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(a, b) Microphotograph of CSID (Patient 4) with thin external muscle layer (hematoxylin and eosin Â40) and dilated lymphatic vessels of the submucosa (hematoxylin and eosin staining, Â40 original magnification); (c) microphotograph of CSID (Patient 3) showing sclerosis of the submucosa (hematoxylin and eosin staining, Â20 original magnification); and (d) microphotograph of the "pyloric tumor" showing hypertrophic muscle layers with some scattered glandular proliferations corresponding to a gastric adenomyoma of the pyloric region (hematoxylin and eosin staining, Â100 original magnification). The yellow arrows in (a) and (b) point to the very thin muscularis propria, while the yellow arrows in (c) and (d) point to the sclerosis of the submucosa and to the gastric adenomyoma of the pylorus, respectively. The gastric adenomyoma of the pylorus is characterized by glandular structures lined by cuboidal to columnar epithelium surrounded by hypertrophic smooth muscle bundles by histological examination. CSID, congenital segmental intestinal dilatation.
Source publication
Background and Aim Congenital segmental intestinal dilatation (CSID) is a neonatal condition with unclear etiology and pathogenesis. Typically, the newborn with CSID presents with a limited (circumscribed) bowel dilatation, an abrupt transition between normal and dilated segments, neither intrinsic nor extrinsic perilesional obstruction, and no aga...
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The prevalence of chronic constipation in the population ranges from 3% to 27%. Women, senile people, and people of low socio‑economic status are in this risk group more often. Many histological studies of the intestinal wall were performed in order to find the causes of slow‑transit constipation [6, 4]. The different pathological changes, includin...
Citations
... The large bowels distal to the dilatation were normal, differentiating it from a congenital pouch colon. Sergi reported a case series of 4 patients diagnosed with congenital segmental bowel dilatation, associated with congenital heart disease [15]. Early detection of CSID in this patient was a challenge due to the non-specific nature of symptoms. ...
... Antenatal ultrasound may show minor cystic changes in the fetal abdomen. Differential diagnosis includes duplication cyst, meconium pseudocyst, mesenteric cyst, meconium ileus, or duodenal atresia [15]. In most instances, congenital segmental bowel dilatation is identified incidentally during a surgical operation. ...
Background
Congenital segmental intestinal dilatation (CSID) is a rare cause of neonatal bowel obstruction. Patients can present in the early newborn period with feeding intolerance and abdominal distension. Diagnosis is challenging due to its rarity and non-specific clinical and radiological findings in a well neonate. Surgical excision remains the mainstay treatment for this disease.
Case presentation
We report a case of a newborn male with congenital segmental ileal dilatation, who presented with abdominal distension and feeding intolerance. He was born preterm at 32 weeks and was treated for presumed sepsis due to a premature rupture of the membrane. The patient developed persistent abdominal distension upon initiation of feedings with large amount of gastric aspirates. Otherwise, there was no delay in the passage of meconium. Serial abdominal radiographs showed persistent focal bowel dilatation on the right side. He was initially diagnosed with small bowel atresia. Ultrasound abdomen and lower gastrointestinal contrasted study showed focal ileal dilatation with no evidence of pneumoperitoneum. Intraoperative findings revealed a segmental ileal dilatation with no evidence of mechanical obstruction. The abnormal dilated bowel was excised and sent for histopathological examination. He recovered well after surgery.
Conclusion
Diagnosis of CSID can be challenging due to its clinical polymorphism and non-specific radiological findings. CSID should be suspected in neonates with feeding intolerance and focal intestinal dilatation without clinical features of sepsis, peritonitis, or mechanical obstruction. Surgical excision is curative with a good outcome.
... Swenson et al. first reported three cases of congenital segmental dilatation of the intestine in 1959; two cases involved the sigmoid colon mimicking Hirschsprung's disease, while the third involved the proximal transverse colon [1]. Since his first description, approximately 120 cases of congenital segmental dilatation of the intestine have been reported, most involving the ileum [2]. The second most commonly reported site of congenital segmental dilatation of the intestine is the colon (with fewer than fifteen reported cases in neonates) [3]. ...
... The second most commonly reported site of congenital segmental dilatation of the intestine is the colon (with fewer than fifteen reported cases in neonates) [3]. Involvement of the duodenum or jejunum is exceedingly uncommon [2]. ...
... In neonates, the condition most commonly presents with symptoms mimicking intestinal obstruction including abdominal distention and feeding intolerance [1]. Depending on the degree of functional bowel obstruction, the presentation may be delayed [2]. Older infants and children most commonly present with constipation and abdominal tenderness [5]. ...
A newborn with congenital segmental dilatation of the intestine affecting the colon is presented. This rare condition, unrelated to Hirschsprung's disease, may affect any portion of the bowel and is characterized by focal dilatation of a segment of bowel flanked by normal proximal and distal bowel. While reported in the surgical literature, congenital segmental dilatation of the intestine has not been reported in the pediatric radiology literature even though pediatric radiologists may be the first to encounter imaging suggesting the diagnosis. We therefore present the characteristic imaging findings, including abdominal radiographs and images from a contrast enema, and discuss the clinical presentation, pathology findings, associations, treatment, and prognosis of congenital segmental dilatation of the intestine to increase awareness of this unusual diagnosis.
... [4] We also published a case report in 2012 and a series of 3 cases in 2016. [3,6] Two patterns of presentation exist: early with neonatal intestinal obstruction; and late with chronic constipation and related sequelae such as failure to thrive, malnutrition, malabsorption, anemia, etc. [2,4,7,8] Ileal segmental dilatation usually presents early whereas colonic segmental dilatation appears to escape the early presentation, though not a rule. [2,6,9] Associated anomalies may alter the presentation as in the index case; we initially missed the segmental dilatation because the colostomy was formed with a small incision; later we identified it on exploratory laparotomy for persistent intestinal obstruction. ...
... [6] Antenatal and preoperative diagnoses are seldom, with most cases diagnosed at surgery. [4,7] Abdominal X-ray, ultrasound, CT scan, and GIT contrast study may help the diagnosis preoperatively; though needs a high index of suspicion. It can mimic other surgical conditions on radiological investigations: pneumoperitoneum on an abdominal radiograph, [6] and duplication cyst on contrast imaging are few examples. ...
... IV Tubular Longer lesions with any type of transition. (7,9,12) V Complex ...
Background: Congenital segmental dilatation of intestine is a rare entity characterized by a localized dilated segment of the intestine. It manifests as neonatal intestinal obstruction. Occurrence with another surgical anomaly may masquerade it and complicate the treatment course.
Case Presentation: A 1-day-old male neonate with anorectal malformation underwent colostomy as first stage of the treatment. The stoma did not function postoperatively. At repeat surgery, congenital segmental dilatation of the ileum, with slightly different morphology, was found. Resection of the lesion and ileostomy were formed. Postoperative course remained uneventful.
Conclusion: Congenital segmental dilatation of intestine may be missed at initial surgery. Complete surgical resection is curative.
... Se presenta el caso clínico de una recién nacida, cuyo interés radica en la presentación típica de la enfermedad descrita por la literatura y la ausencia de comorbilidades, forma clínica escasamente publicada. [1][2][3][4][5][6] ...
Segmental dilatation of the intestine is an extremely rare congenital entity characterized by a local dilation of the intestine without distal obstruction or the absence of ganglion cells. We present the case of a patient in the neonatal period with typical clinical features in absence of other comorbidities, shortly published in the bibliography. We also describe the surgical resolution and the pathological results.
... 3 4 The differential diagnosis of SD includes more common causes of intestinal obstruction, such as midgut volvulus and Hirschsprung's disease. 3 'Bowel loop sign' is the radiological diagnostic hallmark of SD. After surgical resection, this infant made a complete recovery and 43-month follow-up was uneventful. ...
... On the other hand, the immune system, as well as the pulmonary and gastrointestinal organs, remain difficult to manage. At this age an increased rate of infection has been identified, and gastrointestinal dysfunction is common [1][2][3][4][5][6]. Cardiovascular function and sepsis are intimately related and may trigger early death without NICU or PICU intervention [7]. ...
Pediatrics is rapidly evolving, and the diagnostic tools are expanding the spectrum of diagnoses that can be identified at the bedside [...]
A 5-day-old male presented with bilious vomiting, a grossly distended abdomen, and passage of a small amount of stool. The anal opening was at a normal position. X-ray abdomen showed a large bowel loop with a single air-fluid level occupying more than half of the abdominal width. On laparotomy, the ascending colon was dilated to form a pouch-like structure, and the ileum and appendix were opening into it. Colon distal to pouch was present as microcolon. Histopathology of the dilated segment was suggestive of congenital segmental dilatation (CSD). In CSD, the distal bowel is of normal caliber. This is a rare case of CSD of ascending colon with distal microcolon.
A woman in her 30s at 29 weeks of gestation was diagnosed with a fetal abdominal cyst and polyhydramnios. As the cyst gradually increased in size, an elective caesarean section was performed at 38 weeks of gestation. The neonate experienced respiratory distress due to tense abdominal distension shortly after birth. An emergency laparotomy was performed. The intestinal tract was markedly dilated and contiguous with the cyst. The small bowel distal to the dilated intestine had herniated through the defect. The dilated segment was resected, and an ileostomy was created. The operative and histopathological findings suggested segmental dilatation of the intestine (SDI). SDI is a rare gastrointestinal disorder presenting during the neonatal period. It can cause respiratory failure in newborns by compression owing to its large size. SDI should be considered in the differential diagnosis of relatively large abdominal cysts, and collaboration with paediatric surgeons and neonatologists is necessary for successful outcomes.
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.
Congenital segmental dilatation of intestine is a rare clinical entity encountered in neonates with very few case reports from world over. It usually presents with symptoms of partial or total obstruction in neonatal period or infancy. We report a case of a preterm baby with segmental dilatation of intestine which was managed surgically. Diagnosis was confirmed intraoperatively as well as histologically. Consideration of this entity in differential diagnosis of neonatal intestinal obstruction may enable for better understanding and more reporting of this rare condition.
