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Назва наукового напрямку (модуля): Семестр: 11 Malformations in children-tests questions Опис: 6c-med. Final module control Перелік питань: 1. A. B. C. D. * E. 2. A. B. C. * D. E. 3. A. B. * C. D. E. 4. A. B. C. * D. E. 5. A. B. C. * D. E. 6. A. B. C. D. * E. 7. A. B. * C. D. A pediatrician has diagnosed a newborn baby of having right-sided cryptorchidism (undescended testis). The testis may have been trapped in any site EXCEPT: At the deep inguinal ring Just outside the superficial inguinal ring Pelvic brim Perineum Somewhere in the inguinal canal Congenital absence of one of the testes is known as: cryptorchidism anorchia monorchism ectopia dystopia Which of the following is not a risk factor for development of an inguinal hernia? Presence of a ventriculoperitoneal shunt Congenital heart disease Prematurity Cystic fibrosis Family history of inguinal hernias Which of the following hernias follows the path of the spermatic cord within the cremaster muscle? Femoral Direct inguinal Indirect inguinal Spigelian Interparietal The earliest way to diagnose an anterior abdominal wall defect is: by physical exam by history by fetal ultrasound by fetal CT scan None of the above Gastroschisis: is usually associated with other anomalies is usually associated with chromosomal disorders is located on the left of the umbilical cord repair is followed by prolonged ileus none of the above Gastroschisis is associated with an increased risk of: hepatomegaly intestinal atresia microcephaly cardiac anomalies E. 8. A. B. C. D. E. * 9. A. B. C. D. * E. 10. A. B. C. D. E. * 11. A. B. * C. D. E. 12. A. B. C. * D. E. 13. A. * B. C. D. E. 14. A. B. C. * D. E. 15. A. all of the above The test with the highest diagnostic yield for detecting a colovesical fistula is: Barium enema. Colonoscopy. Computed tomography (CT). Cystography. Cystoscopy. Duodenal atresia: is caused by intrauterine mesenteric vascular accident commonly exhibits normal muscular wall with a mucosal web is seldom associated with normal passage of meconium at birth is rarely associated with other congenital anomalies is often associated with nonbileus vomiting A positive Farber’s test is indicative of which pathological state? Ladd’s syndrome Ileal stenosis Duodenal stenosis Pancreatic cystic fibrosis Duodenal atresia How is CF inherited? Autosomal Dominant Autosomal Recessive Sex-linked Recessive Sex-linked Dominant None of the above The treatment of choice for neonates with uncomplicated meconium ileus is: Observation. Emergency laparotomy, bowel resection, and Bishop-Koop enterostomy. Intravenous hydration and a gastrograffin enema. Emergency laparotomy, bowel resection, and anastomosis. Sweat chloride test and pancreatic enzyme therapy. The conservative method of meconium ileus management is: Gastrografin enema Antibacterial therapy Spasmolytic drugs Intravenous infusion Ultrasound therapy The carrier rate for the CF gene in the white population is: 1 in 10 1 in 15 1 in 25 1 in 50 1 in 5 At what age is surgical orchiopexy recommended for a child with a unilateral undescended testis? Promptly upon discovery, regardless of age B. * 1 year C. D. E. 16. A. B. C. * D. E. 17. A. B. * C. 5 to 6 years Any time prior to puberty 6 to 7 years Cryptorchidism is most frequently found: on the left side bilaterally on the right side retroperitoneally in the abdomen All are possible complications of an undescended testis except: malignization acute scrotum torsion and trauma hypoplasia sterility Which of the following is not part of the differential diagnosis of an inguinal-scrotal swelling in children? Varicocele Undescended or retracted testis Volvulus Testicular torsion Testicular cancer Upon further imaging, what associated finding would be expected? posterior urethral valves on a voiding cystourethrogram (VCUG) hydrocephalus on head ultrasound cardiomegaly on chest x-ray (CXR) bilateral adrenal enlargement on abdominal ultrasound tracheoesophageal fistula on an upper gastrointestinal (UGI) series A 17 year old patient presents for the first time with a small palpable testis in the inguinal region. Orchidectomy is performed. The histological appearances of these testis are most likely to show: Germ cell hyperplasia 'Sertoli cell only' appearances Normal appearances Well-differentiated seminoma Ley dig cell hyperplasia A three year old boy is referred with a unilateral impalpable testis. The most reliable means of confirming the presence or absence of the testis is: Abdominal ultrasonography MRI CT scan Retrograde venography Laparoscopy Which of the following statements is false? Each testis descends through the inguinal canal into the scrotum within the processus vaginalis. D. E. 18. A. B. C. * D. E. 19. A. * B. C. D. E. 20. A. B. * C. D. E. 21. A. B. C. D. E. * 22. A. * B. C. D. E. 23. A. B. C. D. * E. 24. A. B. * C. D. E. 25. A. B. C. D. E. * 26. A. B. C. D. E. * 27. A. B. C. D. * E. 28. A. B. C. D. E. * A hydrocele can result from incomplete fusion of the processus vaginalis. A scrotal hydrocele, or simple hydrocele, is a type of non-communicating hydrocele. A communicating hydrocele can develop into an inguinal-scrotal hernia. Some use the terms interchangeably. A hernia sac can contain intestine, omentum, testis/ovary or fallopian tube. An undescended testis that remains in the pelvic cavity or somewhere in the inguinal canal is known as which of the following? a hydrocele hypospadias epispadias cryptorchidism hermaphroditism A 17 year old patient presents for the first time with a small palpable testis in the inguinal region. Orchidectomy is performed. The histological appearances of these testis are most likely to show: Germ cell hyperplasia 'Sertoli cell only" appearances Normal appearances Well-differentiated seminoma Leydig cell hyperplasia A three year old boy is referred with a unilateral impalpable testis. The most reliable means of confirming the presence or absence of the testis is: Abdominal ultrasonography MRI CT scan Retrograde venography Laparoscopy Which structure passes through the deep inguinal ring? Iliohypogastric nerve Ilioinguinal nerve Inferior epigastric artery Medial umbilical ligament Round ligament of the uterus To maximize fertility potential, orchidopexy for cryptorchidism should be done before: Age 15 years. Age 12 years. Marriage. Age 2 years. Age 5 years If one were to make an incision parallel to and 2 inches above the inguinal ligament, one would find the inferior epigastric vessels between which layers of the abdominal wall? Camper's and Scarpa's fascias External abdominal oblique and internal abdominal oblique muscles Internal abdominal oblique and transversus abdominis muscles Skin and deep fascia of the abdominal wall Tranversus abdominis muscle and peritoneum 29. A. B. C. * D. E. 30. A. B. C. D. E. * 31. A. B. C. D. E. * 32. A. B. * C. D. E. 33. A. B. C. D. * E. 34. A. B. C. D. E. * 35. A. * B. C. D. A loop of bowel protrudes through the abdominal wall to form a direct inguinal hernia; viewed from the abdominal side, the hernial sac would be found in which region? Deep inguinal ring Lateral inguinal fossa Medial inguinal fossa Superficial inguinal ring Supravesical fossa A patient presents with a hernia that is palpable at the superficial inguinal ring. The hernia was diagnosed as: Obdurator hernia A direct inguinal hernia A femoral hernia An incisional hernia An indirect inguinal hernia In a female with an indirect inguinal hernia, the herniated mass lies along side of which structure as it traverses the inguinal canal? Iliohypogastric nerve Inferior epigastric artery Ovarian artery and vein Pectineal ligament Round ligament of the uterus During your peer presentation of the inguinal region dissection, you would indicate the position of the deep inguinal ring to be Above the anterior superior iliac spine Above the midpoint of the inguinal ligament Above the pubic tubercle In the supravesical fossa Medial to the inferior epigastric artery An elderly patient with a large indirect inguinal hernia came to your clinic complaining of pain in the scrotum. You conclude that the hernial sac is compressing the following nerve Femoral branch of the genitofemoral Femoral Iliohypogastric Ilioinguinal Subcostal What is the best diagnostic test for the assessment of inguinal hernia in children? Ultrasonography Plain radiography CT MRI None of the above The boundaries of the inguinal triangle include all except: Arcuate line Inferior epigastric vessels Inguinal ligament Lateral border of rectus abdominus muscle E. 36. A. * B. C. D. E. 37. A. B. * C. D. E. 38. A. B. * C. D. E. 39. A. B. * C. D. E. 40. A. B. * C. D. E. 41. A. B. * C. D. E. 42. A. B. * C. D. Pubis. The superficial inguinal ring is an opening in which structure? External abdominal oblique aponeurosis Falx inguinalis Internal abdominal oblique muscle Scarpa's fascia Transversalis fascia If a hernia enters into the scrotum, it is most likely a: Direct inguinal hernia Indirect inguinal hernia Femoral hernia Obdurator hernia An incisional hernia Which nerve passes through the superficial inguinal ring and may therefore be endangered during inguinal hernia repair? Femoral branch of the genitofemoral Ilioinguinal Iliohypogastric Obturator Subcostal What is the most likely diagnosis in an infant or young child with a history of intermittent swelling in the groin region? Direct inguinal hernia Indirect inguinal hernia Varicocele Adenopathy None of the above During inguinal herniotomy in a 12 year old the testis was found in the hernia sac. What type of hernia is this? Richter’s hernia Congenital indirect hernia Acquired direct hernia Direct hernia Femoral hernia A hernia containing the vermiform appendix in its sac is known as: Richter’s hernia Amyand’s hernia Littre’s hernia Omentocele Indirect hernia A patent processus vaginalis can lead to all of the following, except: Funiculocele Omphalocele Bubonocele Scrotal hernia E. 43. A. * B. C. D. E. 44. A. B. C. * D. E. 45. A. B. * C. D. E. 46. A. B. C. * D. E. 47. A. B. C. * D. E. 48. A. B. C. D. * E. 49. A. B. C. Hydrocele Which of the following statements is false? Each testis descends through the inguinal canal into the scrotum within the processus vaginalis. A hydrocele can result from incomplete fusion of the processus vaginalis. A scrotal hydrocele, or simple hydrocele, is a type of non-communicating hydrocele. A communicating hydrocele can develop into an inguinal-scrotal hernia. Some use the terms interchangeably. A hernia sac can contain intestine, omentum, testis/ovary or fallopian tube. Which of the following is not part of the differential diagnosis of an inguinal-scrotal swelling in children? Varicocele Undescended or retracted testis Volvulus Testicular torsion Testicular cancer Which of the following is not a risk factor for development of an inguinal hernia? Presence of a ventriculoperitoneal shunt Congenital heart disease Prematurity Cystic fibrosis Family history of inguinal hernias The most common hernia in females is: Femoral hernia. Direct inguinal hernia. Indirect inguinal hernia. Obturator hernia. Umbilical hernia. Which of the following statements regarding unusual hernias is incorrect? An obturator hernia may produce nerve compression diagnosed by a positive Howship-Romberg sign. Grynfeltt's hernia appears through the superior lumbar triangle, whereas Petit's hernia occurs through the inferior lumbar triangle. Sciatic hernias usually present with a painful groin mass below the inguinal ligament. Littre's hernia is defined by a Meckel's diverticulum presenting as the sole component of the hernia sac. Richter's hernia involves the antimesenteric surface of the intestine within the hernia sac and may present with partial intestinal obstruction. Staples may safely be placed during laparoscopic hernia repair in each of the following structures except: Cooper's ligament. Tissues superior to the lateral iliopubic tract. The transversus abdominis aponeurotic arch. Tissues inferior to the lateral iliopubic tract. The iliopubic tract at its insertion onto Cooper's ligament. The following Nyhus classification of hernias is correct except for: Recurrent direct inguinal hernia—Type IVa. Indirect inguinal hernia with a normal internal inguinal ring—Type I. Femoral hernia—Type IIIc. D. E. * 50. A. B. C. D. * E. 51. A. * B. C. D. E. 52. A. B. * C. D. E. 53. A. B. * C. D. E. 54. A. B. * C. D. E. 55. A. B. Direct inguinal hernia—Type IIIa. Indirect inguinal hernia with destruction of the transversalis fascia of Hesselbach's triangle—Type II. Which of the following statements about the causes of inguinal hernia is correct? Excessive hydroxyproline has been demonstrated in the aponeuroses of hernia patients. Obliteration of the processus vaginalis is a contributing factor for the development of an indirect inguinal hernia. Physical activity and athletics have been shown to have a protective effect toward the development of inguinal hernias. Elevated levels of circulating serum elastalytic activity have been demonstrated in patients with direct herniation who smoke. The majority of inguinal hernias are acquired. The following statements about the repair of inguinal hernias are true except: The conjoined tendon is sutured to Cooper's ligament in the Bassini hernia repair. The McVay repair is a suitable option for the repair of femoral hernias. The Shouldice repair involves a multilayer, imbricated repair of the floor of the inguinal canal. The Lichtenstein repair is accomplished by prosthetic mesh repair of the inguinal canal floor in a tension-free manner. The laparoscopic transabdominal preperitoneal (TAPP) and totally extraperitoneal approach (TEPA) repairs are based on the preperitoneal repairs of Cheattle, Henry, Nyhus, and Stoppa. Which of the following congenital abnormalities are correctly defined? Omphalocele represents a defect in the abdominal wall lateral to the umbilical cord. The herniated viscera associated with omphaloceles are usually covered with a membranous sac. In case of gastroschisis primary fascial closure can be achieved in only about 25% of these infants. Omphalocele represents a defect in the abdominal wall lateral to the umbilical cord. The herniated viscera associated with are usually covered with a membranous sac. Which of the following statement(s) is/are true concerning the diagnosis and management of epigastric hernias? A large peritoneal sac containing abdominal viscera is common At the time of surgical repair, a careful search for other defects should be performed Recurrent epigastric hernias after simple closure is uncommon Patients with symptoms of a painful midline abdominal mass frequently will contain incarcerated small bowel Patients with symptoms of a painful midline abdominal mass frequently will contain incarcerated large bowel Which statement is true concerning neurovascular structures in the inguinal region. The inferior epigastric artery and vein run upward in the preperitoneal fat posterior to the transversalis fascia close to the lateral margin of the internal inguinal ring The iliohypogastric and ilioinguinal are motor and sensory nerves in the inguinal region which lie beneath the external oblique aponeurosis The ilioinguinal nerve runs posterior to the spermatic cord in the inguinal canal and at the superficial inguinal ring, branches into the sensory supply to the pubic region and the upper scrotum or labium majoris The genital branch of the genitofemoral nerve is a sensory nerve only to the upper thigh and genital area All of the above The following statement(s) is/are true concerning the epidemiology of inguinal hernias. Inguinal hernias occur with a male-to-female ratio of about 1:1 Femoral and umbilical hernias are more common in women, with a female-to-male ratio of 4:1 C. * D. E. 56. A. B. * C. D. E. 57. A. B. * C. D. E. 58. A. B. C. D. E. * 59. A. B. C. D. * E. 60. A. B. C. D. * E. 61. A. B. * The frequency of inguinal hernias increases with age Almost all umbilical hernias occur in the pediatric age group All of the above The following statement(s) is/are true concerning umbilical hernias in adults. Most umbilical hernias in adults are the result of a congenital defect carried into adulthood A paraumbilical hernia typically occurs in multiparous females The presence of ascites is a contraindication to elective umbilical hernia repair. Incarceration is uncommon with umbilical hernias All of the above The following statement(s) is/are true concerning the anterior abdominal wall musculature. The lateral musculature of the abdominal wall consists of three muscle layers. These are, from external to internal, the external oblique, the transversus abdominis, and the internal oblique muscles The transversalis fascia lies on the deep side of the transversus muscle and extends to form an essentially complete fascial envelope of the abdominal cavity Below the semicircular line, the internal oblique aponeurosis splits into posterior and anterior laminae The rectus abdominis muscles originate on the ribs superiorly and on the pubis inferiorly and are clearly distinct throughout their entire length All of the above The following statement(s) is/are true concerning incarceration of an inguinal hernia. All incarcerated hernias are surgical emergencies and require prompt surgical intervention Attempt at reduction of an incarcerated symptomatic hernia is generally considered safe Vigorous attempts at reduction of an incarcerated hernia may result in reduction en masse with continued entrapment and possible progression to obstruction or strangulation All incarcerated hernias are surgical emergencies and require prompt surgical intervention & Attempt at reduction of an incarcerated symptomatic hernia is generally considered safe Attempt at reduction of an incarcerated symptomatic hernia is generally considered safe & Vigorous attempts at reduction of an incarcerated hernia may result in reduction en masse with continued entrapment and possible progression to obstruction or strangulation The following statement(s) is/are true concerning abdominal incisional hernias. Large incisional hernias are associated with a high recurrence rate when closed primarily A large potential space remains anterior to the abdominal wall closure in most patients indicating a need for postoperative wound drainage Incisional hernias are frequently associated with a tissue deficit either due to chronic retraction and scarring or the result of tissue necrosis from either infection or tension at the initial closure All of the above None of the above Which of the following structures are derived from the external oblique muscle and its aponeurosis? The inguinal or Poupart’s ligament The lacunar ligament The conjoined tendon The inguinal or Poupart’s ligament & The lacunar ligament The lacunar ligament & The conjoined tendon A number of special circumstances exist in the repair of inguinal hernias. The following statement(s) is/are correct. Simultaneous repair of bilateral direct inguinal hernias can be performed with no significant increased risk of recurrence The preperitoneal approach may be appropriate for repair of a multiple recurrent hernia C. D. E. 62. A. * B. C. D. E. 63. A. B. C. * D. E. 64. A. * B. C. D. E. 65. A. B. C. * D. E. 66. A. B. C. * D. E. 67. A. * B. C. D. E. 68. A. B. * A femoral hernia repair can best be accomplished using a Bassini or Shouldice repair Management of an incarcerated inguinal hernia with obstruction is best approached via laparotomy incision All of the above Which of the following structures can be found outside of the spermatic cord during a hernia repair? direct hernia sac indirect hernia sac vas deferens testicular artery ovary Which of the following is not part of the differential diagnosis of an inguinal-scrotal swelling in children? Varicocele Undescended or retracted testis Volvulus Testicular torsion Testicular cancer The most common source of bacteria in wound infection after groin hernia repair is: the patient’s skin the patient’s nasopharynx operating room air surgical instruments operating room staff Howship-Romberg sign is characteristic of: femoral hernia Spigelian hernia obturator hernia lumbar hernia epigastric hernia The most common hernia in women is: femoral hernia obturator hernia inguinal hernia umbilical hernia spigelian hernia Spontaneous closure of which of the following congenital abnormalities of the abdominal wall generally occurs by the age of 4? Umbilical hernia Patent urachus Patent omphalomesenteric duct Omphalocele Gastroschisis Which of the following statements regarding direct inguinal hernias is true? They are the most common inguinal hernias in women They protrude medially to the inferior epigastric vessels C. D. E. 69. A. B. C. D. E. * 70. A. B. * C. D. E. 71. A. B. C. D. * E. 72. A. B. C. D. * E. 73. A. B. C. * D. E. 74. A. B. C. * D. E. 75. A. They should be opened and ligated at the internal ring They commonly protrude into the scrotal sac in men They incarcerate more commonly than indirect hernias Upon delivery of a baby with a small intact omphalocele and an enlarged tongue, which of the following is foremost in the physician’s mind in the management of this infant? Cover the omphalocele membrane with plastic wrap to prevent loss of body warmth and fluid. Insert an orogastric tube to decompress the intestinal tract. Promptly arrange consultation with a pediatric surgeon to repair the omphalocele and assess the baby's airway. Insert an IV to correct fluid and electrolyte losses. Insert an IV to provide glucose. A maternal ultrasound reveals a baby with gastroschisis. What should the physician recommend to the parents regarding the baby's management? Amniocentesis and elective cesarean section once lung maturity is verified Serial ultrasound exams with induction of labor and vaginal delivery if polyhydramnios develops or if the intestine appears increasingly dilated Amniocentesis to determine presence of associated genetic defect Fetal echocardiography to determine presence of associated cardiac defect Serial ultrasound exams with delivery by cesarean section if polyhydramnios develops or if the intestine appears increasingly dilated Which of the following statements is true of infants with gastroschisis? It is not associated with malrotation. There is a high incidence of associated anomalies. There is not prolonged adynamic ileus following repair. It is complicated by intestinal atresia in 10% to 12% of cases. It is associated with chromosomal syndromes. The pentalogy of Cantrell includes all of the following except: Epigastric omphalocele. Sternal cleft. Intracardiac defect. Pericardial cyst. Ectopia cordis. Which of the following statements regarding gastroschisis are true? Primary fascial closure can be achieved in only about 25% of these infants These infants have an incidence of approximately 40% to 50% of associated anomalies Overall survival is approximately 80% to 90% When the diagnosis is known prenatally, planned cesarean section is the safest method of delivery It is associated with chromosomal syndromes. Which clinical feature is not present in cloacal exstrophy? Spinal abnormality Hemibladders adjacent to exposed cecum Gastroschisis Omphalocele None of the above An exomphalos is: A strangulated umbilical hernia B. * C. D. E. 76. A. B. * C. D. E. 77. A. B. C. * D. E. 78. A. B. C. D. * E. 79. A. B. C. * D. E. 80. A. B. * C. D. E. 81. A. B. * C. D. E. 82. A. * B. Synonymous with an omphalocele A strangulated femoral hernia A strangulated inguinal hernia A sliding hernia A patent processus vaginalis can lead to all of the following, except: Funiculocele Omphalocele Bubonocele Scrotal hernia Hydrocele The following are correct regarding omphaloceles except: is usually covered by a translucent membrane is frequently associated with other congenital malformations is lateral to the umbilical stump is within the umbilical ring It is a congenital disease The following are true about gastroschisis: occurs lateral to the umbilical stump can be diagnosed antenatally at birth often have edematous matted intestinal loops all of the above none of the above Treatment of abdominal wall defects includes: immediate surgical repair pushing the intestines back into the abdominal cavity while still in the delivery room provide immediate optimal resuscitation and stabilization first, and then surgery always do primary closure in both lesions conservative treatment only The closure of the distal parts of the umbilical arteries after birth forms which of the following structures? the ligamentum venosum the medial umbilical ligaments the ligamentum teres hepatis the ligamentum arteriosus the urachus Whenever the intestinal midgut loop fails to return from the umbilical cord into the abdominal cavity, the defect is known as which of the following? gastroschisis an omphalocele Meckel’s diverticulum a vitelline fistula a left-sided colon Which of the following statements is TRUE with respect to neonatal abdominal wall defects? The bowel in omphalocele is covered by a sac. Gastroschisis is frequently associated with other anomalies. C. D. E. 83. A. * B. C. D. E. 84. A. B. * C. D. E. 85. A. B. * C. D. E. 86. A. B. C. D. * E. 87. A. B. C. D. E. * 88. A. B. C. * D. A Silastic silo is rarely employed in management of these defects. Mortality is higher in gastroschisis. Operative management of omphalocele usually requires bowel resection. A mother of a newborn complains of her baby's constant belching with undigested milk. Which developmental anomaly is it an evidence of? Esophageal atresia Faux lupinum Labium leporium Anal atresia Esophageal fistula A neonate in whom the anus was noted to be absent was observed to be occasionally passing urine with traces of meconium, and clear urine on other occasions. Which type of anomaly is present? Anal atresia Anal atresia with rectourethral fistula Anal atresia with rectovesical fistula Rectal atresia Cloacal malformation A male infant weighing 3 kg is born via spontaneous vaginal delivery at 37 weeks’ gestation. His Apgar score is 6/9 at 1 and 5 minutes. The patient is in no apparent distress. Physical examination reveals no anus. What is the most appropriate initial step in this patient’s management? Colostomy Continued observation for 24 hours Intubation and mechanical ventilation Magnetic resonance imaging (MRI) of the abdomen and pelvis Posterior sagittal anorectoplasty Which of the following statements concerning imperforate anus is true? Imperforate anus affects males more frequently than females In 90% of males, but only 50% of females, the rectum ends below the level of the levator ani complex The rectum usually ends in a blind pouch The chance for eventual continence is greater when the rectum has descended to below the levator ani muscles Immediate definitive repair of the anatomic defect is required to maximize the chance of eventual continence What percentage of children with anorectal malformations have co-existing anomalies of the urinary tract? 15-25% 25-35% 35-15% 45-55% 55-65% A newborn male infant has a low anorectal anomaly. What imaging modality is currently recommended for initial imaging of his spine? MRT CT Spinal x-ray and ultrasound Bone scan E. 89. A. B. C. * D. E. 90. A. B. C. D. E. * 91. A. B. * C. D. E. 92. A. B. C. D. * E. 93. A. B. C. * D. E. 94. A. B. C. D. * E. 95. A. B. C. D. * E. Contrast myelography Which of the following is not a recognized feature of the VACTERL association? Vertebral anomalies Anorectal anomalies Eye anomalies (coloboma) Renal anomalies Limb anomalies Which of the following statements about surgical anatomy of the colon and rectum is/are correct? The cecum has the largest inner diameter of all segments of the colon (13 to 15 cm.). The rectosigmoid junction is situated at approximately 1,5 to 1,8 cm. from the anus. The rectum is entirely an intraperitoneal organ. The ileocolic, right colic, and middle colic arteries are branches from the inferior mesenteric artery. The arterial arcade created by communicating vessels at 1 to 2 cm. from the mesenteric is called the artery of Drummond. Which of the following does not cause bilious vomiting in an infant or child? Intestinal malrotation and volvulus Pyloric stenosis Duodenal atresia Small bowel obstruction Ulcerative colitis with colonic obstruction Infants with anorectal anomalies tend to have other congenital anomalies. Associated abnormalities include which of the following? Abnormalities of the cervical spine Hydrocephalus Duodenal atresia Heart disease Corneal opacities Anal incontinence in a patient with rectal prolapse is primarily due to loss of anal rectal angle weakness of endopelvic fascia stretching of pudendal nerves all of the above none of the above Which type of neonatal obstruction is the most likely to be linked to an associated anomaly? Colonic Ileal Jejunal Duodenal All of the above In infants with duodenal atresia all the following statements are true except: There is an increased incidence of Down syndrome. Duodenal atresia can be detected by prenatal ultrasound examination. It may occur in infants with situs inversus, malrotation, annular pancreas, and anterior portal vein. It is best treated by gastroenterostomy. There is a high incidence of associated cardiac defects. 96. A. B. C. * D. E. 97. A. B. C. * D. E. 98. A. B. C. D. E. * 99. A. B. C. D. E. * 100. A. B. C. D. * E. 101. A. * B. C. D. E. 102. A. B. C. * D. Which of the following statements regarding duodenal atresia are true? Reconstruction is best achieved with duodenocolonostomy When associated with an annular pancreas, division of the pancreas at the site of obstruction is curative Bilious vomiting is typical because the obstruction is usually distal to the ampulla of Vater Reconstruction is best achieved with Roux-en-Y duodenojejunostomy All of the above Which of the following signs and symptoms warrant surgical intervention for patients with small bowel obstruction? abdominal tenderness air-fluid levels on abdominal x-ray worsening abdominal pain feculent vomitus air in the colon and rectum on abdominal x-ray On a plain abdominal radiograph two liquid levels were visible. This testifies to the child having: Pyloric atresia Ileal atresia Hirschsprung’s disease Ladd’s syndrome Duodenal atresia A positive Farber’s test is indicative of which pathological state? Ladd’s syndrome Ileal stenosis Duodenal stenosis Pancreatic cystic fibrosis Duodenal atresia The infant begins to have progressively large amounts of bilious emesis. The infant feeds well and has only a small amount of abdominal distention. What is the most likely diagnosis? pyloric stenosis Hirschsprung disease biliary atresia duodenal atresia milk protein allergy The infant have duodenal atresia. If you were to perform an abdominal x-ray, what is the most likely finding that would be seen? “double-bubble” sign scimitar sign normal gas patterns free fluid in the abdomen pneumatosis intestinalis An infant begins to vomit shortly after birth and his abdomen becomes distended. A radiograph of the abdomen shows a "double-bubble" gas shadow. The infant's problem most likely is congenital megacolon malrotation duodenal atresia esophageal atresia E. 103. A. B. C. * D. E. 104. A. B. C. D. E. * 105. A. B. * C. D. E. 106. A. B. C. D. * E. 107. A. B. C. D. E. * 108. A. B. C. D. * E. 109. A. B. C. * D. tracheoesophageal fistula A neonate has bilious vomiting and a doublebubble sign on plain x-ray. The most appropriate operation is: division of annular pancreas gastroenterostomy duodenoduodenostomy duodenal resection duodenojejunostomy For a symptomatic partial duodenal obstruction secondary to an annular pancreas, the operative treatment of choice is A Whipple procedure Gastrojejunostomy Vagotomy and gastrojejunostomy Partial resection of the annular pancreas Duodenojejunostomy A neonate has persistent vomiting of bilestained material. A two-way abdominal roentgenogram shows “double bubble” sign. The most likely diagnosis is annular pancreas duodenal atresia congenital hypertrophic pyloric stenosis Meckel’s diverticulum none of the above Maternal polyhydramnios is associated with esophageal atresia duodenal atresia jejunal atresia all of the above none of the above Which of the following is TRUE regarding duodenal atresia? It is associated with trisomy 21 in 10% cases. Abdominal X-ray is usually normal. Results from disruption of fetal blood supply. Operative repair involves duodenal resection. Concomitant abnormalities can include annular pancreas, esophageal atresia, or VACTERL lesions. Polyhydramnios is frequently observed in all of the following conditions except: Esophageal atresia. Duodenal atresia. Pyloric atresia. Hirschsprung's disease. Congenital diaphragmatic hernia. Which of the following statements about Hirschsprung's disease is false? There are no ganglion cells seen in Auerbach's plexus. There is an increased incidence of Down syndrome. It is more common in girls. It may be associated with enterocolitis. E. 110. A. * B. C. D. E. 111. A. B. C. * D. E. 112. A. B. * C. D. E. 113. A. B. C. * D. E. 114. A. B. * C. D. E. 115. A. B. * C. D. E. 116. A. * B. C. It may involve the small intestine. Which of the followings statement regarding Hirschprung’s disease are true? Suction rectal biopsy is virtually always diagnostic if the specimen includes submucosa Hirschprung’s disease is the result of a sex linked dominant gene The endorectal pullthrough is demonstrably superior to other forms of surgical construction Ten percent or more of patients have an excellent or good functional result following reconstructive surgery The important cause of mortality in contemporary practice is bleeding Which of the following are typical causes of neonatal intestinal obstruction? Intussusception Tumors Hirschprung’s disease Meckel’s diverticulum Incarcerated hernia A newborn presents with a scaphoid abdomen and bilious emesis. Massive intestinal loss could occur if which one of the following causes of neonatal bowel obstruction is not quickly identified and treated? Pyloric stenosis Malrotation with volvulus Meconium ileus Hirschsprung disease Congenital diaphragmatic hernia Thirty-six hours after delivery, an otherwise healthy term infant has not yet passed meconium (has not yet had a bowel movement). Which one of these modalities would not be an appropriate step in the child's workup? Suction rectal biopsy Contrast enema Colonoscopy Chromosomes for delta F-508 Plain abdominal radiography All of the following are associated with an increased incidence of abdominal wound dehiscence EXCEPT bringing a stoma through the incision closure of the wound with a continuous suture presence of ascites presence of jaundice use of braided sutures The most effective method of treating Ladd’s syndrome is: Conservative management Ladd’s operation Soave’s operation Colostomy Hirschsprung’s operation Which of the following is the most informative in diagnosis of HD? Biopsy Plain radiography Contrast enema D. E. 117. A. B. C. D. * E. 118. A. B. C. D. * E. 119. A. * B. C. D. E. 120. A. B. * C. D. E. 121. A. B. C. D. * E. 122. A. * B. C. D. E. 123. A. B. C. D. * Endoscopy Ultrasonography Which of the following is not a method of surgical management in HD? Duhamel’s operation Swenson’s operation Soave’s operation Ladd’s operation Rehbein’s operation Which is not indicative of HD? Aganglionic bowel on biopsy Abnormal recto-anal inhibitory reflex Abdominal distention Abdominal pain Constipation All the following statements concerning Hirschsprung's disease in children are true EXCEPT .an absence of ganglion cells in a dilated segment of the colon is the underlying problem constipation is a classic symptom and almost always begins in the early days of life fecal incontinence is unusual in older affected children rectal examination of affected patients usually reveals an empty rectal ampulla temporary colostomy prior to definitive surgical correction is frequently the treatment of choice Trisomy 21 is associated with wich diseases: malrotation endocardial cushion defect c cleft palate renal disease sensorineural hearing loss The nurse points out a two day old healthy term infant who is otherwise ready for discharge who still has not passed meconium. Your next step is: Order a suppository prior to discharge. Careful physical examination, including digital rectal examination. Give a normal saline enema to prep for a barium enema. Call radiologist to discuss an unprepped barium enema Rectal biopsy. Which of the following is characteristic of Hirschsprung’s disease? Constipation is the most frequent presenting feature. Severity of the symptoms corresponds with the extent of bowel involvement. Acetylcholinesterase activity is decreased in the aganglionic segment. The proximal colon is most commonly affected. It presents most commonly in young adults. The nurse points out a two day old healthy term infant who is otherwise ready for discharge who still has not passed meconium. Your next step is: Order a suppository prior to discharge. Careful physical examination, including digital rectal examination. Give a normal saline enema to prep for a barium enema. Call radiologist to discuss an unprepped barium enema E. 124. A. B. * C. D. E. 125. A. B. C. * D. E. 126. A. * B. C. D. E. 127. A. B. C. D. * E. 128. A. B. C. D. * E. 129. A. B. * C. D. E. 130. A. B. C. Rectal biopsy. Regarding neonatal Hirschsprung’s disease: diagnosis is confirmed by barium enema enterocolitis is the leading cause of death mainly affects females shows absent nerve trunks in the aganglionic segments It is associated with a high incidence of genitourinary tract anomalies What is the most common serious complication of an end colostomy? Bleeding Skin breakdown Parastomal hernia Colonic perforation during irrigation Stomal prolapsed Which of the following statements concerning Hirschsprung’s disease is true? It is initially treated by colostomy It is best diagnosed in the newborn period by barium enema It is characterized by the absence of ganglion cells in the transverse colon It is associated with a high incidence of genitourinary tract anomalies It is the congenital disease that most commonly leads to subsequent fecal incontinence All of the following statements concerning Hirschsprung’s disease are correct EXCEPT that constipation is the most frequent presenting feature enterocolitis is the major cause of death severity of the symptoms corresponds with the extent of bowel involvement acetylcholinesterase activity is increased in the aganglionic segment of the bowel serum and erythrocyte acetylcholinesterase activity is increased Which of the following findings is considered diagnostic of Hirschsprung’s disease on histologic examination of a rectal biopsy specimen? hypertrophy of the muscle coat of the wall of the rectum atrophy of the mucosal lining of the wall of the rectum absence of the nerve fibers that innervate the wall of the rectum absence of parasympathetic ganglion cells in the submucosal and myenteric plexus presence of multiple small polyps along the mucosal surface of the rectal wall A full-term newborn has not passed meconuim by DOL 2. Which of the following is FALSE regarding his likely diagnosis? It is more common in males. Suction rectal biopsy is rarely adequate for diagnosis. Enterocolitis is a significant cause of mortality. Disease is most often confined to the distal colon. Barium enema may be normal. The initial treatment of choice for a 2.5-kg. infant with a 20.0-cm. long proximal jejunal atresia and 8.0 cm. of distal ileum is: Laparotomy, nasogastric suction, proximal dilatation to lengthen the atretic jejunum, total parenteral nutrition, and delayed anastomosis. Laparotomy and proximal end-jejunostomy. Laparotomy and immediate small bowel transplantation. D. E. * 131. A. B. * C. D. E. 132. A. B. C. D. E. * 133. A. B. C. D. * E. 134. A. B. C. D. E. * 135. A. B. * C. D. E. 136. A. B. C. * D. E. 137. Laparotomy and double-barrel enterostomy (jejunum and ileum), with refeeding of jejunal contents into distal ileum and delayed anastomosis. Laparotomy, tapering jejunoplasty, and end-to-oblique jejunoileal anastomosis. Which of the following is the cause of predisposition for midgut volvulus in patients with malrotation? Twisting of the bowel on itself while the bowel is returning to the abdominal cavity from physiologic herniation Formation of a narrow-base mesentery as the gut lengthens on the superior mesenteric artery without rotation Improperly formed peritoneal bands that pull the bowel into a twisted position Arrest in development at stage III of normal embryological gastrointestinal development None of the above Which of the following statements is incorrect? At the fourth week of gestation, the GI tract is a straight tube centrally located in the fetal abdomen. During the fifth week of gestation, the first stage of rotation begins and lasts until the tenth week. The superior mesenteric artery has its axis in line with the omphalomesenteric duct, and the primitive gut normally rotates in a counterclockwise direction. The second stage of rotation occurs between the tenth and twelfth weeks of gestation and involves the return of the intestine to the fetal abdominal cavity. At the completion of the normal rotation, the duodenojejunal junction is not fixed to the left of the aorta and the cecum has loose attachments to the right abdominal wall. Which of the following is the most informative in diagnosing Ladd’s syndrome? Complete blood count Plain thoracic radiography Bronchoscopy Contrast radiography Plain abdominal radiography On a plain abdominal radiograph two liquid levels were visible. This testifies to the child having: Pyloric atresia Ileal atresia Hirschsprung’s disease Ladd’s syndrome Duodenal atresia The most effective method of treating Ladd’s syndrome is: Conservative management Ladd’s operation Soave’s operation Colostomy Hirschsprung’s operation Which of the following signs and symptoms warrant surgical intervention for patients with small bowel obstruction? abdominal tenderness air-fluid levels on abdominal x-ray worsening abdominal pain feculent vomitus air in the colon and rectum on abdominal x-ray Which of the following does not cause bilious vomiting in an infant or child? A. B. * C. D. E. 138. A. B. C. D. E. * 139. A. B. C. * D. E. 140. A. B. C. * D. E. 141. A. B. C. * D. E. 142. A. B. C. D. E. * 143. A. B. * C. D. E. 144. Intestinal malrotation and volvulus Pyloric stenosis Duodenal atresia Small bowel obstruction Ulcerative colitis with colonic obstruction The primitive intestinal loop rotates around an axis formed by which of the following arteries? the inferior epigastric the superior epigastric the obturator the inferior mesenteric the superior mesenteric Malrotation with volvulus is most likely to be present in which of the following patients? A healthy 15-month-old with severe paroxysmal abdominal pain and vomiting A 15-year-old sexually active girl with lower abdominal pain A 3-day-old term infant with bilious emesis, lethargy, and abdominal distension A 4-day-old premature baby (33-week gestation) who has recently started nasogastric feeds; he now has abdominal distention, bloody stools, and thrombocytopenia A 7-year-old girl with abdominal pain, vomiting, fever, and diarrhea A 3-day-old boy presents with 12 hours of bilious vomiting, abdominal pain, and abdominal distension. Which of the following is the most appropriate next step in management? Order an abdominal ultrasonography. Order a computerized tomography scan of the abdomen. Order a upper GI contrast series. Order a barium enema. Order a chest radiograph. A 3-wk-old baby, previously well, presents with sudden onset of bilious vomiting. What study is most appropriate? Abdominal X-ray. CT scan. Upper GI series. Barium enema. Esophageal pH studies. What organ systems are affected by CF and what are the clinical manifestations? Pulmonary Gastrointestinal Hepatic Reproductive All of the above Which of the following are typical causes of neonatal intestinal obstruction? Intussusception Meconium ileus Tumors Meckel’s diverticulum Incarcerated hernia Which of the following is not a component of meconium disease of infancy? A. B. C. D. E. * 145. A. B. C. * D. E. 146. A. B. C. D. E. * 147. A. B. C. D. * E. 148. A. B. C. * D. E. 149. A. B. * C. D. E. 150. A. B. C. D. E. * 151. A. B. C. Meconium ileus Meconium peritonitis Meconium plug syndrome Meconium ileus equivalent (MIE) Meconium stenosis syndrome Which of the following is the pathogenetic basis for meconium ileus? Congenital hepatitis Ileal stenosis Pancreatic cystic fibrosis Portal hypertension Volvulus neonatorum A positive Farber’s test is indicative of which pathological state? Ladd’s syndrome Ileal stenosis Duodenal stenosis Pancreatic cystic fibrosis Duodenal atresia The percentage of CF patients with pancreatic exocrine dysfunction (decreased lipase, amylase, etc.) is: 10% 25% 50% 85% 5% An abnormal sweat test is: supportive of the diagnosis of duodenal atresia diagnostic of cystic fibrosis supportive of the diagnosis of cystic fibrosis has been replaced by genetic testing an abnormal sodium value The most common CF gene is: R1066C Delta F508 Not detected by genetic screening Present in less than 40% of patients none of the above The percentage of CF patients with sinus opacification and/or infection is: 10% 25% 50% 75% 95% What percentage of CF male patients have azoospermia 10% 25% 50% D. E. * 152. A. B. * C. D. E. 153. A. B. C. D. E. * 154. A. B. * C. D. E. 155. A. * B. C. D. E. 156. A. B. C. * D. E. 157. A. B. * C. D. E. 158. A. B. * C. D. 75% 95% The CFTR gene is located on chromosome: 5 7 9 11 13 The life expectancy of newly diagnosed patients with cystic fibrosis is: 5 years 10 years 15 years 20 years 30 years Organisms characteristically isolated from the sputum of patients with cystic fibrosis includes all the following except: Staphylococcus aureus Streptococcus pneumoniae Klebsiella pneumoniae Pseudomonas aeruginosa Burkholderia cepacia Regarding meconium ileus Is a rare cause of neonatal intestinal obstruction Less than 5% of cases are associated with cystic fibrosis Obstruction usually occurs in the distal ileum Presents with neonatal bile-stained vomiting and abdominal distension A plain x-ray may show an intra-luminal 'ground glass' appearance All the following statements about cystic fibrosis are true EXCEPT the incidence in whites is about 1 in 2000 it may present in the newborn period with meconium ileus it is an autosomal dominant disease hepatic involvement is due to inspissated biliary secretions chronic hypoxia, hypercapnia, and acidosis produce pulmonary hypertension Which of the following is FALSE regarding meconium ileus? Underlying diagnosis is usually cystic fibrosis. Most often requires operative intervention. Presents as a neonatal bowel obstruction. X-rays may reveal a stippled pattern in the RLQ (“soap bubble” sign). May be relieved by water-soluble contrast enema. The most important ion to replace in a patient dehydrated after several days of emesis from an pyloric stenosis is bicarbonate chloride hydrogen potassium E. 159. A. B. * C. D. E. 160. A. B. * C. D. E. 161. A. B. * C. D. E. 162. A. * B. C. D. E. 163. A. B. C. D. E. * 164. A. * B. C. D. E. 165. A. B. * C. D. E. sodium All of the following are associated with an increased incidence of abdominal wound dehiscence EXCEPT bringing a stoma through the incision closure of the wound with a continuous suture presence of ascites presence of jaundice use of braided sutures Which of the following does not cause bilious vomiting in an infant or child? Intestinal malrotation and volvulus Pyloric stenosis Duodenal atresia Small bowel obstruction Ulcerative colitis with colonic obstruction Persistent vomiting with hypertrophic pyloric stenosis usually causes what electrolyte imbalance? Metabolic acidosis Metabolic alkalosis Respiratory acidosis Respiratory alkalosis None of the above A mother of a newborn complains of her baby's constant belching with undigested milk. Which developmental anomaly is it an evidence of? Esophageal atresia Faux lupinum Labium leporium Anal atresia Esophageal fistula On a plain abdominal radiograph two liquid levels were visible. This testifies to the child having: Pyloric atresia Ileal atresia Hirschsprung’s disease Ladd’s syndrome Duodenal atresia What developes in cases with decompensated pyloric stenosis: Isotonic dehydration. Hypertonic dehydration [eksikosis]. Hypotonic dehydration. Intoxication. Renal insufficiency. Which of the following does not cause bilious vomiting in an infant or child? Intestinal malrotation and volvulus Pyloric stenosis Duodenal atresia Small bowel obstruction Ulcerative colitis with colonic obstruction 166. A. B. C. * D. E. 167. A. B. C. * D. E. 168. A. B. C. D. E. * 169. A. * B. C. D. E. 170. A. B. * C. D. E. 171. A. * B. C. D. E. 172. A. B. C. D. E. * The patient undergoes pyloromyotomy. During the procedure, a deep mucosal injury is noted at the distal aspect of the incision. How should this patient’s mucosal injury be managed? Closure of the mucosal defect with the myotomy intact Closure of the mucosal and muscular defects with repeat myotomy at a later date Closure of the mucosal and muscular defects with repeat myotomy on another side of the pylorus Wide local drainage and antibiotics Wide local drainage, antibiotics, and 10 to 14 days of postpyloric tube feeding Which of the following sets of electrolytes could be seen with HPS (Na, K, Cl, bicarb): 130, 2.7, 90, 28 130, 5.8, 94, 22 130, 3.9, 98, 17 148, 4.1, 108, 13 148, 2.7, 90, 13 The most important element in the history of an infant with vomiting is: the frequency of vomiting the amount of vomiting the presence of fever if vomiting is projectile if vomiting is bile stained Regarding hypertrophic pyloric stenosis: more common in males most commonly presents in the first week of life vomit is typically bile stained diagnosis should be confirmed with upper gastrointestinal contrast study most commonly presents in the first year of life Which of the following electrolyte abnormalities is consistent with pyloric stenosis? Na 134, K 4.8, C1 114, bicarb 9, glucose 101 Na 135, K 3.5, CI 86, bicarb 37, glucose 69 Na 130, K 5.0, C1 102, bicarb 14, glucose 400 Na 128, K 6.0, CI 95, bicarb 21, glucose 59 Na 150, K 6.0, C1 110, bicarb 25, glucose 75 A 6-week-old male infant has projectile emesis after feeding. He has an olive-shaped abdominal mass on abdominal examination. Which of the following statements is accurate? He likely has hypochloremic metabolic alkalosis. He likely has metabolic acidosis. This condition is more common in female infants. He should be restarted on feeds when the vomiting resolves. He likely will develop diarrhea. A 5-week-old boy presents with 3 days of non-bilious projectile vomiting and dehydration. Which of the following is TRUE about his condition? Immediate laparotomy is warranted. UGI series is the diagnostic procedure of choice. Delay in diagnosis leads to metabolic acidosis. Most commonly seen in females. Fluid replacement consists of ? NS + KCL 173. A. B. C. D. * E. 174. A. B. * C. D. E. 175. A. B. * C. D. E. 176. A. B. * C. D. E. 177. A. * B. C. D. E. 178. A. B. * C. D. E. 179. A. B. C. D. All of the following conditions are derived from the primitive embryonic foregut except: Bronchogenic cyst. Cystic adenomatoid malformation. Gastric duplication. Mesenteric cyst. Pulmonary sequestration. Of the following cystic malformations of the tracheobronchial tree, which is most likely to be asymptomatic when discovered? Intralobar pulmonary sequestration Extralobar pulmonary sequestration Congenital cystic adenomatoid malformation Congenital lobar emphysema None of the above The definitive evaluation of a child with a suspected congenital cystic abnormality of the tracheobronchial tree is best done using which of the following? Rigid bronchoscopy Computerized tomography or magnetic resonance imaging Chest x-ray Angiography Barium esophagogram Which of the following lesions contain no cartilage? Bronchogenic cyst Congenital cystic adenomatoid malformation Congenital lobar emphysema Both of the above Neither of the above Which of the following lesions is a form of foregut cyst? Bronchogenic cyst Congenital cystic adenomatoid malformation Congenital lobar emphysema Both of the above Neither of the above Which of the following lesions is usually associated (has a direct connection or communication) with the tracheobronchial tree? Bronchogenic cyst Congenital cystic adenomatoid malformation Congenital lobar emphysema Both of the above Neither of the above In symptomatic lesions, both CCAM and bronchogenic cysts should be resected. In which of the following, can asymptomatic lesions be followed clinically? Bronchogenic cyst Congenital cystic adenomatoid malformation Congenital lobar emphysema Both of the above E. * E. * Neither of the above Which of the following lesions frequently cause symptoms by mass effect? Bronchogenic cyst Congenital cystic adenomatoid malformation Congenital lobar emphysema Bronchogenic cyst & Congenital cystic adenomatoid malformation Neither of the above Which type of CCAM has the best prognosis? Type 0 Type I Type II Type III Type IV Which type of CCAM is most common? Type 0 Type I Type II Type III Type IV A 9-year-old girl has had recurrent pulmonary infections. Chest roentgenography reveals an anterior mediastinal mass. All the following conditions could result in an anterior mediastinal mass EXCEPT teratoma thymoma lymphoma thymic cyst bronchogenic cyst Which statement is false regarding extrapulmonary sequestration? The parenchyma is not connected to the tracheobronchial tree Arterial blood supply is systemic Venous blood supply is pulmonary Most frequently in males Commonly associated with other anomalies In neonates with congenital diaphragmatic hernia, which of the following statements is true? The defect is more common on the right side. Survival is significantly improved by administration of pulmonary vasodilators. An oxygen index of 20 is an indication for extracorporeal membrane oxygenation (ECMO). Oligohydramnios is a frequent occurrence. Mortality is the result of pulmonary hypoplasia. 186. A. B. C. D. E. * 187. The most common type of congenital diaphragmatic hernia is caused by: A defect in the central tendon. Eventration of the diaphragm in the fetus. A defect through the space of Larrey. An abnormally wide esophageal hiatus. A defect through the pleuroperitoneal fold. Which of the following statements regarding congenital diaphragmatic hernia are true? 180. A. B. C. D. * E. 181. A. B. * C. D. E. 182. A. B. * C. D. E. 183. A. B. C. D. E. * 184. A. B. C. * D. E. 185. A. B. C. D. A. A. B. The incidence of right and left-sided lesions is equal Malrotation is not to be expected Left-to-right shunting via a patent ductus arteriosus is a serious but expected physiologic consequence of pulmonary hypoplasia Survival rates of 5% are reported in several contemporary series Congenital heart disease is present in approximately 20% of these infants There is an emerging consensus that the surgical repair for congenital diaphragmatic hernia is best done: Emergently at the bedside, eliminating the risks of transporting an unstable neonate While on extracorporeal membrane oxygenation When the infant is potentially extubatable Within the first 48 to 72 hours of life All of the above Which of the following hernias is diaphragmatic? Richter’s hernia Bogdalech’s hernia Amyand’s hernia Littre’s hernia None of the above In congenital diaphragmatic hernia all are seen exeptCommon on left side Abdominal distension Can be detected antenatally Heart beat shifted to right Bowel sound in the chest The earliest way to diagnose a diaphragmatic hernia is: by physical exam by history by fetal ultrasound by fetal CT scan by fetal MRI The following are correct regarding diaphragmatic hernia except: is usually on the left side is frequently associated with hypoplastic lungs can present similar to a tension pneumothorax bowel sound in the chest is frequently asymptomatic at birth The following are true about diaphragmatic hernias: often have scaphoid abdomen on exam can be diagnosed antenatally by ultrasound C. D. * E. 194. A. B. at birth often have persistent cyanosis and respiratory distress all of the above none of the above Treatment of diaphragmatic hernia includes: immediate surgical repair pulling the intestines back into the abdominal cavity while still in the delivery room B. C. D. E. * 188. A. B. * C. D. E. 189. A. B. * C. D. E. 190. A. B. * C. D. E. 191. A. B. C. * D. E. 192. A. B. C. D. E. * 193. C. * B. C. provide immediate optimal resuscitation and stabilization first, and then surgery always do primary closure of the diaphragm all of the above The true statement for diaphragmatic hernia below is: The surgeon does not need to worry about medical problems as the neonatologist will already have treated them. There are essentially no medical problems after surgical repair. Improved ultrasound diagnosis has resulted in some women seeking termination of pregnancy. The long term outcome of survivors reveals no significant chronic pulmonary problems. All enumarated You are assisting in the nursery and are the first to examine a newborn. On your examination you find a palpable abdominal mass. Which of the following is the most likely diagnosis? hydronephrosis neuroblastoma Wilms tumor hepatoma diaphragmatic hernia The main cause of postoperative death in children with chronic diaphragmatic hernia is: increased intra-abdominal pressure persistent lung collapse patent ductus arteriosus abnormal pulmonary microvasculature all of the above In determining the proper treatment for a sliding hiatal hernia, the most useful step would be Barium swallow with cinefluoroscopy during Valsalva maneuver Flexible endoscopy 24-h monitoring of esophageal pH Measuring the size of the hernia Assessing the patient’s smoking and drinking history Which of the following is most common after primary esophagostomy for esophageal atresia with a distal tracheoesophageal fistula? Anastomotic leak Esophageal stricture Recurrent tracheoesophageal fistula Gastroesophageal reflux Tracheomalacia requiring aortopexy A mother of a newborn complains of her baby's constant belching with undigested milk. Which developmental anomaly is it an evidence of? Esophageal atresia Faux lupinum Labium leporium D. E. 201. A. Anal atresia Esophageal fistula Esophageal atresia is diagnosed immediately after birth on the basis of: Excessive salivation and foaming at the mouth D. E. 195. A. B. C. * D. E. 196. A. * B. C. D. E. 197. A. B. C. D. * E. 198. A. B. * C. D. E. 199. A. B. C. D. * E. 200. A. * B. C. * D. E. 202. A. B. C. D. E. * 203. A. B. C. * D. E. 204. A. B. * C. D. E. 205. A. B. C. * D. E. 206. A. B. C. D. E. * 207. A. B. Excessive vomiting on feeding Esophageal catheterization Respiratory distress Abdominal distention Which of the following statements about the anatomic course of the esophagus is correct? The cervical esophagus passes behind and to the right of the trachea. The thoracic esophagus enters the posterior mediastinum anterior to the aortic arch. The thoracic esophagus passes behind the right mainstem bronchus and the pericardium. The esophagus enters the diaphragmatic hiatus at the level of T8. The esophagus deviates anteriorly and to the left as it enters the abdomen. Which of the following statements about esophageal anatomy is correct? The esophagus has a poor blood supply, which is segmental in distribution and accounts for the high incidence of anastomotic leakage. The esophageal serosa consists of a thin layer of fibroareolar tissue. The esophagus has two distinct muscle layers, an outer, longitudinal one and an inner, circular one, which are striated in the upper third and smooth in the distal two thirds. Injury to the recurrent laryngeal nerve results in vocal cord dysfunction but does not affect swallowing. The lymphatic drainage of the esophagus is relatively sparse, localized primarily to adjacent paraesophageal lymph nodes. Which of the following statements about the lower esophageal sphincter (LES) mechanism, or high-pressure zone (HPZ), is true? The LES is a circular smooth muscle ring that is 3 to 5 cm. long. In assessing esophageal manometric data, mean HPZ pressure less than 6 mm. Hg or overall length less than 2 cm. is more likely to be associated with incompetence of the LES and gastroesophageal reflux. Esophageal manometry and the acid perfusion (Bernstein) test reliably identify the patient with an incompetent LES mechanism. Distal HPZ relaxation occurs within 5 to 8 seconds of initiating a swallow. Twenty-four–hour distal esophageal pH monitoring is achieved with an intraesophageal pH electrode positioned at the esophagogastric junction. The best management for a 48-hour-old distal esophageal perforation is: Antibiotics and drainage. Division of the esophagus and exclusion of the perforation. Primary repair with buttressing. Resection with cervical esophagostomy, gastrostomy, and jejunostomy. T-tube fistula and drainage. When a stricture is present in association with gastroesophageal reflux, each of the following is an acceptable repair for reflux control except one. Identify the poorest repair. Intrathoracic total fundoplication. Lengthening gastroplasty with total fundoplication. Total fundoplication. Lengthening gastroplasty with partial fundoplication. Partial fundoplication. Which of the following is most reliable for confirming the occurrence of a significant esophageal caustic injury? History of the event. Physical examination of the patient. C. D. * E. 208. A. B. C. D. * E. 209. A. B. C. D. * E. 210. A. B. * C. D. E. 211. A. * B. C. D. E. 212. A. B. C. * D. E. 213. A. B. * C. Barium esophagraphy. CT scaning Endoscopy. Which of the following statement(s) is/are true concerning the blood supply and lymphatic drainage of the esophagus? The thoracic esophagus receives no direct branches from the aorta therefore allowing the technique of transhiatal (blunt) esophagectomy Bleeding esophageal varices are most prominent in the mid-esophagus Lymphatic drainage of the lower third of the esophagus goes entirely to the abdominal lymphatic system Nodal involvement in esophageal cancer is quite common even if the tumor is limited to the level of the submucosa All of the above Regarding the anatomy of the esophagus: the cervical esophagus lies to the right of the midline the thoracic esophagus is anterior to the aortic arch the left vagus nerve passes posterior to the esophagus the cervical esophagus is supplied by the inferior thyroid artery the abdominal esophagus is supplied by the right gastric artery Which of the following contributes to the arterial supply of the thoracic esophagus? right gastric artery bronchial artery pulmonary artery innominate artery inferior thyroid artery An infant with a history of recurrent pneumonia is diagnosed with TEF at 8 months of age. Which of the following statements is correct? The infant most likely has a “H-type” TEF. The infant most likely has proximal esophageal atresia with distal fistula. The infant likely has a previously undetected, associated finding of imperforate anus. The infant is unlikely to have gastroesophageal reflux. The infant is likely to have cystic fibrosis. A 2-year-old girl with a history of esophageal atresia and a ventricular septal defect is hospitalized with Pneumocystis carinii pneumonia. Her immunodeficiency is likely a result of which of the following? Bruton agammaglobulinemia Chronic granulomatous disease DiGeorge syndrome Hyperimmunoglobulin E syndrome Severe combined immunodeficiency syndrome A 2-year-old boy, living with new foster parents for 3 weeks, has become progressively short of breath. When he first arrived at their home, he was active and playful, but now he is too tired to play. They have few details, but they know that he had neonatal surgery for a problem with his “esophagus being connected to his lungs” and that he takes no medications. On examination, he is afebrile, diaphoretic, tachycardic, and tachypneic. His symptoms can most likely be attributed to which of the following? disorder Adjustment Heart failure secondary to ventricular septal defect Kawasaki disease D. E. Reactive airway disease Rheumatic heart disease