Challenges in Diagnosis and Management of Pneumoperitoneum Associated with Pneumatosis Cystoides Intestinalis in Children: A Systematic Review
Abstract
1. Introduction
2. Methods
2.1. Search Strategy
2.2. Study Selection
2.3. Data Extracted
2.4. Quality Assessment
3. Results
Quality Assessment
4. Discussion
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
Abbreviations
PCI | Pneumatosis cystoides intestinalis |
No | Number |
N/A | Not Applicable |
M | Male |
F | Female |
y.o. | Years Old |
TB | Tuberculosis |
ALL | Acute Lymphoblastic Leukemia |
AML | Acute Myeloid Leukemia |
TBI | Traumatic Brain Injury |
BMT | Bone Marrow Transplant |
GvHD | Graft-versus-Host Disease |
MTX | Methotrexate |
ACTH | Adrenocorticotropic Hormone |
SB | Small Bowel |
TI | Terminal Ileum |
AC | Ascending Colon |
HF | Hepatic Flexure |
TC | Transverse Colon |
ICV | Ileocecal Valve |
U/S | Ultrasound |
CT | Computed Tomography |
CXR | Chest X-ray |
JBI | Joanna Briggs Institute |
References
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Study (Year) | Patient No. | Gender | Age (y.o.) | Underlying Disease (No Patients) | Etiology | Symptoms (No Patients) | PCI Location (No Patients) | Diagnosis (No Patients) | Treatment (No Patients) | Outcome (No Patients) | Follow-Up (No Patients) |
---|---|---|---|---|---|---|---|---|---|---|---|
Alp et al. (2010) [7] | 1 | N/A | 8 | Abdominal TB | Abdominal TB | Distension, tenderness | SB, gastritis, duodenitis | AXR, U/S, CT, endoscopy | Conservative | Died (unknown cause) | 3 weeks: little improvement |
Schuster et al. (2017) [4] | 1 | M | 14 | Fanconi anemia | Corticosteroids for GvHD, incomplete atrophic desmosis of colon | Bloody diarrhea, pain, anemia | TI, colon (sparing sigmoid + rectum) | CT | Surgical | Ostomy reversal after 4 months | 1 year: resolved |
Berard et al. (2010) [11] | 1 | F | 8 | Juvenile dermatomyositis | Corticosteroids, MTX | Asymptomatic | Colon | CXR, CT | Conservative | Resolved | 6 weeks: resolved |
Chang et al. (2015) [13] | 1 | F | 13 | Granulomatosis with polyangiitis | Corticosteroids, MTX | Asymptomatic | SB, colon | AXR, CT | Conservative | Resolved | 17 months: resolved |
Kim et al. (2021) [19] | 1 | F | 1 | B-ALL | Blinatumomab | Constipation | AC, HF | CT | Conservative | Resolved | 1 year: resolved |
McCarville et al. (2008) [20] | 16 | 11M/3F | 1–13 | AML (5) ALL (5) Sickle cell anemia (2) Neuroblastoma (1) Myeloblastoma (1) | Corticosteroids | Emesis (2) Pain/diarrhea (9) Asymptomatic (3) | Colon | AXR, CT | Conservative | Resolved | N/A |
Ryan et al. (2020) [22] | 8 | 4M/4F | 1–7 | Hepatoblastoma (3) Biliary atresia (4) Alpha-1 antitrypsin deficiency (1) | Immunosuppressive therapy post-transplant, corticosteroids | Diarrhea (5) Bloody stools (1) Asymptomatic (2) | Cecum, AC, TC | US, CT | Conservative | Resolved | N/A |
Naiditch et al. (2010) [21] | 1 | M | 3 | ALL | Immunosuppressive therapy for GvHD, corticosteroids, antibiotics | Pain, bilious emesis, fever | SB | CXR, CT | Conservative | Resolved | N/A |
Jaffe et al. (1972) [17] | 6 | 4M/2F | 1–5 | ALL | Chemotherapy, corticosteroids, ACTH, blood transfusions, antibiotics | Tenderness/pain (3) Left pneumothorax, pleural effusion, lobar pneumonia (1) Distention (1) Asymptomatic (1) | Colon (1) Colon + rectum (1) Cecum (1) AC (1) Ileus (1) ICV, cecum, AC (1) | AXR (3) Autopsy (3) | Conservative (4) N/A (2) | Resolved (1) Died (5) | Died due to sepsis 5 years later (1) Autopsy showed PCI (5); cause of death was leukemia and sepsis (4) and electrolyte imbalance (1) |
Aygunes et al. (2023) [9] | 1 | F | 4 | AML | Chemotherapy | Fever, pain | Colon | AXR, CT | Conservative | Resolved (21 days) | Inpatient for transplant |
D’Agostino et al. (2000) [14] | 1 | M | 3 | Down syndrome | N/A | Distension, emesis | Jejunum, ileum, colon | AXR | Surgical | Resolved | 1 year: resolved |
Tang et al. (1998) [2] | 5 | N/A | 4 mo–11 y.o. | Combined immunodeficiency | GvHD, corticosteroids | Diarrhea ± blood, distension, pain, fever (4) Asymptomatic (1) | N/A | AXR, US | Conservative | Resolved (4) Died (1) | 2–3.5 weeks: resolved |
Kim et al. (2005) [18] | 1 | F | 9 | TBI | N/A | Distension, bloody diarrhea | N/A | AXR | Conservative | Resolved | 10 days: resolved |
Hochwald et al. (2007) [16] | 1 | F | 1.5 | Rhabdomyosarcoma | N/A | Diarrhea, pain, distention | Colon | CT | Conservative | Resolved | N/A |
Chan et al. (2010) [12] | 1 | M | 2 | Nephrotic syndrome | Corticosteroids | Diarrhea, distension, | Colon, rectum | CXR, CT | Conservative | Resolved | N/A |
Awad et al. (2017) [8] | 5 | 2M/3F | 5–9 | Cerebral palsy | No corticosteroids or immunosuppression | Pain, distention | Colon | AXR | Surgical (4) Conservative (1) | Resolved | Recurrence (4): conservative management |
Yeager et al. (1987) [24] | 1 | M | 5 | Aplastic anemia/BMT | Corticosteroids | Tenderness | Colon | AXR | Conservative | Resolved | 220 days: resolved |
Galal et al. (1981) [15] | 2 | 2F | 4–6 | ALL | Corticosteroids, chemotherapy | Maxillary sinusitis (1) Cough, thoracic tenderness, distention (1) | AC, TC | AXR, CXR | Conservative | Resolved | N/A |
Ade-Ajayi et al. (2002) [6] | 3 | 1M/2F | 11 | ALL (1) AML (2) | Corticosteroids | Pain, diarrhea, distension | N/A | AXR | Conservative | Resolved | Died due to leukemia |
Wallace et al. (2021) [23] | 15 | N/A | N/A | GvHD | Corticosteroids | Distention (11) Asymptomatic (4) | N/A | AXR | Conservative | Resolved | 100 days: died (8) due to other reasons |
Kurbegov et al. (2001) [3] | 8 | N/A | N/A | Tissue transplant, CHD, GI dysmotility | GvHD, Colitis, Ischemia | Distension, pain. diarrhea, fever, emesis | N/A | AXR | Conservative (5) Surgical (2) None (1) | Resolved | N/A |
Bailey et al. (2022) [10] | 8 | N/A | N/A | Oncology patients | Corticosteroids | Pain, distention | N/A | AXR | Conservative (6) Surgical (2) | Resolved | N/A |
Galea et al. (2016) [1] | 7 | 4M/3F | 5–14 | Developmental delay, ALL, encephalitis, eosinophilic gastroenteritis | N/A | Chest pain, seizure, gastroenteritis | Cecum, AC | CT | Conservative (6) Surgical (1) | Resolved | Died (1) due to encephalitis |
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Siouli, C.; Dimopoulou, K.; Dimopoulou, D.; Krikri, A.; Kelaidi, N.; Zavras, N.; Dimopoulou, A. Challenges in Diagnosis and Management of Pneumoperitoneum Associated with Pneumatosis Cystoides Intestinalis in Children: A Systematic Review. J. Clin. Med. 2025, 14, 6479. https://doi.org/10.3390/jcm14186479
Siouli C, Dimopoulou K, Dimopoulou D, Krikri A, Kelaidi N, Zavras N, Dimopoulou A. Challenges in Diagnosis and Management of Pneumoperitoneum Associated with Pneumatosis Cystoides Intestinalis in Children: A Systematic Review. Journal of Clinical Medicine. 2025; 14(18):6479. https://doi.org/10.3390/jcm14186479
Chicago/Turabian StyleSiouli, Christina, Konstantina Dimopoulou, Dimitra Dimopoulou, Aggeliki Krikri, Natalia Kelaidi, Nikolaos Zavras, and Anastasia Dimopoulou. 2025. "Challenges in Diagnosis and Management of Pneumoperitoneum Associated with Pneumatosis Cystoides Intestinalis in Children: A Systematic Review" Journal of Clinical Medicine 14, no. 18: 6479. https://doi.org/10.3390/jcm14186479
APA StyleSiouli, C., Dimopoulou, K., Dimopoulou, D., Krikri, A., Kelaidi, N., Zavras, N., & Dimopoulou, A. (2025). Challenges in Diagnosis and Management of Pneumoperitoneum Associated with Pneumatosis Cystoides Intestinalis in Children: A Systematic Review. Journal of Clinical Medicine, 14(18), 6479. https://doi.org/10.3390/jcm14186479