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Tracheal agenesis: approach towards this severe diagnosis. Case report and review of the literature.

ABSTRACT: Tracheal agenesis (TA) is a severe congenital disorder with often an unexpected emergency presentation. There is complete or partial absence of the trachea below the larynx, with presence or absence of a tracheoesophageal fistula (TOF). A neonate with TA is described, and another 48 cases found in literature are reviewed. Due to absence of a TOF, five cases were diagnosed prenatally because of congenital high airway obstruction syndrome (CHAOS). When a TOF is present, polyhydramnion and several other congenital malformations seen on the ultrasound examination should alert clinicians of potential tracheal problems. Prenatal magnetic resonance imaging (MRI) may provide a definitive diagnosis. Postnatal diagnosis is based on recognition of specific clinical signs in the newborn with TA: respiratory distress with breathing movement without appropriate air entry, no audible cry, and failed endotracheal intubation. Despite progress in surgical interventions, mortality remains high. Prenatal diagnosis of TA is possible, but only if a TOF is absent resulting in CHAOS. Prenatal diagnosis of polyhydramnion and other congenital malformation should alert clinicians of potential tracheal problems. Prenatal MRI may provide a definitive diagnosis.

SUBMITTER: de Groot-van der Mooren MD

PROVIDER: S-EPMC3284653 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:Peripartum pancreatitis is a rare clinical condition that occurs usually in the third trimester of pregnancy. Pancreatitis is usually secondary to gallstones, and it can lead to life-threatening and rare complications. We report a case of necrotizing postpartum pancreatitis that developed abdominal compartment syndrome (ACS) in early course, posterior reversible encephalopathy syndrome (PRES), and splanchnic and extrasplanchnic thrombosis later on. Case. 31-year-old female, one week after delivery, presented to the emergency department with abdominal pain, nausea and vomiting, tenderness in the epigastrium, and raised pancreatic enzymes. Ultrasound (USG) showed bulky pancreas with gallstones. She was diagnosed as having acute biliary pancreatitis and started to be hydrated and was supplemented with analgesia. Her condition deteriorated on the 2nd day, and she was shifted to the surgical intensive care unit (SICU) where she developed abdominal compartment syndrome (ACS), respiratory distress, and acute kidney injury, requiring endotracheal intubation and ventilation. Computerized tomography (CT) showed pancreatic necrosis with multiple fluid collections and significant left-sided pleural effusion. Percutaneous drainage of pleural effusion was done, and she was stabilized to be weaned off from mechanical ventilation. On day 15, she underwent USG-guided drainage of the pancreatic collection and ERCP (endoscopic retrograde cholangiopancreatography) on day 19. Post-ERCP, she had tonic colonic convulsions which were treated with benzodiazepines and phenytoin. It was diagnosed by imaging studies as posterior reversible encephalopathy syndrome (PRES). Her abdomen was still distended and tender; CT showed a significant pseudocyst with splanchnic and extrasplanchnic thrombosis. She had laparotomy, gastrocystostomy, and cholecystectomy on day 28th. She made uncomplicated recovery and discharged in good health. Conclusion. Peripartum pancreatitis can be complicated by ACS, PRES, and splanchnic and extrasplanchnic thrombosis.

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Tracheal agenesis: approach towards this severe diagnosis. Case report and review of the literature.

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