Left Upper-Quadrant Appendicitis in a Patient with Congenital Intestinal Malrotation and Polysplenia

Abstract

Patient: Female, 13Final Diagnosis: Left upper quadrant appendicitisSymptoms: Left upper quadrant abdominal painMedication: —Clinical Procedure: Laparoscopic ladd’s procedureSpecialty: SurgeryObjective:Congenital defects/diseasesBackground:Appendicitis is the most common cause of abdominal pain requiring emergent surgical intervention. Although typically presenting as right lower-quadrant pain, in rare cases it may present as left upper-quadrant pain secondary to abnormal position due to intestinal malrotation. Since atypical presentations may result in diagnostic and management delay, increasing morbidity and mortality, accurate and prompt diagnosis is important. Therefore, acute appendicitis should be considered in the differential diagnosis of left upper-quadrant abdominal pain. In this setting, medical imaging plays a key role in diagnosis. We report a case of a 13-year-old female with undiagnosed intestinal malrotation presenting with left-sided acute appendicitis.Case Report:A 13-year-old Hispanic female presented at the emergency room with anorexia and left upper-quadrant abdominal pain with involuntary guarding. The laboratory work-up was remarkable for elevated white blood cell count and elevated erythrocyte sedimentation rate. A nasogastric tube was placed and abdominal x-rays performed to rule-out bowel obstruction, showing distended bowel loops throughout all abdominal quadrants, with sigmoid and proximal rectal gas, raising concern for ileus rather than an obstructive pattern. Lack of symptomatic improvement prompted an IV contrast-enhanced abdominopelvic CT, revealing intestinal malrotation and with an inflamed left upper-quadrant appendix. Surgical management proceeded with a laparoscopic Ladd’s procedure.Conclusions:Acute appendicitis may present with atypical symptoms due to unusual appendix locations, such as in malrotation. Most cases are asymptomatic until development of acute complications, requiring imaging for diagnosis. Clinicians and radiologists should have a high index of suspicion and knowledge of its clinical presentations to achieve early diagnosis and intervention.

An uncommon cause of well-known symptoms: acute abdomen in a 2-year-old boy with intestinal malrotation. Case report and literature review

“…Ultrasonography of the abdomen has almost 100% sensitivity in detecting neonatal malrotation and was suggested to be a good screening tool for effectively ruling out malrotation at risk of volvulus [10]. It shows the inversion of the SMA and superior mesenteric vein (SMV), which can be also revealed in the computed tomography study [11][12][13]. Additionally, USG may show dilated bowel loops which was the only ultrasound finding present in the present case.…”

A Rare Case of Congenital Intestinal Malrotation Presented as an Intestinal Obstruction in an Adult

“…Intestinal malrotation is a congenital anomaly that causes the duodenojejunal junction to be positioned in the right middle during development by rotating the gut around the superior mesenteric artery axis. Although it has early-onset symptoms, a few individuals (0.1-0.5%) live until adulthood with no symptoms [ 1 ]. The prevalence of cases reported is one in 6000 and one in 200 of all live births, with most cases occuring in the first month of life and 90% within the first year.…”

“…The prevalence of cases reported is one in 6000 and one in 200 of all live births, with most cases occuring in the first month of life and 90% within the first year. Due to the vague nature of malrotation in adolescence and adulthood, the radiologist may be the first to arrive at this crucial diagnosis because of an accidental imaging discovery or as the reason for unexpected abdominal symptoms [ 1 , 2 ]. Several congenital obstructive defects, such as duodenal atresia and stenosis, are related to intestinal malrotation.…”

“…Although it has early-onset symptoms, a few individuals (0.1-0.5%) live until adulthood with no symptoms [1]. The prevalence of cases reported is one in 6000 and one in 200 of all live births, with most cases occuring in the first month of life and 90% within the first year.…”