European Journal of Clinical Nutrition (2014), 1–3 © 2014 Macmillan Publishers Limited All rights reserved 0954-3007/14 www.nature.com/ejcn

CLINICAL CASE REPORT

Congenital cataracts following total parenteral nutrition (TPN) use during pregnancy N Heerasing and D Dowling We describe a case of congenital cataracts in a newborn whose mother received total parenteral nutrition (TPN) throughout her pregnancy. We discuss the potential mechanisms by which TPN may have been causally linked to cataract formation. European Journal of Clinical Nutrition advance online publication, 28 May 2014; doi:10.1038/ejcn.2014.103

BACKGROUND Adequate maternal nutrition is essential for optimal fetal growth. Maternal malnutrition is associated with intrauterine growth retardation and increased perinatal morbidity and mortality. Total parenteral nutrition (TPN) is indicated for any pregnancy where oral intake or enteral feeding is unable to maintain adequate maternal nutrition.1 The successful use of TPN in women with the new onset of nutritional disorders (that is, hyperemesis gravidarum) during pregnancy is widely documented.1 However, there is minimal literature regarding pregnancy in women maintained on long-term TPN before conception.2–7 CASE REPORT Ms AB was a 20-year-old nulliparous woman with short gut syndrome secondary to superior mesenteric artery avulsion at the time of a motor vehicle accident. Residual small bowel included the duodenum and proximal jejunum only (Figure 1). Before her pregnancy, she had been on home TPN for 4 years receiving TPN 4 nights weekly via a Hickman’s line. Her medical history included chronic back pain and a right renal calculus. Her regular medications included calcium, zinc, vitamin E and vitamin D supplements. Her TPN included amino acids 100 g, carbohydrates 250 g, routine trace elements (as per Australian parenteral enteral nutrition guidelines) with total calories estimated to be 1450 kcal. Owing to increasing energy requirements in pregnancy, Ms AB received nocturnal TPN 5 nights per week throughout the pregnancy and additional intravenous magnesium weekly. Liver function tests, electrolytes, renal function, iron studies and serum B12, folate and vitamin D were monitored regularly. Ms AB gained ~ 13 kg during her pregnancy and this is in line with the average weight gain during pregnancy, which is estimated to be between 11.5 and 16 kg. Fetal size throughout the pregnancy was monitored with serial ultrasound examinations. All daytime blood glucose measurements were normal but nocturnal blood glucose levels were not monitored. The glycated haemoglobin level was not done in this case. Ms AB smoked heavily throughout the pregnancy. TPN use was complicated by one episode of line sepsis at week 37 of pregnancy, which was treated with intravenous antibiotics with conservation of the Hickman’s catheter.

Ms AB had an uncomplicated planned elective lower uterine caesarean section at 39 weeks pregnant. The infant weighed 2700 g and was estimated to be at the 10th percentile for weight (Figure 2). During the first day post delivery, the male neonate was diagnosed with bilateral congenital total cataracts. The infant was referred for paediatric ophthalmology opinion. Laboratory studies included complete blood count, blood urea nitrogen, TORCH (toxoplasmosis, rubella, cytomegalovirus and herpes simplex) titres, Venereal Disease Research Laboratory test, urine for reducing substances, red cell galactokinase, amino acids, calcium and phosphorus. Those tests were essentially normal. The newborn screening test for galactosaemia was negative. Molecular genetic testing for mutations in the 100+ lens genes was not performed. DISCUSSION Successful pregnancy in patients requiring long-term TPN before conception has been reported, in the English literature, on only six prior occasions. Tresadern et al.2 reported the first published case of a patient maintained on TPN from conception to delivery in 1982. This involved a 31-year-old woman with severe Crohn’s disease who became pregnant 6 months after commencing TPN for treatment of malnutrition. A healthy infant weighing 2620 g was born at 37 weeks via normal vaginal delivery. Yoshifumi et al.3 reported a successful pregnancy in a 33-year-old woman with short bowel syndrome due to the occlusion of the superior mesenteric artery who was maintained on TPN. She had no obstetric complications, and during the pregnancy there was no alteration to her nutrition regimen except adding iron intravenously to treat anaemia. Campo et al.4 described the case of a 30-year-old woman with chronic intestinal pseudo-obstruction (CIP), on TPN for longer than 6 years, who became pregnant and delivered a healthy near-term boy. There were no metabolic complications during the pregnancy and the patient only needed slight modifications to her parenteral nutrition regimen during lactation. Successful pregnancy in a woman with Crohn's disease and short bowel syndrome maintained on long-term TPN was reported by Nugent et al.5 Fetal development was normal, and a healthy, full-term infant was delivered. Elchlal et al.6 described a case whereby a 25-year-old woman, who had been on TPN since

Department of Gastroenterology, Geelong Hospital, Geelong, Victoria, Australia. Correspondence: Dr N Heerasing, Department of Gastroenterology, Corner Bellerine Street and Ryrie Street, Barwon Health, Geelong, Victoria 3220, Australia. E-mail: [email protected] Received 6 January 2014; revised 12 April 2014; accepted 17 April 2014

TPN and congenital cataracts N Heerasing and D Dowling

2 infancy due to CIP, achieved a successful pregnancy that was complicated by polyhydramnios, premature uterine contractions and an emergency caesarean section at 33 weeks.6 The infant had normal Apgar scores and progressed well. A case of normal gestation in a 30-year-old woman who had been on TPN for 5 years following resection of her entire jejunum, ileum and right colon secondary to gangrene was reported by Wu et al.7 TPN needed adjustment in the third trimester to account for anaemia, hypoalbuminaemia, low zinc levels and jaundice. A healthy baby was delivered via caesarean section at 36 weeks. These cases illustrate that pregnancy in women on long-term TPN can be achieved through strict medical and nutritional support. There has been no previous report suggesting a link between TPN during pregnancy and the development of congenital cataracts. Congenital cataracts occur in 3–4 per 10 000 births.

Figure 1. Barium follow through of the patient in this case shows that she has only a small length of small bowel, which includes the duodenum and proximal jejunum only.

In most patients, no cause can be found. Some rare causes include hypoglycaemia, trisomy (for example, Down, Edward and Patau syndromes), myotonic dystrophy, infectious diseases (for example, TORCH) and prematurity. Prakalapakorn et al.8 in a case control study reported low birth weight to be associated with both bilateral and unilateral congenital cataracts. In the case we report, the neonate was at the lower end of the normal birth weight range. Potential explanations for the neonate’s weight include maternal smoking during the pregnancy, unrecognised daytime hypoglycaemia and inadequate TPN caloric content, although this latter factor is unlikely as maternal weight gain during pregnancy was within the expected range. Animal studies report an increased risk of congenital cataracts associated with maternal hyperglycaemia. Roversi and Giavini9 reported a high incidence of cataracts in infant rats born to diabetic mothers. The suggested pathogenetic mechanism is precipitated by fetal hyperglycaemia and involves the following steps: (1) a high glucose concentration in the lens; (2) reduction of glucose to sorbitol by aldose reductase; (3) accumulation of sorbitol into the fibres of the lens creating a hyperosmotic effect, leading to (4) an infusion of liquid into the fibres, which (5) becomes hydropic and degenerate with vacuolisation.9 The link between maternal diabetes and infantile cataracts has been the subject of only limited human studies. The study by Prakalapakorn et al. showed no evidence of an association between maternal gestational diabetes and infantile cataracts. During pregnancy the risk of TPN-associated hyperglycaemia is increased due to the increase in physiologic insulin resistance that occurs as the pregnancy progresses. Russo-Stieglitz et al.10 described a cohort of 26 women treated with TPN during pregnancy. Although no patient had pregestational diabetes, seven of the cohort required the initiation of insulin therapy in the later stages of pregnancy for treatment of TPN-related hyperglycaemia. In the case reported, nocturnal blood glucose levels (during TPN administration) were not monitored and the possibility of nocturnal hyperglycaemia cannot be excluded.

Figure 2. Graph representing fetal growth. The weight is represented by the red line and the green line represents the percentile. The infant was born at the 10th percentile, which is near the low birth weight range. The full colour version of this figure is available at European Journal of Clinical Nutrition online. European Journal of Clinical Nutrition (2014) 1 – 3

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TPN and congenital cataracts N Heerasing and D Dowling

3 Successful pregnancy can occur in the setting of home TPN. This is the first report of congenital cataracts associated with TPN in pregnancy. Although no definite link between TPN and cataracts can be suggested, it is possible that TPN-related abnormalities of glycaemic control may have contributed to the cataracts. CONFLICT OF INTEREST The authors declare no conflict of interest.

REFERENCES 1 Badgett T, Feingold M. Total parenteral nutrition in pregnancy: case review and guidelines for calculating requirements. J Maternal Fetal Med 1997; 6: 215–217. 2 Tresadern JC, Falconer GF, Turnberg LA, Irving MH. Successful completed pregnancy in a patient maintained on home parenteral nutrition. Br Med J 1983; 286: 602–603. 3 Yoshifumi I, Akihisa T, Yoshihiro H. Successful pregnancy in a patient with short bowel syndrome maintained on long-term home parenteral nutrition from conception to delivery. Jpn J Surg Metab Nutr 2006; 6: 277–282.

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4 Campo M, Albinana S, Garcia-Burguillo A, Moreno JM, Leo Sanz M. Pregnancy in a patient with chronic intestinal pseudo-obstruction on long-term parenteral nutrition. Clin Nutr 2000; 19: 455–457. 5 Nugent FW, Rajala M, O’Shea RA, Kolack PF, Hobin MA, Haimes MK et al. Total parenteral nutrition in pregnancy: conception to delivery. JPEN J Parenter Enteral Nutr 1987; 11: 424–427. 6 Elchlal U, Selaa HY, Gimmon Z. Defying physical limitations: Successful pregnancy and birth in a patient on home total parenteral nutrition since infancy. Eur J Obstet Gynecol Reprod Biol 2009; 147: 111–115. 7 Wu ZH, Huang YW, Zhang W, Wu ZG. Normal gestation after 5 years on home parenteral nutrition. Clin Nutr 1993; 12: 43–46. 8 Prakalapakorn SG, Rasmussen SA, Lambert SR, Honein MA. Assessment of risk factors for infantile cataracts using a case-control study, National Birth Defects Prevention Study, 2000–2004. Opthalmology 2010; 117: 1500–1505. 9 Roversi GD, Giavini E. Damage to the crystalline lens in infants of diabetic mothers: a pathology so far neglected? Opthalmologica 1992; 204: 175–178. 10 Russo-Stieglitz KE, Levine AB, Wagner BA, Armenti VT. Pregnancy outcome in patients requiring parenteral nutrition. J Matern-Fetal Med 1999; 8: 164–167.

European Journal of Clinical Nutrition (2014) 1 – 3

Congenital cataracts following total parenteral nutrition (TPN) use during pregnancy.

We describe a case of congenital cataracts in a newborn whose mother received total parenteral nutrition (TPN) throughout her pregnancy. We discuss th...
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