Superior Mesenteric Artery Syndrome and Nutcracker Syndrome as the Presentation of Crohn's Disease in a Young Patient: A Case Report and Review of Literature.

Superior mesenteric artery syndrome and nutcracker syndrome are rare vascular complications most often seen after marked weight loss caused by compression of the duodenum and left renal vein between the superior mesenteric artery and the aorta, respectively. The coexistence of superior mesenteric artery syndrome and nutcracker syndrome has been rarely reported. Herein, we present the case of a 16-year-old male with intermittent periumbilical abdominal pain, bilious vomiting, and weight loss who was found to have both of these vascular complications of significant weight loss as the initial presentation of Crohn's disease. This report provides insight into the diagnosis and treatment of these syndromes while highlighting the importance for practitioners to keep vascular complications on their differential diagnosis of vomiting and abdominal pain in patients with Crohn's disease.

Clinical presentations of erosive esophagitis found at endoscopy in neurologically impaired children: a historical study.

BACKGROUND: Data is lacking as to the clinical presentation of erosive esophagitis (EE) in neurologically impaired children compared to non-neurologically impaired children (non-NIC). To determinate the clinical presentation, associations, management, and outcomes of EE in neurologically impaired children compared to children without neurologic impairment. METHODS: Retrospective chart review of all esophagogastroduodenoscopies performed in pediatric patients at the University of Mississippi Medical Center from 1998 to 2020 with the diagnosis of EE. Fisher's exact test was used to compare results from neurologically impaired children group and non-NIC. A probability <0.05 was considered statistically significant. RESULTS: Forty-seven patients were diagnosed with EE and met study criteria. Twenty-six patients were neurologically impaired children, and 21 were non-neurologically impaired children. No significant difference was seen between age at diagnosis, sex, or hematologic markers of anemia. The most common indication for esophagogastroduodenoscopies in neurologically impaired children was hematemesis (65.4%), whereas abdominal pain (33.3%) was the most common in non-NIC. Neurologically impaired children were more likely to be treated with acid-blockade. Nine neurologically impaired children had gastrostomy tubes prior to diagnosis as opposed to 0 non-neurologically impaired children. After diagnosis, 8 neurologically impaired children underwent gastrostomy tube placement compared to 0 non-neurologically impaired children, and fundoplication was performed in 11 neurologically impaired children as compared to 1 non-NIC. The sensitivity of fecal occult blood test for detecting EE was higher for neurologically impaired children (91.7%) than for non-NIC (33.3%). CONCLUSIONS: EE in neurologically impaired children presents differently than in non-neurologically impaired children with blood loss being the most common presentation in neurologically impaired children. Neurologically impaired children are more likely to be treated with acid-blockade prior to diagnosis, likely due to heightened risk for gastroesophageal reflux disease (GERD). Additionally, they are more likely to undergo surgical management of EE than non-neurologically impaired children.

Cirrhosis complicating Shwachman-Diamond syndrome: A case report.

BACKGROUND: The features of Shwachman-Diamond syndrome (SDS) include exocrine pancreatic insufficiency, skeletal abnormalities and bone marrow dysfunction; an often overlooked feature is hepatic involvement. CASE SUMMARY: We report a child who initially presented with failure to thrive and mildly elevated transaminase levels and was determined to have pancreatic insufficiency due to SDS. During follow-up he had persistently elevated transaminase levels and developed hepatosplenomegaly. An investigation was performed to determine the etiology of ongoing liver injury, including a liver biopsy which revealed hepatic cirrhosis. CONCLUSION: Cirrhosis has rarely been reported with SDS. While many of the hepatic disorders associated with SDS improve with age, there are rare exceptions with serious implications for long-term outcome.