Case Report An Unusual Case of an Abdominal Mass in a 3 Year Old Charlene Antony, MSc, MD Candidate, Institute of Medical Science, Faculty of Medicine, University of Toronto, Toronto, Ontario Michael G. DeGroote School of Medicine, McMaster University, Hamilton, Ontario, Canada Andrew Latchman, MD, FRCPC, Division of General Pediatrics, McMaster Children’s Hospital Assistant Professor, Department of Medicine, McMaster University, Hamilton, Ontario Mark Walton, MD, FRCSC, Division of Pediatric General Surgery, McMaster Children’s Hospital, Hamilton, Ontario Assistant Dean, Post Graduate Medical Education, McMaster University, Hamilton, Ontario Abstract An abdominal mass in a young child is a significant concern that requires urgent assessment. The differential diagnosis is often initially focused on malignancies including Wilm’s tumor, hepatoblastoma, neuroblastoma, and lymphoma. We present an unusual case of an abdominal mass in a 3 year old boy who clinically presented with post-prandial emesis and increasing fatigue. An upper GI series confirmed the presence of a gastric bezoar, which upon surgical removal was found to be composed of human hair and carpet fabric. His laboratory studies had revealed a severe iron-deficiency anemia, which required a transfusion prior to surgery. In addition, the child also presented with associated fine motor and language delays. This case highlights the importance of primary prevention of iron deficiency amongst infants and toddlers. Introduction I ron deficiency (ID) is the single most common nutritional deficiency globally, with resulting anemia affecting approximately 750 million children worldwide.1 In developed countries, risk factors associated with iron deficiency anemia (IDA) include high consumption of cow’s milk before 12 months of age, prolonged exclusive breastfeeding, and chronic infection.1 Corresponding Author: Charlene Antony University of Toronto Faculty of Medicine 1 King’s College Circle Toronto, Ontario Email: [email protected] 140 Pica is often an associated symptom of IDA, occurring in up to 58% of these patients.2 Ingested substances typically include ice chips, clay, and starch; however, we present a case of chronic hair consumption that resulted in the formation of a trichobezoar. Trichobezoars are concretions of mostly hair, which accumulate in the gastrointestinal tract. They are often associated with underlying psychiatric disorders, and most commonly present in the female adolescent population.3 Rapunzel syndrome is an unusual and rare form of trichobezoar extending into the small intestine, which can be fatal as it can cause abdominal obstruction, which can progress to perforation. Here, we present a rare case of Rapunzel syndrome within a 3 year old boy secondary to pica associated with IDA. CASE A 3 year old boy, presented to the ER with a four week history of post-prandial emesis and progressive fatigue. Although he had minimal tolerance for solids, he was able to consume 27-36 ounces (800-1064 mL) of homogenized milk daily. He also had a nine month history of ingesting hair and fabric off the carpet but had maintained regular bowel moments. The child was born full-term and had been breastfed up to 8 months, at which point he was transitioned to homogenized cow’s milk. Developmentally, he demonstrated delays in fine motor skills such as an inability to take off his shoes or socks. In addition, he had gross delays in language development with only 10 words in his vocabulary up to this point, and would communicate largely through pointing. All other developmental parameters were within normal limits. His physical examination was significant for a non-tender palpable mass in the epigastric area. An abdominal x-ray revealed an increased opacity within the stomach, which was later confirmed with an upper GI series to likely be a bezoar. Laboratory investigations revealed a severe hypochromic microcytic anemia with a hemoglobin level of 63 g/L and mean corpuscular volume of 52.4 as well as low ferritin levels. He was subsequently transfused and a gastrostomy was performed, which revealed a bezoar weighing 412 g, that was largely composed of hair and coloured yarn (Figure 1). The trichobe- 65.+t7PMVNF/VNCFS.BZ Case Report An Unusual Case of an Abdominal Mass in a 3 Year Old zoar had conformed to the entire shape of the stomach and had a tail that extended into the proximal duodenum, as seen in Rapunzel syndrome. The patient was subsequently started on an iron supplement, and counseled on iron-rich foods as well as restricted on milk intake. Active measures to aid in his developmental progress were also undertaken. Figure 1. A large trichobezoar (15 cm x 8 cm x 5 cm) casting the shape of the stomach and the proximal duodenum is removed via gastrostomy. 65.+t7PMVNF/VNCFS.BZ Discussion Iron is a vital micronutrient that is used by virtually every cell and organ system in the body. As a consequence, ID leads to anemia but also has profound effects on cognitive and behavioural performance, physical growth and immune function, and can even result in death.4 Recent studies have also shown that children who have had a stroke are ten times more likely to be iron deficient.5 Of great concern, however, is the fact that several studies have demonstrated a consequence of cognitive and motor delay in infants and children with IDA. Decreased iron stores in the brain may hinder the synthesis, functioning and degradation of neurotransmitters such as serotonin, noradrenaline, and dopamine, thus impacting the overall development of these children.6 Extensive reviews exploring the effect of IDA have concluded that these children had poorer cognitive scores, delays in acquisition of fine motor skills and an overall lower scholastic achievement.7,8,9 However, possible confounding factors such as low socioeconomic status, lack of stimulation in the home, low maternal IQ, low birth weight and undernutrition prevent causal inferences from being made.8 Unfortunately, the efficacy of treatment for IDA is still under much debate, as a large number of randomized controlled trials have shown little convincing evidence that elemental iron can improve the psychomotor development scores of IDA children under or over the age of two.9 This indicates, that the developmental consequences of IDA may remain largely irreversible or are compounded with other factors associated with the condition. Thus, primary prevention becomes paramount. The avoidance of the sequelae of ID begins in utero as the mother’s intake of dietary iron affects the iron stores present in the baby at birth. As full-term infants, iron stores typically last from ages four to six months after which, the introduction of ironfortified cereals, including foods enriched with vitamin C to improve iron absorption, are strongly encouraged. Pre-term and low-birth weight infants have even lower stores of iron and are at an even greater risk of developing IDA sooner.10 Moreover, it is important to counsel families on avoiding homogenized milk for a baby younger than 12 months old, as it impairs iron absorption.1,10 Limitations on daily milk intake of less than 24 ounces (710 mL) should also be advised.11 Our patient had been introduced to homogenized milk at eight months of age. This was further compounded by a large intake of milk and a corresponding decrease in his intake of iron-containing solids. As his iron stores became deplete and resulted in anemia, his associated pica for human hair and yarn resulted in intestinal obstruction. As a consequence, he required a blood transfusion to raise his hemoglobin level and an operation to remove the gastric bezoar. He also had fine motor and language delays, which may be secondary to 141 Case Report An Unusual Case of an Abdominal Mass in a 3 Year Old the IDA. This case serves to highlight the importance of dietary screening in the anticipatory guidance for infants and toddlers with the goal of prevention of IDA and its many complications. References 1. Christofides A, Schauer C, Zlotkin SH. Iron deficiency anemia among children: Addressing a global public health problem within a Canadian context. Paeditr Child Health 2005; 10(10):597-601. 2. Mark AM. “Tomatophagia” and iron-deficiency anemia. NEJM 1999; 341:60–1. 3. Gonuguntla V, Joshi DD. Rapunzel Syndrome: A comprenhensive review of an unsual case of trichobezoar. Clin Med & Research 2009; 7(3):99-102. 4. Ghosh K. Non hematological effects of iron deficiency- a perspective. Ind J Med Science 2006; 60:30-7. 5. Maguire JL, deVeber G, Parkin PC. Association between iron-deficiency anemia and stroke in young children. Pediatrics 2007; 120:1053-7. 142 6. Dallman PR. Biochemical basis for the manifestation of iron deficiency. Annu Rev Nutr 1986; 6:13-40. 7. Martins S, Logan S, Gilbert RE. Iron therapy for improving psychomotor development and cognitive function in children under the age of three with iron deficiency anemia. Cochrane Database of Systemic Reviews, 2001(Issue 2). 8. Grantham-McGregor S, Ani C. A review of studies on the effect of iron deficiency on cognitive development in children. J Nutr 2001; 131:649S-68S 9. Sachdev HPS, Gera T, Nestel P. Effect of iron supplementation on mental and motor development in children: systemic review of randomized controlled trials. Public Health Nutrition 2004; 8(2):117-32. 10. Earl R, Woteki CE éd. Iron deficiency anemia: recommended guidelines for the prevention, detection, management among U.S. children and women of childbearing age. Washington DC: National Academy Press, 1993. 11. Centers for Disease Control and Prevention. Recommendations to prevent and control iron deficiency in the United States. Morb Mortal Wkly Rev 1998; 47(RR-3):1–29. 65.+t7PMVNF/VNCFS.BZ
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