Overview
Spontaneous intestinal perforation (SIP) is a serious neonatal condition characterized by a sudden, isolated perforation (hole) of the intestinal wall, most commonly occurring in the terminal ileum (the last part of the small intestine). It predominantly affects extremely premature and very low birth weight infants, typically within the first two weeks of life. SIP is distinct from necrotizing enterocolitis (NEC), although the two conditions can initially present similarly. Unlike NEC, SIP involves a focal perforation without the widespread intestinal inflammation and necrosis that characterizes NEC. The condition primarily affects the gastrointestinal system. Key clinical features include sudden abdominal distension, bluish discoloration of the abdomen, and signs of clinical deterioration such as low blood pressure, respiratory distress, and metabolic instability. Diagnosis is often confirmed by the presence of free air in the abdominal cavity (pneumoperitoneum) seen on abdominal X-ray. Risk factors include extreme prematurity, very low birth weight, early postnatal use of indomethacin or ibuprofen (used to close a patent ductus arteriosus), and postnatal corticosteroid exposure, particularly when combined with these anti-inflammatory medications. Treatment requires urgent surgical intervention. The two main approaches are primary peritoneal drainage, where a drain is placed into the abdominal cavity at the bedside, or laparotomy with surgical repair of the perforation. The choice of intervention depends on the infant's clinical stability and the surgeon's assessment. Outcomes have improved with advances in neonatal intensive care, but SIP remains associated with significant morbidity and mortality, particularly in the most premature infants. Long-term outcomes are generally more favorable compared to NEC, with lower rates of intestinal complications such as short bowel syndrome.
Also known as:
Sporadic
Usually appears on its own, not inherited from a parent
Neonatal
Begins at or shortly after birth (first 4 weeks)
FDA & Trial Timeline
10 eventsCairo University — NA
Armed Forces Institute of Dentistry, Pakistan — NA
The Rotunda Hospital — NA
Fayoum University — NA
Future University in Egypt — NA
Temple University — NA
Aula Dental Avanzada — NA
Tehran University of Medical Sciences — NA
Tatsuo Kawai, MD, PhD — EARLY_PHASE1
University of Fujairah — NA
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Spontaneous intestinal perforation.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Spontaneous intestinal perforation at this time.
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Spontaneous intestinal perforation.
Community
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Start the conversation →Latest news about Spontaneous intestinal perforation
Disease timeline:
New recruiting trial: Intraligamentary vs Inferior Alveolar Nerve Block for Pulpitis: A Randomized Clinical Trial
A new clinical trial is recruiting patients for Spontaneous intestinal perforation
New recruiting trial: Sleep in Psychiatric Care (SIP): A Transdiagnostic Group-based Sleep-school as Treatment for Comorbid Insomnia
A new clinical trial is recruiting patients for Spontaneous intestinal perforation
New recruiting trial: Postoperative Pain After Pulpotomy in Children Using Calcium Silicate Cements With and Without Laser Therapy
A new clinical trial is recruiting patients for Spontaneous intestinal perforation
New recruiting trial: VAC-SIP-YF (Vaccination- Safety & Immunogenicity During Pregnancy- Yellow Fever)
A new clinical trial is recruiting patients for Spontaneous intestinal perforation
New recruiting trial: Sip and Snack Better (SSB) Study: Improving Added Sugar in Adolescents
A new clinical trial is recruiting patients for Spontaneous intestinal perforation
New recruiting trial: Comparison of 'Sip Til Send' Regimens Prior to Elective Caesarean Delivery Using Bedside Gastric Ultrasound
A new clinical trial is recruiting patients for Spontaneous intestinal perforation
New recruiting trial: A Randomized Controlled Trial Evaluating Whether Pre-operative Trinase Reduces Post-operative Pain in Patients With Symptomatic Irreversible Pulpitis Compared to Placebo, Using VAS Scores and Analgesic Intake Over 48 Hours.
A new clinical trial is recruiting patients for Spontaneous intestinal perforation
New recruiting trial: Short Term Comparative Assessment of MTA and Biodentine in Complete Pulpotomy for Symptomatic Irreversible Pulpitis
A new clinical trial is recruiting patients for Spontaneous intestinal perforation
New recruiting trial: Sleep in Psychiatric Care (SIP): Treatment for Comorbid Delayed Sleep-Wake Phase Disorder (DSWPD)
A new clinical trial is recruiting patients for Spontaneous intestinal perforation
New recruiting trial: Postoperative Pain After Full Pulpotomy With TheraCal PT, Biodentin, and MTA
A new clinical trial is recruiting patients for Spontaneous intestinal perforation
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Common questions about Spontaneous intestinal perforation
What is Spontaneous intestinal perforation?
Spontaneous intestinal perforation (SIP) is a serious neonatal condition characterized by a sudden, isolated perforation (hole) of the intestinal wall, most commonly occurring in the terminal ileum (the last part of the small intestine). It predominantly affects extremely premature and very low birth weight infants, typically within the first two weeks of life. SIP is distinct from necrotizing enterocolitis (NEC), although the two conditions can initially present similarly. Unlike NEC, SIP involves a focal perforation without the widespread intestinal inflammation and necrosis that characteriz
How is Spontaneous intestinal perforation inherited?
Spontaneous intestinal perforation follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Spontaneous intestinal perforation typically begin?
Typical onset of Spontaneous intestinal perforation is neonatal. Age of onset can vary across affected individuals.
Which specialists treat Spontaneous intestinal perforation?
16 specialists and care centers treating Spontaneous intestinal perforation are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.