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Семестр: 11
Malformations in children-tests questions
Опис:
6c-med. Final module control
Перелік питань:
1.
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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.
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B.
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15.
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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.
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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.
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30.
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34.
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35.
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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.
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37.
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39.
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40.
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41.
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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.
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B.
C.
D.
E.
44.
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B.
C. *
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45.
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46.
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47.
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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.
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52.
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B. *
C.
D.
E.
53.
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B. *
C.
D.
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54.
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B. *
C.
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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