Neurofibromatosis Type I Presenting with Incomplete Ileal Volvulus in a Pediatric Patient.

BACKGROUND Neurofibromatosis 1 is a neurocutaneous disorder with multisystemic manifestations. When patients are lacking overt cutaneous manifestations, diagnosis may be delayed and may complicate diagnosis and management of atypical presentations of this disease. It is thus important to strive to obtain relevant and/or complete history to arrive at the appropriate diagnosis. Furthermore, maintaining an index of suspicion in cases of vague abdominal pain may guide the clinician in establishing the correct diagnosis of mesenteric plexiform neurofibroma in the setting of known/presumed neurofibromatosis 1 patients presenting with acute and/or chronic vague abdominal symptoms. CASE REPORT This is a case of a teenage boy who presented with acute, vague abdominal pain over a period of 2 weeks. Laboratory tests and physical exam findings in primary and secondary care settings were unremarkable, and thus the patient was discharged home only to continue with abdominal pain, thus seeking additional medical care. After admission to our facility and exhaustive history taking, physical examination, and imaging, a prospective diagnosis of neurofibromatosis with mesenteric neurofibroma was made. Upon surgical exploration, a mesenteric mass with corresponding volvulized, ischemic small bowel was removed. Histopathology confirmed a plexiform neurofibroma. The patient recovered adequately and was discharged home without complications. CONCLUSIONS This case highlights the importance of exhaustive history taking to obtain an accurate diagnosis as well as the importance of a high index of clinical suspicion for mesenteric neurofibromatosis in patients with presumed or known neurofibromatosis and presenting with vague abdominal symptoms.

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

BACKGROUND 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.

Silent Presentation of a Solid Pseudopapillary Neoplasm of the Pancreas.

BACKGROUND Solid pseudopapillary neoplasm (SPN) is a rare tumor frequently found in the head or tail of the pancreas. It mainly presents in young women between the 2nd and 3rd decades of life. A predilection for African Americans and Asians has been observed and is rarely reported in children. Most patients are symptomatic, with abdominal pain as the most common presenting symptom. Clinical laboratory test results are usually normal and pancreatic markers are not typically elevated. Metastatic disease is very uncommon, but most often metastasizes to the liver and regional lymph nodes. Prognosis is usually excellent after surgical resection. CASE REPORT We present the case of a 14-year-old Hispanic female who presented to the emergency department after a high-speed motor vehicle accident. She suffered multiple body traumas. Specifically, the patient referred severe epigastric pain. No significant past medical or surgical history was obtained. Laboratory workup was non-contributory. Further evaluation with abdomen and pelvis contrast-enhanced computed tomography and magnetic resonance imaging revealed a pancreatic tail mass. Distal pancreatectomy followed. Pathologic diagnosis of SPN was established. CONCLUSIONS SPN is a rare exocrine tumor with excellent prognosis following resection. Imaging findings are suggestive, but a pathology evaluation is necessary to make the final diagnosis. Differential diagnosis includes entities such as mucinous cystic pancreatic tumor, pancreatic ductal carcinoma, and pancreatic serous cystadenoma. Radiologists play a vital role in the diagnosis, since many times, as in our case, it presents as an incidental finding. A small percentage of SPN neoplasms are associated with metastasis or local recurrence. Therefore, the aim of our case presentation is to review key imaging findings to guide early management and surgical planning.