1' Which finding is NOT present in babies with Gastroschisis and differentiates them from babies wit

1
Baby Quiz Q1

• 1. Which finding is NOT present in babies with
  Gastroschisis and differentiates them from babies
  with Omphalocele?
• Eccentric defect located to the right of the
  umbilicus
• Beckwith-Wiedeman syndrome
• Absence of sac covering extruded gut
• Inflammed thickened gut with matted loops
• All of the above can occur in both lesions.

B Beckwith Weideman syndrome (Macroglossia,
hypoglycemia, exomphalos) presents with
hypoglycemia, which, if untreated or unsuspected,
will cause irreversible brain damage.
Gastroschisis is characterized by the defect
being eccentric to the right of the umbilicus
with extruded, inflamed gut and no sac. These
babies are at risk of gut atresia from the
cincture effect of constriction at the belly wall
defect. Otherwise, associated anomalies are rare.
2
Baby Quiz Q2

• The most common configuration of esophageal
  atresia is
• isolated esophageal atresia
• proximal esophageal atresia, distal TEF
• H type communication between intact esophagus
  and trachea
• Distal esophageal atresia, proximal TEF
• N type cervical fistula.

B 85 of cases have proximal atresia with distal
fistula, demonstrated by air in the GI tract. H
and N are essentially the same connection. N
reflects the downward migration of the trachea
with fetal maturation.
3
Baby Quiz Q3

• Jejuno-ileal atresia
• is a failure of recannulation of primitive
  foregut
• is associated with mucoviscidosis
• results from intra-uterine mesenteric ischemia
• most commonly presents as an apple peel
  deformity
• is best treated with decompressive enterostomy
  and delayed repair.

C Bernard and Louw first defined the embryopogy
of intestinal atresia as the result of
intrauterine disruption of mesenteric blood flow.
The extent of disruption determines the stage of
atresia from stenosis through combined
disruption of intestine and mesentery to apple
peel deformity.
4
Baby Quiz Q4

• Inguinal hernia in infants
• must be repaired immediately upon diagnosis
• are routinely bilateral
• require ligation of the processus vaginalis and
  repair of the internal ring
• are best treated with high ligation of the
  indirect sac
• often resolve by age five years.

D The classic approach to the indirect hernia of
infancy and childhood is high ligation of the
processus vaginalis (indirect sac). The muscles
of the floor are routinely normal with the rare
exception of the premature with an enormous
hernia that effaces the groin musculature.
Inguinal hernias should be repaired electively
when diagnosed, unless incarceration or
strangulation is the presenting finding. There
is no question that there is some continued post
parturition obliteration of the processus
vaginalis. This usually occurs in very premature
infants who are noted to have hernia in the NICU,
but become asymptomatic when seen in follow up
after discharge. Automatic exploration of the
asymptomatic contralateral side is NOT
appropriate, and is one of the few surgical
controversies for which compelling evidence has
been published.
5
Baby Quiz Q5

• Babies with duodenal atresia
• may have Downs syndrome
• usually present with a gasless abdomen
• commonly have bilious emesis
• are usually jaundiced
• require gastro-jejunostomy for bypass of the
  obstruction.

A Downs syndrome is common in infants who present
with duodenal atresia, which is associated with
the classic double bubble on KUB. Despite that
fact that the ampulla of Vater is usually in the
wall of the duodenum proximal to obstruction,
bilious emesis is relatively uncommon, as is
pre-operative jaundice. Most infants can be
repaired with duodenodoudenostomy, however the
safest option when this is not anatomically
feasible is duodenojejunostomy.
6
Baby Quiz Q6

• Meconium ileus
• always requires surgical correction
• is also known as meconium plug syndrome
• is often associated with cystic fibrosis
• routinely presents as a meconium cyst
• is never treated with a Bishop-Koop procedure

C Approximately 98 of babies with meconium ileus
will be determined to have cystic fibrosis.
Surgical intervention is an option when contrast
enema fails, or there are indications of a
surgical abdomen. If inspissated meconium causes
an intractable obstruction early in gestation,
the proximal gut may rupture spilling meconium
intraperitoneally and causing a sterile
peritonitis that walls off a meconium cyst. The
Bishop-Koop procedure is an excellent surgical
option in that it allows normal prograde fecal
flow and access to the distal colon for
irrigation of persistent viscus meconium.
7
Baby Quiz Q7

• Intestinal malrotation
• may lead to midgut volvulus
• is commonly associated with non-bilious emesis
• can only be diagnosed by contrast enema
• usually presents with a scaphoid abdomen
• can be excluded if the infant passes meconium
  within 24 hours of birth.

A Bilious emesis with a distended abdomen is
midgut volvulus until proven otherwise. In the
presence of peritonitis immediate laparotomy is
indicated and may be life saving. If the baby
will tolerate imaging either upper or lower
contrast studies can confirm the diagnosis.
Passage of meconium may simply be the distal
hindgut emptying in response to increased
peristalsis stimulated by the intractable
obstruction of a midgut twisted on its mesentery.
8
Baby Quiz Q8

• Congenital diaphragmatic hernia (CDH)
• are most commonly para-esophageal in location
• are associated with cardiac amomalies
• usually occur in premature infants
• usually involve the posterior-lateral foramen of
  Bockdalek
• require emergency operative repair to salvage the
  baby.

D The classic CDH occurs through the foramen of
Bockdalek. It is associated with a scaphoid
abdomen as most of the midgut is in the left
chest. Operative repair is indicated when the
baby is optimally oxygenated, which may require
EMCO. Prior to development of ECMO, emergency
operative reduction was frequently attempted with
virtually no success.
9
Baby Quiz Q9

• The VATER association
• occurs only in premature infants less than 1000 g
  BW
• is commonly encountered in infants with belly
  wall defects
• only occurs with imperforate anus
• represents simultaneous anomalies in
  organogenesis that occur early in gestation
• is often associated with polycystic renal
  disease.

D In 1974 Smith and Quan reported a constellation
of anomalies consisting of Vertebral, Anal
atresia, Tracheol-Esophageal, and Radial
anomalies. Later the association of cardiac and
renal anomalies were added (VACTERR). The
importance is in recognizing the concomitant
existence of what represents anomalous
organogenesis early in gestation at a point when
multiple organ systems are developing.
10
Baby Quiz Q10

• Necrotizing enterocolitis (NEC)
• usually occurs in healthy term infants
• is a newborn surgical emergency
• is routinely fatal
• is usually caused by rotovirus
• can be treated by simple peritoneal drainage.

E NEC is a medical disease associated with
prematurity or associated anatomic defect. It
can progress to a surgical emergency as manifest
by sudden appearance of free intraperitoneal air,
indicating gut perforation. It has been
associated with rotovirus, however this is an
infectious disease of the hindgut, versus NEC,
which is a multifactorial result of ischemia and
exposure of an immature gut barrier to pathogenic
organisms