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.
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Which statement on Caudal Duplication Syndrome (CDS) is incorrect?
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CDS represents a rare entity of congenital anomalies that involve the gastrointestinal and urogenital tracts and neural tube
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CDS describes a spectrum of structures derived from the embryonic cloaca and notochord which are duplicated to various degrees
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The clinical presentation of each patient varies considerably
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Colonic duplication is a common malformation that usually presents in adolescence
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When the duplicated colons share a common wall, a septotomy is a practical option
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Which description of Microgastria is incorrect?
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Microgastria is almost always associated with other congenital anomalies
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Total Esophageal Gastric Dissociation (TEGD) may be surgical option
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The Hunt-Lawrence jejunal pouch to augment the stomach usually achieves age-appropriate catch up of growth without additional feeding tubes
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Patients with microgastria have normal swallowing abilities
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Neurologically normal patients who underwent TEGD with microgastria may tolerate full oral feeds without symptoms of dumping
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Which statement on Congenital Lung Lesions is correct?
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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
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Malignancy has been reported to be associated with cystic lesions, in particular with type I congenital cystic adenomatoid malformation in 10% of cases
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Hypercapnia and acidosis during thoracoscopy in newborns frequently leads to neurologic impairment
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The feeding vessel of pulmonary sequestration (PS) often rises from a pulmonary artery
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If the feeding vessel of PS not properly closed it usually results in only minor postoperative bleeding
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Which statement on Persistent Urogenital Sinus (UGS) is incorrect?
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The anterior sagittal approach usually requires splitting of the rectum
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UGS may be associated with external genitalia virilization (congenital adrenal hyperplasia [CAH])
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The length of the common channel between the urethra and vagina has an impact on the choice of surgical approach
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Simple flap vaginoplasties are usually sufficient to treat low persistent urogenital sinus (UGS)
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Most patients with UGS are identified at birth
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Which answer on Neonatal Gardner Fibroma (GF) and Familial Adenomatous Polyposis (FAP) is correct?
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The diagnosis of GF in the neonatal period requires the exclusion of FAP in the child but not the parents
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The growth behavior of GF can be well predicted, also the onset of intestinal polyps
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Gardner syndrome is defined as the triad of soft tissue lesions, osteomas, and dental abnormalities in FAP patients
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GF are not regarded as a precursor lesion of desmoid tumors
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The most common sites are the head and extremities, whereas a location at the back, paraspinal region, and chest does rarely occur
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All descriptions of Infantile Myofibromatosis (IM) are correct - except
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IM is a proliferative disorder of mesenchymal origin
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Tumors are characterized by positive staining for smooth muscle actin and muscle-specific actin
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The clinical course is less aggressive than in adult cases
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Radiotherapy is used in noncomplete resection, nonresectable tumors, and reoccurrences
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Postoperative surveillance should avoid radiation exposure; thus, MRI is recommended as a more favorable imaging modality
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Which statement on the reconstruction of large chest wall defects is incorrect?
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The morbidity and mortality of chest wall resections have been reported to be as high as 30 and near 50%, respectively
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Posterior deficiency usually needs more than only soft tissue coverage as is has a greater impact on respiratory function than anterior chest wall defects
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Synthetic meshes are easy to fix to the ribs and can be an excellent choice to cover small defects
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The idea of using “neoribs” made from metal dates back to 1909
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Custom-made titanium implants offer personalized reconstruction with excellent function and cosmetic results
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Which statement on Ovarian Teratoma is incorrect?
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Familial ovarian teratoma is rare
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Ultrasound is a suitable tool to distinguish mature from immature ovarian teratoma
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Both CT and MRI have an excellent sensitivity and specificity for detecting mature ovarian teratoma
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Fat in the cystic lumen is the most specific finding in mature ovarian teratoma
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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%
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Pediatric Necrotizing Fasciitis - Which statement is incorrect?
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Mortality is found to be around 15%
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Delay of surgical intervention by more than 24 hours doubles mortality
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At presentation these lesions are typically painless
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For the pediatric patient cohort Type 2 infections (caused by a single organism infection) seem to be more frequent
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Children should receive broad-spectrum IV antibiotics to cover streptococci, staphylococci, gram negative rods, and anaerobes.
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Which statement on urethral duplication is incorrect?
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Urethral duplication is a rare, congenital lower urinary tract anomaly
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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
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In the majority of patients with “Y-type” duplication, the dorsal urethra is the more functional, while the ventral urethra is less developed
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The use of a voiding cystourethrogram is adequate to trace the course and caliber of the two channels
