A 6-year-old boy with a history of DiGeorge syndrome, severe gastroesophageal reflux disease, and chronic constipation underwent a laparoscopic Nissen fundoplication in November 2008. Soon after surgery, and despite adequate dosing of Miralax and lansoprazole, the patient’s parents noted abdominal discomfort and fullness. In early February 2009, the patient was admitted to an outlying hospital with abdominal distention and suspected fecal impaction. He underwent a colonic lavage with polyethylene glycol for 6 days and was discharged home. However, over the course of the following week, the patient again developed abdominal distention and peri-umbilical pain. Abdominal X-rays showed centralization of bowel loops on the supine view, suggestive of ascites. Subsequent abdominal ultrasound and a computed tomography (CT) scan confirmed a large amount of ascites, and he was admitted to Lucile Packard Children’s Hospital at Stanford for further evaluation. On admission, his pertinent physical examination findings included a small chin and mouth typical of DiGeorge syndrome, no appreciable murmur, abdominal fluid wave, general discomfort on palpation of the abdomen with no masses or organomegaly, and dullness to percussion throughout the abdomen. Laboratory results upon admission were remarkable for a white blood cell count of 4,700 mm and an albumin of 3.1 mg/dl. An ultrasoundguided paracentesis with placement of a peritoneal drain was performed under general anesthesia. Peritoneal fluid cell count showed a white blood cell count of 5,320 mm (80% lymphocytes) and a red blood cell count of 314 mm. The peritoneal fluid concentrations of albumin and triglycerides were 2.5 g/dl and 1,295 mg/dl, respectively. Approximately 3 l of milky white chylous fluid was extracted by gravity within the first 24 h after placement of the drain. An magnetic resonance angiography (MRA)/ magnetic resonance imaging (MRI) of the abdomen that was obtained to outline the lymphatic system identified no specific site of lymphatic injury. Initially, the patient was treated with a low-fat diet, and 2 days later he was switched to a complete no-fat diet. In an attempt to further decrease the chylous drainage, a central line was placed and total parenteral nutrition (TPN) was initiated by the end of the first week of hospitalization. The drain output significantly decreased with the nil per os (NPO) and TPN therapy, and the peritoneal drain was discontinued on the 10th day of hospitalization (Fig. 1). The patient was discharged from the hospital after 13 days and was followed in the outpatient clinic every 1–2 weeks. Complete metabolic panels including albumin and prealbumin as well as abdominal ultrasounds were obtained with each outpatient appointment. Physical and K. T. Park (&) K. G. Sylvester J. A. Kerner Lucile Packard Children’s Hospital, Stanford, CA, USA e-mail: ktpark@stanford.edu
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Apr 22, 2013
Matthew Seidler + 3
Open Access