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"Malrotation"

Case Report

[English]
Colonic Atresia, Malrotation, and Hirschsprung’s Disease in a Newborn: Report of 2 Cases
Sungjoo Park, Wontae Kim, Sanghoon Lee, Jeong-Meen Seo
Adv Pediatr Surg 2025;31(1):41-45.   Published online June 16, 2025
DOI: https://doi.org/10.13029/aps.2025.31.1.41
The concurrent occurrence of colonic atresia, malrotation, and Hirschsprung’s disease in neonates is extremely rare. These anomalies often share embryologic origins and present overlapping clinical symptoms that complicate diagnosis and management. We report two neonatal cases with this rare triad. Case 1 involved a term neonate initially diagnosed with esophageal atresia and later found to have colonic atresia, malrotation, and Hirschsprung’s disease. Case 2 was a preterm neonate presenting with abdominal distension and perforation, ultimately diagnosed with the same triad. Both underwent staged surgical management, including Duhamel’s procedures after confirming aganglionosis. Awareness of the possible coexistence of these anomalies is essential in neonates with colonic atresia and non-fixed colon. Surgical planning should anticipate aganglionosis and include rectal biopsy. This report emphasizes the importance of early suspicion and multidisciplinary approach for optimal outcomes.
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Original Articles

[English]
Comparison of the Clinical Characteristics of Intestinal Malrotation in Infants and Children
Jeung Min Huh, Suk Bae Moon, Soo Min Jung, Hyun Baik Shin, Jeong Meen Seo, Suk Ku Lee
J Korean Assoc Pediatr Surg 2010;16(2):126-133.   Published online December 31, 2010
DOI: https://doi.org/10.13029/jkaps.2010.16.2.126

Malrotation is a congenital anomaly that becomes symptomatic more frequently during infancy. The indication for surgical treatment at that age is straightforward. In older children, the diagnosis may be more difficult because of chronic and vague complaint. The aim of this study is to compare the symptoms, rate of volvulus and surgical findings in children younger and older than one year. A retrospective study of 40 patients in a a single medical center diagnosed with malrotation from April 1996 to May 2010 was performed. There were 20 (50%) boys and 20 (50%) girls. At the time of operation, 27 (67.5%) patients were younger and 13 (32.5%) were older than 1 year. Vomiting was seen in 20 cases (74.1%) of the younger group compared to 2 cases (15.4%) of the older group. Abdominal sonography and upper gastrointestinal series showed a sensitivity of 100%. Operative findings: 12 (44.4%) of the younger group presented with volvulus compared to none of the older group. The Ladd's procedure was routinely performed with appendectomy in all cases and bowel resection was requires when volvulus included bowel necrosis or other anomalies were found. After definite procedures, surgical correction for adhesive obstruction was necessary in 5 menbers (18.5%) of the younger group and 1 patient (7.7%) in the older group. There was 1 death due to respiratory failure and pneumonia. Abdominal pain was more frequent symptom and bilious vomiting was less frequent. Volvulus did not occur in the older group. Malrotation should be diagnosed promptly in children over 1 year of age by upper gastrointestinal series and abdominal ultrasonography even though symptoms are not as clear cut as in infants.

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[English]
Age-related Differences Effect the Clinical Characteristics of Intestinal Malrotation
Jong Jin Kim, Kyu Whan Jung, Tae Jin Park, Sung Eun Jung, Kwi Won Park
J Korean Assoc Pediatr Surg 2009;15(2):121-131.   Published online December 31, 2009
DOI: https://doi.org/10.13029/jkaps.2009.15.2.121

Intestinal malrotation presents with different clinical characteristics, depending upon the age of the patient. The medical records of 44 patients treated for intestinal malrotation with/without midgut volvuls between January 2002 and August 2009 at Seoul National University Children's Hospital were reviewed retrospectively. Patients were grouped by the age criteria of 1 and 12 months. Fourteen patients were under 1 month of age (31.8%), 9 patients between 1 month and 12 months (20.5%) and 21 patients over 12 months (47.7%). Twenty patients (45.5%) presented with volvulus. Vomiting (66%) and abdominal pain (20%) were the most common symptoms. UGIS (68.2%) was the most frequent diagnostic tool. Mean postoperative hospital stay was 13.7 days. There were 7 mild postoperative complications. Volvulus was more frequent in patients under 1 month (p=0.025) than over 1 month. The interval between diagnosis and operation was shorter in patients under 1 month (p=0.003) than in patients over one month of age. In the age between under and over 12 months, volvulus was more common in those under 12 months of age, but the difference was not significant. The interval from diagnosis to operation was shorter in patients under 12 months than over 12 months of age (p=0.001). Vomiting was the most frequent symptom in patients under 12 months. On the other hand, abdominal pain was the most frequent symptom in patients over 12 months.

In conclusion, patients with intestinal malrotation had age-related differences in the presence of midgut volvulus, the interval between diagnosis and operation, and clinical symptoms. The age of the patient should be considered in order to determine adequate treatment of malrotation.

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[English]
Clinical Study of Midgut Volvulus
Si Youn Rhim, Poong Man Jung
J Korean Assoc Pediatr Surg 2007;13(1):30-36.   Published online June 30, 2007
DOI: https://doi.org/10.13029/jkaps.2007.13.1.30

Intestinal malrotation is usually asymptomatic and most often is found during abdominal exploration for other surgical diseases. However, a serious complication of intestinal malrotation, midgut volvulus is a true surgical emergency of childhood. The clinical findings, diagnostic procedures, treatment, and prognosis of midgut volvulus were reviewed by a retrospective study. Between 1980 and 2005, 29 patients with midgut volvulus ranging in age from 1 day and 15 years were treated at HanYang University Hospital. Seventy-nine percent of the patients presented before 1 month of age. Midgut volvulus occurred 2 times more frequently in male. The clinical findings were bilious vomiting (96.6 %), irritability (34.5 %), abdominal distention (13.8 %), abdominal pain (10.3 %), and palpable abdominal mass (6.9 %). The diagnosis was made by abdominal simple x-ray (17.2 %), upper gastro-intestinal contrast study (37.9 %), abdominal sonogram (20.7 %), abdominal CT (3.4 %), and abdominal exploration (20.7 %). Among the 29 patients, 5 patients developed gangrene of small intestine due to strangulation and underwent resection of bowel. Two patients died due to sepsis.

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[English]
Acute Left-sided Appendicitis with Intestinal Malrotation in a Child
Hak Hoon Jun, Suk Woo Son
J Korean Assoc Pediatr Surg 2006;12(2):257-261.   Published online December 31, 2006
DOI: https://doi.org/10.13029/jkaps.2006.12.2.257

Appendicitis is a common disease in children. But left lower abdominal pain in acute appendicitis is a rare clinical feature. A 6 year-old-girl complained of left sided abdominal pain for 2 days. Past medical history was not contributory. Abdominal tenderness and guarding in left lower quadrant were noticed. Abdominal sonography and abdominal computed tomography scan demonstrated reversed position between superior mesenteric artery and vein, and a mass in the left lower quadrant abdomen suggesting appendicitis. Acute appendicitis in left lower quadrant, associated with intestinal malrotation, was found at laparatomy.

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