1. Lily Ghavi PGY1 JMH/Holtz Pediatrics EM conference 6/2/16
Midgut volvulus
Lily Ghavi
PGY1
JMH/Holtz Pediatrics
EM conference 6/2/16

2. FT4 week old male, previously well, began experiencing symptoms of fussiness, poor feeding since the morning of presentation. Presents to ED 8 hours since onset of symptoms. While waiting, was reported to have a vomit as seen in Figure 1. Mom denies blood in his stool. The mother then asks you, the physician, if its “normal that his belly looks so big?” when it typically is not so large. Patient is non-toxic appearing, fussy but consolable. Abdomen is significantly distended patient is diffusely very TTP, no organomegaly or palpable masses no bowel sounds are present and infant is vitals are as follows: HR: 140 BP 90/75 Temp 37.2 C RR: 36. You decide to obtain an abdominal x-ray from the ED as seen in Figure 2. What is the most common presenting symptom in a patient with this diagnosis?
a) Vomiting, nonbilious, abdominal distension
b) Vomiting bilious, abdominal distension
c) Vomiting bilious OR nonbilious, abdominal distension
d) Blood in stool, poor urine output
e) Loose, bloody stools

3. Figure 1
Figure 2

4. FT4 week old male previously well, began experiencing symptoms of fussiness, poor feeding since the morning of presentation. Presents to ED 8 hours since onset of symptoms. While waiting, was reported to have a vomit as seen in Figure 1. Mom denies blood in his stool. The mother then asks you, the physician, if its “normal that his belly looks so big?” when it typically is not so large. Patient is non-toxic appearing, fussy but consolable. Abdomen is significantly distended patient is diffusely very TTP, no organomegaly or palpable masses no bowel sounds are present and infant is vitals are as follows: HR: 140 BP 90/75 Temp 37.2 C RR: 36. You decide to obtain an abdominal x-ray from the ED as seen in Figure 2. What is the most common presenting symptom in a patient with this diagnosis? HR: 140 BP 90/75 Temp 37.2 C RR: 36. You decide to obtain an abdominal x-ray from the ED as seen in Figure 2. What is the most common presenting symptom in a patient with this diagnosis? a) Vomiting, nonbilious, abdominal distension b) Vomiting bilious, abdominal distension c) Vomiting bilious OR nonbilious, abdominal distension d) Blood in stool, poor urine output e) Loose, bloody stools

5. Midgut Volvulus
Complication of gut malrotation (during fetal development) that can occur in neonates, infants (and children, adults)
1 in 500 births
bowel’s abnormal position and connections lead to excessive mobility, which predisposes to bowel compression, kinking, or volvulus and also intussusception.
dense fibrous bands that can further predispose to volvulus or occasionally cause obstruction themselves.
50% present in first week and 60% before the end of the 1st month with this complication
Twisting of bowel around SMA  vascular compromise of midgut  ischemia and necrosis which can become irreversible

6. Malrotation with midgut volvulus

7. Question 1 explanation
Bilious vomiting and bloody stools were the two most common clinical presentations in neonates with midgut volvulus
Bilious vomiting, recurrent abdominal pain and failure to thrive were the most common symptoms after the newborn period.
Typically volvulus also presents with abdominal distension and tenderness
Hematochezia or melena (20%)  bowel ischemia
Remember: vomit can be bilious OR non bilious
=/- bile is not an indicator of severity of volvulus or indicator for ischemia
Does not signify morbidity or mortality
Uptodate and Lin JN , Lou CC , Wang KL
Department of Pediatric Surgery, Chang Gung Children's Hospital, Kweishan, Taoyuan, Taiwan, ROC.
Journal of the Formosan Medical Association = Taiwan yi zhi [1995, 94(4): ]
Type: Journal Article, Review

8. Presentation and complications
Abdominal tenderness associated with hemodynamic instability/deterioration
Septic presentation
Metabolic acidosis
Apneic episodes
Third spacing, fluid loss
Typically an acute presentation but can present as chronic/episodic vomiting/abdominal pain with failure to thrive, chronic diarrhea, malabsorption
Vomiting or intolerance to feeds occurs in > 90% of newborns with malrotation

9. 4 -week old FT female presents with persistent crying for 12 hours
4 -week old FT female presents with persistent crying for 12 hours. Mother has tried all typical methods of soothing the infant but continues to be only intermittently consolable. Infant had one episode of non bilious, non bloody emesis and has been feeding poorly since yesterday morning. Last stool from this morning noted had bright red blood per mother’s report. Vitals normal at presentation, however abdomen is distended, no bowel sounds present, generalized tenderness on palpation to abdomen, no organomegaly. You tell the mother not to feed the baby while you do the workup. You place orders for BMP You return to check the infant 15 min later, infant is lethargic, patient is becoming ashen and repeat vitals are as follows: PE: Vitals: HR 172 beats/min, RR 50breaths/min, BP 59/39. You do a stat blood gas and results are as follows: pH 6.8, PCO2 31, PO2 163 and base deficit 29. Which is most accurate? a) patient is hemodynamically stable, monitor clinically – no imaging required b) patient is hemodynamically stable, imaging – abdominal X-ray c) patient is not hemodynamically stable, STAT CT scan of the abdomen d) patient is not hemodynamically stable, IV fluids and monitor over several hours for further emesis e) patient is not hemodynamically stable, rapid and aggressive IV fluid (IV/IO) resuscitation and emergent surgical management with laparotomy/laparoscopy

10. b) patient is hemodynamically stable, imaging – abdominal X-ray
4 -week old FT female presents with persistent crying for 12 hours. Mother has tried all typical methods of soothing the infant but continues to be only intermittently consolable. Infant had one episode of non bilious, non bloody emesis and has been feeding poorly since yesterday morning. Last stool from this morning noted had bright red blood per mother’s report. Vitals normal at presentation, however abdomen is distended, no bowel sounds present, generalized tenderness on palpation to abdomen, no organomegaly. You tell the mother not to feed the baby while you do the workup. You place orders for BMP You return to check the infant 15 min later, infant is lethargic, patient is becoming ashen and repeat vitals are as follows: PE: Vitals: HR 172 beats/min, RR 50breaths/min, BP 59/39. You do a stat blood gas and results are as follows: pH 6.8, PCO2 31, PO2 163 and base deficit 29. Which is most accurate?
a) patient is hemodynamically stable, monitor clinically – no imaging required
b) patient is hemodynamically stable, imaging – abdominal X-ray
c) patient is not hemodynamically stable, STAT CT scan of the abdomen
d) patient is not hemodynamically stable, IV fluids and monitor over several hours for further emesis
e) patient is not hemodynamically stable, rapid and aggressive IV fluid (IV/IO) resuscitation and emergent surgical management with laparotomy/laparoscopy

11. Stability vs. instability
Malrotation complicated by volvulus is emergent situation
Systemic decompensation
Hematemesis
Hematochezia
Abdominal distention
Peritonitis
Shock
Skin color changes, ashen
--> IMMEDIATE surgical management with aggressive fluid resuscitation
When malrotation is complicated by volvulus, it is a potentially life-threatening condition and requires emergent evaluation and treatment. If the child has volvulus with signs of systemic decompensation (eg, hematemesis, hematochezia, abdominal distension, peritonitis, and shock), no additional evaluation is needed. The child should be rapidly resuscitated and immediately taken to surgery for exploration. (See 'Treatment' below.)
If the child is hemodynamically stable, the diagnosis should be confirmed by radiologic evaluation. This evaluation typically begins with plain radiographs, which are rarely diagnostic but are important to exclude obvious perforation indicated by pneumoperitoneum. Plain radiographs are followed by an upper gastrointestinal (GI) contrast series, which is the best examination to visualize the duodenum in infants and children. It should be performed, whenever possible, under fluoroscopy by an experienced pediatric radiologist. Barium enema and ultrasonography can be useful adjuncts when abnormal findings are present, but normal findings do not exclude malrotation. Computed tomography of the abdomen with intravenous contrast is not the recommended diagnostic study for malrotation in infants and children but may be appropriate in adults. Radiographic findings suggestive of malrotation are listed in the table (table 2).

12. If hemodynamically stable: Confirm with radiologic evaluation
Plain XR
Not typically diagnostic (and do not rule it out) , but r/o pneumoperitoneum or signs of bowel perforation
Upper GI studies (Gold standard) best exam; fluoroscopy with pedi radiologist.
Ultrasound

14. XR

15. XR

16. Upper GI
A limited upper gastrointestinal (UGI) contrast series is the best examination to visualize the duodenum; failure of the duodenum to cross the midline confirms the presence of malrotation. UGI contrast series should be performed whenever possible, under fluoroscopy and by an experienced pediatric radiologist. The addition of a small bowel follow-through or barium enema to check for colonic malrotation is prudent in patients in whom the UGI is normal but there is a high index of suspicion or signs of a distal bowel obstruction. Barium contrast studies may reveal a corkscrew appearance of the twisted small bowel, or a "bird's peak" if complete obstruction is present. The clinical presentation, evaluation, and management of malrotation are discussed in greater detail separately. (See "Intestinal malrotation in children".)

17. Ultrasound
Whirpool sign In experienced hands, intestinal malrotation can be diagnosed by color Doppler ultrasonography. Malrotation is seen as inversion of the superior mesenteric artery (SMA) and superior mesenteric vein (SMV) relationship, with the SMA on the right and SMV on the left, along with other findings such as duodenal dilatation with a tapering configuration [10-12]. A normal ultrasound, however, does not exclude malrotation. (See "Intestinal malrotation in children", section on 'Ultrasonography'.)  

18. Key points
Malrotation with midgut volvulus can be successfully treated with timely diagnosis
Delay in diagnosis  time is bowel (and life).
Sudden, catastrophic event
Ischemic bowel necrosis, Metabolic acidosis
Sepsis, systemic shock
Bilious/nonbilious vomit with other normal physical findings
Bilious emesis in FT neonate should be considered midgut volvulus until proven otherwise.
(2) J Emerg Med 2013
*delay in diagnosis leads to increased mortality or more severe outcomes.
*Mortality higher in immediate newborn period vs older infant. Median age survival 30 days. Worse outcomes presenting in first week.

19. What to do? Surgical consultation Labs +/- blood gas
CMP
CBC
CRP
+/- blood gas
+/- imaging depending on presentation
IV fluids
NPO
Consider Nasogastric tube
antibiotics initiated to cover gram-positive, gram-negative, and anaerobes if septic

20. Resources
Brandt, Mary L., MD. "Intestinal Malrotation in Children." Intestinal Malrotation in Children. Up To Date, Oct.-Nov Web. 18 June 2016.
Lee, Henry Chong, Sarah S. Pickard, Sunita Sridhar, and Sanjeev Dutta. "Intestinal Malrotation and Catastrophic Volvulus in Infancy." The Journal of Emergency Medicine 43.1 (2012): n. pag. Web. 18 June 2016.
Stanich, Jessica A., MD. "Intussusception and Midgut Volvulus." AHC Media Continuing Medical Education Publishing RSS. Mayo Clinic, Web. 18 June 2016.
F, Sean. "Malrotation Presentation and Management." Pediatric EM Morsels. PedEM Morsels, 27 Jan Web. 20 June 2016.
Kandahar, P. "1129: Malrotation with Midgut Volvulus Presenting as Pediat... : Critical Care Medicine." LWW. Beaumont Hospital, Web. 20 June 2016