**Background:** Essential fatty acid deficiency (EFAD) is a serious condition that can develop rapidly in premature infants who do not receive adequate lipid intake. Consequences include elevated transaminases, thrombocytopenia, poor wound healing, scaly dermatitis, hypertriglyceridemia (HTG), growth failure, and adverse effects on brain development. This case report discusses neonatal lipid management and its relationship with EFAD, HTG, and intestinal failure-associated liver disease (IFALD) in a premature infant with short bowel syndrome who developed severe EFAD secondary to intravenous lipid emulsion (ILE) dose restriction.
**Methods:** This is a case report of an infant born at 23 weeks gestational age who was transferred at 2 weeks of age for renal failure, septic shock, and necrotizing enterocolitis. At 4 weeks of age, the infant underwent laparotomy for bowel perforation with necrosis. Postoperative complications included respiratory failure, IFALD, postnatally acquired cytomegalovirus (CMV), chronic lung disease, bilateral intraventricular hemorrhages (grades 2 and 3), periventricular leukomalacia, and growth failure. Parenteral nutrition was started on admission and advanced (glucose infusion rate 13 mg/kg/min, amino acid 4 g/kg/d, 84 kcal/kg/d). ILEs were held for 12 days after admission due to limited vascular access and fluid restriction from renal failure. At 3.5 weeks of age, a mixed oil ILE with 15% fish oil (SMOFlipid) was prescribed at 0.5 g/kg/d for 4 days. At 5 weeks of age, a single dose of 3.5 g/kg of this mixed oil ILE was given due to concern for HTG (triglyceride range 148–563 mg/dL). The infant remained nil per os.
**Key Results:** At 5 weeks of age, the infant was diagnosed with severe EFAD, hallmarked by a high triene:tetraene ratio (T:T) of 1.3 (reference 0.013–0.050), elevated transaminases (AST 247 U/L, ALT 105 U/L), thrombocytopenia (platelets 53 × 10^3/uL), poor healing, dry skin, and growth failure (weight 811 g, z-score –1.2; length 32 cm, z-score –1.8; head circumference 20.6 cm, z-score –1.8). One hundred percent fish oil ILE (Omegaven) dosed at 1.5 g/kg/d was prescribed. By 21 weeks of age, after 16 weeks of 100% fish oil ILE, the T:T normalized to 0.022, transaminases normalized (AST 49 U/L, ALT 41 U/L), conjugated bilirubin decreased to 1.8 mg/dL, and platelet count increased to 164 × 10^3/uL. However, growth failure persisted (weight 3025 g, z-score –2.4; length 46 cm, z-score –3.9; head circumference 31 cm, z-score –4.5). Linoleic acid remained low (640 nmol/mL; reference 1000–3300), arachidonic acid remained low (24 nmol/mL; reference 110–1110), while docosahexaenoic acid (739 nmol/mL; reference 10–220) and eicosapentaenoic acid (820 nmol/mL; reference 2–60) were elevated. At 20 weeks of age, the infant underwent laparotomy for lysis of adhesions, jejunal stricture resection, and jejunoileostomy. Parenteral nutrition was discontinued 4 weeks later. The infant was treated with ganciclovir/valganciclovir for 6 months for CMV.
**Clinical Implications:** This case demonstrates that withholding ILE or using suboptimal doses in premature infants can rapidly lead to severe EFAD, which exacerbates HTG and IFALD. The practice of ILE dose restriction is not appropriate for premature infants; consistent, adequate lipid dosing is essential. A 100% fish oil ILE at 1.5 g/kg/d safely and effectively treated HTG, EFAD, and IFALD in this patient, although growth failure and microcephaly persisted due to multiple comorbidities. The case underscores the need for careful monitoring of fatty acid profiles and individualized lipid management in high-risk neonates.