Air may be considered the sine qua non of life (without it there is nothing); however, we prefer to have it in specific areas, like our lungs. Finding air where it isn’t supposed to be can often portend serious medical conditions and complications. Previously, we have discussed abnormal collections of air (ex, pneumothorax, pneumomediastinum). Now, let us look at another potentially alarming situation- Pneumatosis Intestinalis:
Pneumatosis Intestinalis – Basics
- Pneumatosis intestinalis refers to gas within the bowel wall.
- Can be visualized on:
- Plain radiographs
- Fluoroscopy (ex, air-contrast enemas)
- CT scans
- It is NOT a diagnosis.
- It is a radiographic or physical finding.
- The cause of the pneumatosis intestinalis is the diagnosis.
- Pneumatosis intestinalis is relatively rare in children.
- When encountered, it is typically in the premature infant or neonate.
- Does occur in older children, who are more likely to have a benign course. [Kurbegov, 2001]
- Divided into two groups:
- Primary (Idiopathic)
- Often benign or incidental findings.
- Usually with no associated symptoms (don’t just look at the image, look at the patient!)
- Radiographically, has very small bubbles of air in the lamina propria.
- More common version (~85%)
- Associated with larger collections of gas – often in linear or curvilinear fashion parallel to the bowel wall.
- Appear like “tram tracks” without railroad ties.
- Potentially ominous sign.
- Primary (Idiopathic)
Pneumatosis Intestinalis – Diagnoses
Once pneumatosis intestinalis is noticed on imaging studies, consider what the cause may be. Below is an abridged list, but a good place to start:
- Process leading to Mucosal Disruption
- Hirschsprung Disease
- Inflammatory Bowel Disease
- Pyloric Stenosis
- Bowel Obstruction
- Blunt Abdominal Trauma (think of Non-accidental Trauma too)
- Process leading to Bowel Necrosis
- Process leading to Immunocompromised State
- Chemotherapies and Steroid Therapies
- Solid Organ Transplant [Abdel-Aziz, 2013]
- Bone Marrow / Stem Cell Transplant [Shulman, 2012; Korhonen, 2012]
- Leukemia / Lymphoma
- Other Associated Conditions
- Rheumatologic Condition [Chang, 2014]
- Collagen Vascular Condition
- Congenital Heart Disease (CHD)
- Pulmonary Disease (ex, Asthma, Cystic Fibrosis)
- Gastroenteritis in immunocompromised patients
- Cases associated with Norovirus known [Kim, 2011]
- Cases associated with Rotavirus, CMV, adenovirus, and C. Diff
- Iatrogenic (ex, placement of G-tube)
Pneumatosis Intestinalis – Management
- Obviously, ABCDEs — manage the patient not the X-ray.
- Management should be geared toward underlying etiology.
- Many cases can be managed conservatively with: [Kim, 2011; Kurbegov, 2001]
- Bowel rest
- IV antibiotics
- Serial examinations
- Supportive therapies for the underlying etiology (ex, treatment for Graft vs Host Disease) [Korhonen, 2012]
- Since steroids are often implicated as possible influencing agent, they should be decreased or discontinued. [Fleenor, 2002]
- Surgery rarely needed.
- Reasonable to consult surgical team, but most can be treated conservatively without surgery. [Shulman, 2012; Li, 2012; Korhonen, 2012; Kurbegov, 2001]
- Cases with free intra-abdominal air do not necessarily benefit from surgery either. [Korhonen, 2012]
- Patients with portal venous gas, evidence of mechanical obstruction, or “high risk” underlying medical condition (CHD or transplant) were more likely to require surgery in one study. [Kurbegov, 2001]
Moral of the Morsel
- Pneumatosis intestinalis is not just for neonates!
- Fortunately, when it occurs in non-neonates, it is often more benign and can be managed conservatively.
- Even though it may be managed conservatively, we should be vigilant and guard against complacency.
Kalkwarf KJ1, Betzold RD, Hung SY, Rinewalt AN, Robertson RD. Management of Pneumatosis Intestinalis in a Pediatric Burn Patient. J Burn Care Res. 2016 Mar-Apr;37(2):e193-5. PMID: 25377864. [PubMed] [Read by QxMD]
Chang CY1, Marzan KA2. Benign pneumatosis intestinalis in a pediatric patient with multiple risk factors including granulomatosis with polyangiitis: a case report and review of the literature. Semin Arthritis Rheum. 2015 Feb;44(4):423-7. PMID: 25455684. [PubMed] [Read by QxMD]
Ye X1, Van JN1, Munoz FM2,3,4, Revell PA2,4,5, Kozinetz CA6, Krance RA2,4, Atmar RL1,3, Estes MK3, Koo HL1,4. Noroviruses as a Cause of Diarrhea in Immunocompromised Pediatric Hematopoietic Stem Cell and Solid Organ Transplant Recipients. Am J Transplant. 2015 Jul;15(7):1874-81. PMID: 25788003. [PubMed] [Read by QxMD]
Abdel-Aziz O1, Elaffandi AH, El Shazly M, Hosny A, El-Karaksy H. Pneumatosis intestinalis following pediatric live-related liver transplant: a case report and successful conservative approach. Pediatr Transplant. 2014 Feb;18(1):E18-21. PMID: 24283569. [PubMed] [Read by QxMD]
Shulman SC1, Chiang F, Haight AE, Steelman CK, Chiang KY, Gow K, Shehata BM. Pneumatosis intestinalis in pediatric hematopoietic stem cell transplantation patients: an uncommon complication. Fetal Pediatr Pathol. 2012 Oct;31(5):309-14. PMID: 22432915. [PubMed] [Read by QxMD]
Korhonen K1, Lovvorn HN 3rd, Koyama T, Koehler E, Calder C, Manes B, Evans M, Bruce K, Ho RH, Domm J, Frangoul H. Incidence, risk factors, and outcome of pneumatosis intestinalis in pediatric stem cell transplant recipients. Pediatr Blood Cancer. 2012 Apr;58(4):616-20. PMID: 21721114. [PubMed] [Read by QxMD]
Kim MJ1, Kim YJ, Lee JH, Lee JS, Kim JH, Cheon DS, Jeong HS, Koo HH, Sung KW, Yoo KH, Choe YH. Norovirus: a possible cause of pneumatosis intestinalis. J Pediatr Gastroenterol Nutr. 2011 Mar;52(3):314-8. PMID: 21150655. [PubMed] [Read by QxMD]
Fleenor JT1, Hoffman TM, Bush DM, Paridon SM, Clark BJ 3rd, Spray TL, Bridges ND. Pneumatosis intestinalis after pediatric thoracic organ transplantation. Pediatrics. 2002 May;109(5):E78-8. PMID: 11986484. [PubMed] [Read by QxMD]
Kurbegov AC1, Sondheimer JM. Pneumatosis intestinalis in non-neonatal pediatric patients. Pediatrics. 2001 Aug;108(2):402-6. PMID: 11483806. [PubMed] [Read by QxMD]