ABSTRACT
Littre's umbilical hernia (UH) is a rare disease, the third most common Littre hernia. Most case reports interest adult patients. We reported the case of a four-year-old girl with anemia and symptomatic UH, with an incidentally diagnosed Meckel's diverticulum (MD) containing pancreatic ectopic tissue. We reviewed case reports on Littre's umbilical hernia without a date or language restriction. Including our patient, 21 cases were reviewed, of whom 15 (71.4%) were adults and 13 (61.9%) were males. Complicated umbilical hernia occurred in 13 patients (61.9%) and symptomatic MD in two children (9.5%). Investigations preoperatively diagnosed two patients (9.5%). Eighteen patients (85.7%) underwent open surgery, Meckel's diverticulum removal was performed in 18 patients (85.7%), and primary umbilical hernia repair was performed in 16 (76.2%). Ectopic tissue was present in four patients (19.1%), and long-term outcomes were excellent in all patients.
ABSTRACT
OBJECTIVE: Congenital segmental dilatation of the intestine (CSDI) is a rare gastrointestinal condition. We conducted a scoping review through MEDLINE and Google Scholar, collecting data from 1959 through August 2020 to better understand this peculiar disease. METHODS: The clinical and pathological features of 150 patients were reviewed. RESULTS: The mean age was 25.9 days, and 61.3% of patients were male. An antenatal diagnosis was made in 15.3% of patients. Predominant symptoms included abdominal distension (83.9%) and vomiting (61.3%). Pallor and anemia were associated with ileal CSDI. The most common sites of the lesion were the ileum (56%) and colon (27.3%). Associated anomalies occurred in 57.3% of the patients, of which the most common included other abnormalities of the digestive system (69.8%), abdominal wall (19.8%), and cardiovascular system (11.6%). Resection and anastomosis was performed in 83.3% of patients. Postoperative complications occurred in 10%. Normal ganglion cells were commonly found (97.3%), while muscle layer hypertrophy and atrophy were found in 14.7% and 13.3% of the patients, respectively. Abnormal interstitial cells of Cajal were identified in four patients. Death occurred in 12.7% of patients. Demise was significantly associated with the duodenal location of CSDI (Mantel-Cox test, p = 0.002). CONCLUSION: CSDI remains poorly understood, and mortality is associated chiefly with its duodenal location. Further research is needed, and biorepositories should be promptly set up to study this disease in the future better.
What is currently known about the topic? CSDI is a sporadic condition, which can represent a challenge for neonatologists.What new information is contained in this article? Pallor and anemia are found explicitly in ileal CSD, and the duodenal location of CSDI is associated with the highest mortality rate.
