Authors
Jaime Shalkow, MD
Chief, Department of Surgical Oncology
Division of Surgery
National Institute of Pediatrics
Head Professor, Pediatric Surgical Oncology
Assistant Professor, Surgery and Pediatrics
Universidad Nacional Autonoma de Mexico
Mexico City, Mexico
Disclosure: Jaime Shalkow, MD, has disclosed no relevant financial relationships.
Editors
Catherine A. Lynch, MD
Clinical Instructor and Global Health Fellow
Attending Physician, Department of Emergency Medicine
Emory University School of Medicine, Emory Healthcare
Atlanta, Georgia
Disclosure: Catherine A. Lynch, MD, has disclosed no relevant financial relationships.
Lars Grimm, MD, MHS
House Staff
Department of Internal Medicine
Duke University Medical Center
Durham, North Carolina
Disclosure: Lars Grimm, MD, MHS, has disclosed no relevant financial relationships.
Bruce Buehler, MD
Professor, Department of Pediatrics, Pathology and Microbiology
Executive Director, Hattie B. Munroe Center for Human Genetics and Rehabilitation
University of Nebraska Medical Center
Omaha, Nebraska
Disclosure: Bruce Buehler, MD, has disclosed no relevant financial relationships.
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.
He was delivered vaginally at home at 39 weeks of gestation without any complications. He initially tolerated breastfeeding well and passed meconium during the first day of life. 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 addition, he has not passed any stool for the last 24 hours.
Which of the following pieces of additional information would be most helpful to you at this time?
A. Any antenatal maternal complication or diagnostics including ultrasound
B. Maternal age
C. Any family history of genetic diseases
D. The infant's intake and output status
Answer: A. Any antenatal maternal complication or diagnostics including ultrasound
The mother stated she did not have any antenatal testing or ultrasounds.
On exam, you have a full-term baby boy in poor general condition. He is awake but hypotonic and hyporeactive. His temperature is 96.6°F (35.9°C), heart rate is 175 beats/min, respiratory rate is 48 breaths/min, and blood pressure is 75/40 mm Hg. He has a generalized grayish coloration, with acrocyanosis, and poor skin turgor (as seen in the images above). Our patient's head is normocephalic, with a depressed anterior fontanel, and the mucous membranes are dry. The trachea is in a central position and there is no 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.
Similar to the above image, our 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 easily seen. The baby retracts his legs upwards and cries while the abdomen is being palpated. No masses are palpated, and no bowel sounds are noted. There is no rebound tenderness. On rectal examination with a thermometer, bloody mucus is seen. The external genitalia are normal for the patient's age and gender. The extremities are thin and there is skin tenting. The capillary refill time is documented at 4 seconds.
You should initiate all of the following EXCEPT:
A. 20 cc/kg bolus of ringer lactate
B. Heel-stick glucose
C. Oxygen by nasal cannula at 1 L/min
D. Lumbar puncture
Answer: D. Lumbar puncture
The nurse starts a 20 cc/kg bolus of ringer lactate, oxygen by nasal cannula at 1 L/min, serum glucose, places a foley catheter with no urine present in the bladder, and places an orogastric tube and evacuates 35 cc of bilious material. The infant is then brought to the radiology department for a plain supine abdominal x-ray that is depicted above.
This x-ray should be followed by which diagnostic test?
A. CT of the abdomen and pelvis
B. Ultrasonography
C. Upper gastrointestinal (GI) series
D. Barium enema
Answer: A. Malrotation with midgut volvulus
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. 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.
Between the 4th and 10th week of embryonic life, the developing small intestine moves outside the abdominal cavity and into the umbilical cord. By the 11th week, rotation and final placement of the intestines occurs, including a 270° counterclockwise turn that leaves the duodenojejunal junction at the ligament of Treitz fixed to the left of midline and the cecum fixed in the right lower quadrant.[1] When normal rotation is not completed, or it does not happen at all, the small bowel is fixed and supported only by a narrow base of the mesentery. It can twist in a clockwise direction, causing both a bowel obstruction and simultaneously compromising perfusion to the entire midgut, giving it a dark, dusky appearance when viewed surgically.[2,3]
Malrotation is found in 0.5%-2% of asymptomatic patients, and it is twice as common in boys as it is in girls.[4]
Midgut volvulus, as depicted in this image, is the most common and catastrophic complication of a pre-existing malrotation. Approximately 30% of cases occur during the first week of life, and greater than 50% of cases occur before 1 month of age.[2] 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.[5,6] It is commonly associated with polyhydraminos on prenatal ultrasound.
The initial management of suspected midgut volvulus should include fluid resuscitation, nasogastric suctioning, and imaging with plain radiography.[2] Blood should be sent to the laboratory for a complete blood cell count and metabolic panel. A finding of acidosis should raise suspicion. 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 includes infectious etiologies, congenital malformations like malrotation or tracheoesophogeal fistula, or surgical causes including NEC or pyloric stenosis.
Since malrotation can cause a very proximal obstruction, it can be easily confused with duodenal atresia, which presents in the same time period as malrotation with volvulus. X-ray evidence of duodenal atresia can show a "double bubble" sign, which is evidence of a proximal small bowel obstruction or a gasless abdomen.
While malrotation can present at ages from infancy to older childhood depending on the amount of malrotation and obstruction, older children would present with failure to thrive, malabsorption, and recurrent abdominal pain.
If our patient had a very similar acute presentation of inconsolable episodic crying, vomiting, and with blood in the stool but presented at 6 months of age and had the above x-rays, what would you expect on your next diagnostic test?
A. Ultrasound with a round, hyperechoic mass at the gastric outlet
B. Ultrasound with a swirled appearance of sonolucent and hyperechoic bowel wall with a loop-within-a-loop appearance
C. CT scan with thickened enlarged appendix
D. A normal x-ray, no need for further evaluation at this time
Answer: B. Ultrasound with a swirled appearance of sonolucent and hyperechoic bowel wall with a loop-within-a-loop appearance
Intussusception is the predominant cause of intestinal obstruction in children 6 months to 6 years, with a 1-4 per 1000 live births incidence and male predominance.[7,8] Mortality with treatment is 1%-3%, but if untreated, this condition is uniformly fatal in 2-5 days.[9] Intussusception is the invagination of bowel into more distal bowel caused by intestinal abnormality, adhesions, or bowel swelling. Ultrasound demonstrates a swirled appearance of sonolucent and hyperechoic bowel wall with a loop-within-a-loop appearance, as shown above. Treatment is reduction with air or contrast enema and can be complicated by recurrence or bowel perforation.
Our patient was delivered at full term, but if he was delivered prematurely with very similar symptoms of crying inconsolably, blood in his stool, lethargy, and vomiting and had the above x-ray, what is your most likely diagnosis and treatment?
A. Pyloric stenosis, order an ultrasound and call a surgeon
B. Malrotation with midgut volvulus, place a nasogastric tube and call a surgeon
C. NEC, fluid resuscitation, place a nasogastric tube, antibiotics, and admit to the intensive care
D. Toxic megacolon, fluid resuscitation, place a nasogastric tube, and admission to the intensive care unit
Answer: C. NEC, fluid resuscitation, place a nasogastric tube, antibiotics, and admit to the intensive care
NEC is a disease that, while it is classically a disease of premature neonates diagnosed in the neonatal intensive care unit, it may occasionally occur in the term neonate after discharge from the newborn nursery. Symptoms include vomiting, feeding intolerance, and inconsolable crying, and the management would be similar and also include stabilization with fluid resuscitation and nasogastric tube placement. An abdominal x-ray like the one above demonstrates pneumatosis intestinalis or portal air that is pathognomonic of NEC. Administration of broad-spectrum antibiotics, pediatric surgical consultation, and critical care management is required.
As soon as the diagnosis of malrotation with midgut volvulus is seriously entertained, as it was in our patient, a general 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 mortality of approximately 15% and, when surgery is delayed, significant morbidity associated with necessary resection of ischemic bowel as seen by the dark dusky bowel pictured above.
Treatment of malrotation with midgut volvulus is a surgical Ladd's procedure through a transverse supraumbilical incision. In this procedure, initially the bowel is untwisted in a counterclockwise fashion. The Ladd's bands, which are peritoneal attachments from the duodenum, right colon, and cecum, are divided to prevent further twisting. Recent studies have shown that laparoscopic derotation and Ladd's procedure proved effective in 75% of cases, with a conversion to an open procedure in 25% of cases with similar rates of complications of recurrent malrotation and/or volvulus (19%) and significantly shorter times to starting feeds and postoperative length of stay.[10,11]
Postoperative complications include recurrent volvulus (2%-6%), short bowel syndrome, adhesions causing small bowel obstruction, postoperative intussusception, and the need for total parenteral nutrition. Mortality rates have been reported between 2%-24% depending on the amount of bowel necrosis and the age of the patient.[3] Less than 10% of necrosis at the time of surgery carries nearly a 100% survival rate, while 75% necrosis has only a 35% survival rate.[2]
In our case, the patient was found to have necrosis of the entire midgut similar to the image above. Given the dismal chance of survival, after discussion with the family, palliative care was initiated and the child died peacefully. This case underscores the importance of diagnosis and treatment in a timely fashion.
Authors
Jaime Shalkow, MD
Chief, Department of Surgical Oncology
Division of Surgery
National Institute of Pediatrics
Head Professor, Pediatric Surgical Oncology
Assistant Professor, Surgery and Pediatrics
Universidad Nacional Autonoma de Mexico
Mexico City, Mexico
Disclosure: Jaime Shalkow, MD, has disclosed no relevant financial relationships.
Editors
Catherine A. Lynch, MD
Clinical Instructor and Global Health Fellow
Attending Physician, Department of Emergency Medicine
Emory University School of Medicine, Emory Healthcare
Atlanta, Georgia
Disclosure: Catherine A. Lynch, MD, has disclosed no relevant financial relationships.
Lars Grimm, MD, MHS
House Staff
Department of Internal Medicine
Duke University Medical Center
Durham, North Carolina
Disclosure: Lars Grimm, MD, MHS, has disclosed no relevant financial relationships.
Bruce Buehler, MD
Professor, Department of Pediatrics, Pathology and Microbiology
Executive Director, Hattie B. Munroe Center for Human Genetics and Rehabilitation
University of Nebraska Medical Center
Omaha, Nebraska
Disclosure: Bruce Buehler, MD, has disclosed no relevant financial relationships.
Abdominal Vascular Occlusion
Can't Miss Intestinal Complications on Abdominal Radiographs
Case Quiz: A Can't-Miss Abdominal Catastrophe
Case Quiz: A Newborn With Strange Coloration
