A 3-day-old boy presented with vomiting and lack of stool for 2 days. Examination found poor condition, abdominal distension, and bloody mucus on rectal exam. Imaging showed signs of malrotation with midgut volvulus. Urgent surgery was needed but found complete necrosis of the midgut. Despite palliative care, the infant did not survive due to the extensive necrosis from the untreated malrotation and volvulus.

1. 03/03/2013
A DEADLY CAUSE OF VOMITING IN A NEWBORN
CLINICAL PRESENTATION A 3-day-old boy is brought to the emergency
department (ED) by his parents with a 2-day history
of feeding intolerance and persistent vomiting of
green fluid.
Dr. Juan Carlos Díaz Torre He was delivered vaginally at home at 39 weeks of
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gestation without any complications. He initially
Pediatra Neonatólogo tolerated breastfeeding well and passed meconium
during the first day of life.
dr_diaz_torre@hotmail.com
(779) 100 - 40 - 26
1 2
Since the second day of life, he has not tolerated
breastfeeding and has been vomiting often. Initially,
the emesis consisted of ingested milk and occurred
30 minutes after eating, but now it is green,
voluminous, and occurs even without oral intake. In
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addition, he has not passed any stool for the last 24
hours.
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Which of the following pieces of additional
information would be most helpful to you at this Correct answer:
time?
A. Any antenatal maternal complication or
A. Any antenatal maternal complication or diagnostics including ultrasound
diagnostics including ultrasound
The mother stated she did not have any
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B. Maternal age antenatal testing or ultrasounds.
C. Any family history of genetic diseases
D. The infant's intake and output status
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2. 03/03/2013
On exam, you have a full-term baby boy in poor
general condition. He is awake but hypotonic and
Our patient's head is normocephalic, with a
hyporeactive. His temperature is 96.6°F (35.9°C),
depressed anterior fontanel, and the mucous
heart rate is 175 beats/min, respiratory rate is 48
membranes are dry.
breaths/min, and blood pressure is 75/40 mm Hg.
He has a generalized grayish coloration, with
The trachea is in a central position and there is no
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acrocyanosis, and poor skin turgor.
jugular venous distension. On chest examination, the
respiratory movements are fast and shallow. Both
lungs are clear to auscultation. Although tachycardic,
the heart rate is regular and without murmurs.
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The patient's upper abdomen is grossly distended
and the lower abdomen is scaphoid. There is a mild
bluish discoloration of the abdominal skin, which
also appears shiny and thin. Subcutaneous veins are You should initiate all of the following
easily seen. The baby retracts his legs upwards and EXCEPT:
cries while the abdomen is being palpated. No
masses are palpated, and no bowel sounds are A. 20 cc/kg bolus of ringer lactate
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noted. There is no rebound tenderness. On rectal
examination with a thermometer, bloody mucus is B. Heel-stick glucose
seen.
The external genitalia are normal for the patient's C. Oxygen by nasal cannula at 1 L/min
age and gender. The extremities are thin and there is
skin tenting. The capillary refill time is documented 9
D. Lumbar puncture 10
at 4 seconds.
The nurse starts a 20 cc/kg bolus of
Correct answer: ringer lactate, oxygen by nasal
cannula at 1 L/min, serum glucose,
A. 20 cc/kg bolus of ringer lactate places a urine catheter with no urine
present in the bladder, places an
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B. Heel-stick glucose orogastric tube and evacuates 35 cc
of bilious material.
C. Oxygen by nasal cannula at 1 L/min
D. Lumbar puncture ****
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3. 03/03/2013
The infant is then brought to the radiology
department for a plain supine abdominal x-ray that This x-ray should be followed by
is depicted below. which diagnostic test?
A. CT of the abdomen and pelvis
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B. Ultrasonography
C. Upper gastrointestinal (GI) series
D. Barium enema
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Correct answer:
A. CT of the abdomen and pelvis
B. Ultrasonography
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C. Upper gastrointestinal (GI) series ****
D. Barium enema
The upper GI contrast study lateral
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view comes back as the above. 16
What is the cause of the child's persistent Correct answer:
vomiting?
A. Malrotation with midgut volvulus ****
A. Malrotation with midgut volvulus
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B. Intussusception
B. Intussusception
C. Necrotizing enterocolitis (NEC)
C. Necrotizing enterocolitis (NEC)
D. Hirschsprung disease
D. Hirschsprung disease
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4. 03/03/2013
This upper GI study confirms the malrotation with
midgut volvulus, with a dilated fluid-filled duodenum
(blue arrow), a "corkscrew" pattern (red arrow), and
the classic "C" shape of the small bowel on the right
side of the abdomen.
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An upper GI study is preferable because malrotation
includes a spectrum of conditions, which may prevent
the intestine from being completely nonrotated to
only partially rotated and can be missed on a barium
enema. 19 20
When normal rotation is not completed, or it does
not happen at all, the small bowel is fixed and
Between the 4th and 10th week of embryonic life, supported only by a narrow base of the mesentery.
the developing small intestine moves outside the
abdominal cavity and into the umbilical cord. By the It can twist in a clockwise direction, causing both a
11th week, rotation and final placement of the bowel obstruction and simultaneously
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intestines occurs, including a 270° counterclockwise compromising perfusion to the entire midgut, giving
turn that leaves the duodenojejunal junction at the it a dark, dusky appearance when viewed surgically.
ligament of Treitz fixed to the left of midline and the
cecum fixed in the right lower quadrant. Malrotation is found in 0.5%-2% of asymptomatic
patients, and it is twice as common in boys as it is in
girls.
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Bilious emesis is the hallmark feature of the diagnosis,
with more than 95% of volvulus patients presenting
with this symptom; an infant who presents with
acidosis should heighten your suspicion for volvulus. It
is commonly associated with polyhydraminos on
prenatal ultrasound.
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Midgut volvulus, as depicted in this image, is the most The initial management of suspected midgut volvulus
common and catastrophic complication of a pre- should include fluid administration, nasogastric
existing malrotation. Approximately 30% of cases occur suctioning, and imaging with plain radiography. Blood
during the first week of life, and greater than 50% of should be sent to the laboratory for a complete blood
cases occur before 1 month of age. cell count and metabolic panel. A finding of acidosis
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should raise suspicion.
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5. 03/03/2013
The other causes of vomiting in an infant should be
excluded with history, physical examination, or
diagnostic imaging. The treatment for malrotation
with or without volvulus is surgical fixation.
The differential diagnosis of a vomiting infant
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includes infectious etiologies, congenital
malformations like malrotation or tracheoesophogeal
fistula, or surgical causes including NEC or pyloric
stenosis.
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While malrotation can present at ages from infancy
to older childhood depending on the amount of
malrotation and obstruction, older children would
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Since malrotation can cause a very proximal present with failure to thrive, malabsorption, and
obstruction, it can be easily confused with duodenal recurrent abdominal pain.
atresia, which presents in the same time period as
malrotation with volvulus.
X-ray evidence of duodenal atresia can show a 27 28
"double bubble" sign, which is evidence of a proximal
small bowel obstruction or a gasless abdomen.
If our patient had a very similar acute presentation of Correct answer:
inconsolable episodic crying, vomiting, and with
blood in the stool but presented at 6 months of age A. Ultrasound with a round, hyperechoic mass at the
and had the above x-rays, what would you expect on gastric outlet
your next diagnostic test?
B. Ultrasound with a swirled appearance of
A. Ultrasound with a round, hyperechoic mass at the sonolucent and hyperechoic bowel wall with a
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gastric outlet loop-within-a-loop appearance ****
B. Ultrasound with a swirled appearance of
sonolucent and hyperechoic bowel wall with a C. CT scan with thickened enlarged appendix
loop-within-a-loop appearance
C. CT scan with thickened enlarged appendix D. A normal x-ray, no need for further evaluation at
D. A normal x-ray, no need for further evaluation at 29 this time 30
this time
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6. 03/03/2013
Intussusception is the predominant cause of Ultrasound demonstrates a swirled appearance of
intestinal obstruction in children 6 months to 6 sonolucent and hyperechoic bowel wall with a loop-
years, with a 1-4 per 1000 live births incidence and within-a-loop appearance, as shown above.
male predominance.
Treatment is reduction with air or contrast enema
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Mortality with treatment is 1%-3%, but if untreated, and can be complicated by recurrence or bowel
this condition is uniformly fatal in 2-5 days. perforation.
Intussusception is the invagination of bowel into
more distal bowel caused by intestinal abnormality,
adhesions, or bowel swelling. 31 32
A. Pyloric stenosis, order an ultrasound and call a
surgeon
B. Malrotation with midgut volvulus, place a
nasogastric tube and call a surgeon
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C. NEC, I.V. fluids administration, place a nasogastric
tube, antibiotics, and admit to the intensive
care
Our patient was delivered at full term, but if he was
delivered prematurely with very similar symptoms of D. Toxic megacolon, I.V. fluids administration, place a
crying inconsolably, blood in his stool, lethargy, and nasogastric tube, and admit to the intensive
33 34
vomiting and had the above x-ray, what is your most care unit.
likely diagnosis and treatment?
Correct answer:
NEC is a disease that, while it is classically a disease
A. Pyloric stenosis, order an ultrasound and call a of premature neonates diagnosed in the neonatal
surgeon intensive care unit, it may occasionally occur in the
term neonate after discharge from the newborn
B. Malrotation with midgut volvulus, place a nursery.
nasogastric tube and call a surgeon
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Symptoms include vomiting, feeding intolerance,
C. NEC, I.V. fluids administration, place a nasogastric and inconsolable crying, and the management
tube, antibiotics, and admit to the intensive would be similar and also include stabilization with
care **** I.V. fluids administration and nasogastric tube
placement.
D. Toxic megacolon, I.V. fluids administration, place a 35 36
nasogastric tube, and admit to the
intensive care unit
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7. 03/03/2013
As soon as the diagnosis of malrotation with midgut
volvulus is seriously entertained, as it was in our
patient, a pediatric surgeon should be contacted to
discuss management and to expedite both
confirmatory studies and definitive care. This
condition is a true surgical emergency, with a
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mortality of approximately 15% and, when surgery is
delayed, there is significant morbidity associated with
An abdominal x-ray like the one above necessary resection of ischemic bowel as seen by the
demonstrates pneumatosis intestinalis or portal dark dusky bowel pictured.
air that is pathognomonic of NEC. Administration
of broad-spectrum antibiotics, pediatric surgical
37 38
consultation, and critical care management is
required.
Treatment of malrotation with midgut volvulus is a Recent studies have shown that laparoscopic
surgical Ladd's procedure through a transverse derotation and Ladd's procedure proved effective in
supraumbilical incision. In this procedure, initially the 75% of cases, with a conversion to an open
bowel is untwisted in a counterclockwise fashion. procedure in 25% of cases with similar rates of
complications of recurrent malrotation and/or
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The Ladd's bands, which are peritoneal attachments volvulus (19%) and significantly shorter times to
from the duodenum, right colon, and cecum, are starting feeds and postoperative length of stay.
divided to prevent further twisting.
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Postoperative complications include recurrent
volvulus (2%-6%), short bowel syndrome, adhesions
causing small bowel obstruction, postoperative
intussusception, and the need for total parenteral
nutrition.
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Mortality rates have been reported between 2%-24% In our case, the patient was found to have necrosis of
depending on the amount of bowel necrosis and the the entire midgut similar to the image above. Given
age of the patient. Less than 10% of necrosis at the the dismal chance of survival, after discussion with the
time of surgery carries nearly a 100% survival rate, family, palliative care was initiated and the child died
while 75% necrosis has only a 35% survival rate. peacefully. This case underscores the importance of
41 diagnosis and treatment in a timely fashion. 42
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8. 03/03/2013
Gracias por su atención
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Dr. Juan Carlos Díaz Torre
Pediatra Neonatólogo
dr_diaz_torre@hotmail.com
(779) 100 - 40 - 26
43
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