of intestinal polyps

   • Gardner syndrome is defined as the triad of soft tissue lesions, osteomas, and dental abnormalities in FAP patients

   • GF are not regarded as a precursor lesion of desmoid tumors

   • The most common sites are the head and extremities, whereas a location at the back, paraspinal region, and chest does rarely occur

6. All descriptions of Infantile Myofibromatosis (IM) are correct - except

   • IM is a proliferative disorder of mesenchymal origin

   • Tumors are characterized by positive staining for smooth muscle actin and muscle-specific actin

   • The clinical course is less aggressive than in adult cases

   • Radiotherapy is used in noncomplete resection, nonresectable tumors, and reoccurrences

   • Postoperative surveillance should avoid radiation exposure; thus, MRI is recommended as a more favorable imaging modality

7. Which statement on the reconstruction of large chest wall defects is incorrect?

   • The morbidity and mortality of chest wall resections have been reported to be as high as 30 and near 50%, respectively

   • Posterior deficiency usually needs more than only soft tissue coverage as is has a greater impact on respiratory function than anterior chest wall defects

   • Synthetic meshes are easy to fix to the ribs and can be an excellent choice to cover small defects

   • The idea of using “neoribs” made from metal dates back to 1909

   • Custom-made titanium implants offer personalized reconstruction with excellent function and cosmetic results

8. Which statement on Ovarian Teratoma is incorrect?

   • Familial ovarian teratoma is rare

   • Ultrasound is a suitable tool to distinguish mature from immature ovarian teratoma

   • Both CT and MRI have an excellent sensitivity and specificity for detecting mature ovarian teratoma

   • Fat in the cystic lumen is the most specific finding in mature ovarian teratoma

   • In adult patients with nonfamilial teratomas, bilaterality is reported in 10 to 25% cases whereas in familial ones the reported risk is up to 46%

9. Pediatric Necrotizing Fasciitis - Which statement is incorrect?

   • Mortality is found to be around 15%

   • Delay of surgical intervention by more than 24 hours doubles mortality

   • At presentation these lesions are typically painless

   • For the pediatric patient cohort Type 2 infections (caused by a single organism infection) seem to be more frequent

   • Children should receive broad-spectrum IV antibiotics to cover streptococci, staphylococci, gram negative rods, and anaerobes.

10. Which statement on urethral duplication is incorrect?

    • Urethral duplication is a rare, congenital lower urinary tract anomaly

    • Of the cases of urethral duplication reported in the literature, type I lesions (classification system proposed by Effman, Lebowitz, and Colodny ) are thought to be the most common and generally asymptomatic

    • In the majority of patients with “Y-type” duplication, the dorsal urethra is the more functional, while the ventral urethra is less developed

    • The use of a voiding cystourethrogram is adequate to trace the course and caliber of the two channels