AIDS-associated cholangiopathy is a form of biliary tract inflammation with stricture formation seen in AIDS patients who are severely immunosuppressed. It is no longer common in countries in which HAART therapy is widely employed but is still seen in underdeveloped countries. The majority of patients are symptomatic at the time of presentation. Herein, we describe a seventy-four-year-old woman who presented with unilateral leg swelling after a prolonged airplane flight. She was otherwise entirely asymptomatic. Routine laboratory testing was notable for a hypochromic microcytic anemia, slight leukopenia, and mild hypoalbuminemia. Liver enzymes were all elevated. Deep venous thrombosis was confirmed, and a CT scan of the chest disclosed no pulmonary emboli. However, the visualized portion of the abdomen showed dilatation of the common bile and pancreatic ducts. This was confirmed on ultrasonography and MRCP, and no obstructive lesions were noted. An ERCP revealed a dilated common bile duct without filling defects or strictures. A balloon occlusion cholangiogram showed strictures and beading of the intrahepatic ducts. Shortly thereafter, serology for HIV returned positive along with a depressed CD4 cell count, and the patient was diagnosed with AIDS-associated cholangiography.
Rationale:There is a high prevalence of obstructive sleep apnea (OSA) in patients with Pierre Robin sequence (PRS), and treatment approaches are highly variable. One approach is a temporary tongue-lip adhesion (TLA) that acts as a temporizing measure while the mandible continues to grow and is usually taken down at 1 year of age.Patient concerns:Side effects of prolonged tongue-lip adhesion and optimal workup and treatment of persistent OSA in the setting of a tongue-lip adhesion.Diagnoses:Pierre Robin sequence (PRS), persistent obstructive sleep apnea (OSA), and tongue-lip adhesion (TLA).Interventions:Mandibular distraction osteogenesis (MDO), adenotonsillectomy, and tongue-lip adhesion takedown.Outcomes:Resolution of OSA.Lessons:This case puts into question the efficacy of isolated TLA in infants with Pierre Robin sequence and OSA, and places emphasis on the importance of considering an earlier workup of other potential causes of obstruction and the potential need for MDO as a primary or adjunctive approach to treatment.
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