Foreign Body Ingestion in Children with Severe ... - Springer Link

Dysphagia 16:68–73 (2001) DOI: 10.1007/s004550000047 © Springer-Verlag New York Inc. 2001

Foreign Body Ingestion in Children with Severe Developmental Disabilities: A Case Study Sheena Reilly, BAppSc, PhD,1 and Lucinda Carr, MD, MBChB, DCH, MRCP, FRCPCH2 1

School of Human Communication Sciences, Faculty of Health Sciences, LaTrobe University, and Royal Children’s Hospital, Melbourne, Victoria, Australia. 2Neuro-disability Unit, Great Ormond Street Children’s Hospital and Institute of Child Health, London, England

Abstract. Dysphagia is common in children with severe developmental disabilities. The nature of these difficulties can predispose them to foreign body ingestion. This article presents a case that highlights the need for vigilance in diagnosing dysphagia in children with multiple and complex developmental disabilities where severe cognitive impairment and an inability to communicate may mask the presence of underlying problems. Key words: Dysphagia — Foreign body ingestion — Cornelia de Lange syndrome — Developmental disability — Deglutition — Deglutition disorders.

Children with severe developmental disabilities (SDD) present a challenge to pediatric services. They are likely to require access to specialist multidisciplinary dysphagia teams for management throughout infancy, childhood, and adolescence. Although there has been increasing recognition of the prevalence and nature of dysphagia in children with complex and multiple disabilities, problems can be overlooked, particularly when they are rarely the presenting feature. Foreign body ingestion (FBI) is a frequent and a potentially lethal occurrence in children [1]. While for many children ingested foreign bodies cause little trouble and pass through the gut uneventfully, in approximately 20%–30% of children, problems may occur [2]. Symptoms associated with FBI may include choking, coughing, wheezing, decrease in appetite, or refusal to eat. However, these symptoms may differ in severity and are

Correspondence to: Sheena Reilly, Ph.D., School of Human Communication Sciences, Faculty of Health Sciences, LaTrobe University, Melbourne, Victoria 3083, Australia. E-mail: [email protected]

sometimes absent. As a result, the diagnosis of FBI may be missed or delayed. We report on a male with Cornelia de Lange syndrome who was referred to our multidisciplinary pediatric dysphagia clinic when he was 5 years 2 months old. The child had previously been under the care of a craniofacial team since birth and was referred by his consultant plastic surgeon because of concern regarding feeding. Cornelia de Lange syndrome affects between 1:40,000 and 1:100,000 children. It is associated with severe intrauterine growth retardation, resulting in short stature and moderate to severe cognitive impairment. A number of other congenital anomalies are associated with the syndrome including typical orofacial features such as bushy eyebrows and synophrys, long curly eyelashes, downward curving of the mouth, thin small midline beak of the upper lip and notch in the lower lip, high arched palate, late eruption of teeth, and micrognathia. Children tend to be hirsute with hypoplastic nipples and umbilicus present. Limb abnormalities (such as small arms or absence of forearms) may be present. Feeding difficulties are common and include regurgitation, projectile vomiting, and chewing and swallowing difficulties. Speech is very slow to develop.

Background Information and History AZ was born at 37 weeks and weighed 2.5 kg. He was markedly dysmorphic, with cleft palate, micrognathia, large posterior fontanelle, small ears, webbed neck, short limbs, a deep sacral dimple, and other minor congenital anomalies. A systolic murmur was detected and echocardiography demonstrated a large left patent arterial duct. Soon after birth he was diagnosed as having Cornelia de Lange syndrome.

S. Reilly and L. Carr: Foreign Body Ingestion in Children

Gastroesophageal reflux, with projectile vomiting, was diagnosed within the first year and medical management (cisapride, Gaviscon, and ranitidine) was ongoing. The cleft palate remained unrepaired; an attempt to repair the palate at 18 months was unsuccessful because of intubation problems. He also had hip displacement and was under the care of an orthopedic surgeon. No history of recurrent chest infections was reported but frequent eye and ear infections occurred. He attended a special school for children with SDD.

Feeding History Feeding was problematic from birth, resulting in nasogastric tube feeding for the first two years of life. Bottle feeding was commenced for a short period, although intermittent tube feeding continued to be required over the next 2–3 years usually in association with intercurrent illness. The nasogastric tube was removed at 3.5 years and the child was weaned onto liquids and purees fed from a soft spouted bottle. Although initially successful, he gradually began to refuse to feed orally. Parental concern increased as attempts at oral feeding resulted in choking, gagging, and coughing. Around this time he suffered a viral infection with marked changes in bowel habit which his parents attributed to the attempted reintroduction of particular solid feeds.

Concerns on Referral Both parents and the referrer were concerned that there had been deterioration in his feeding. He was almost completely reliant on liquid bottle feeds; these deteriorated to the extent that feeding times were erratic and he fed little and often. Despite this, he was said to show interest in food and liked to play “food games,” feeling the food and taking it from other’s plates. His parents said they were frightened of feeding him more solid foodstuffs because of the coughing and choking but would like him to take a more age-appropriate diet if this was both advisable and possible.

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from his bottle. No overt oral motor and/or pharyngeal dysfunction was evident; lip seal was adequate, a reasonable volume of liquid was taken in a short period, and there was no coughing or choking. He assisted with spoon feeding puree by taking the spoon and placing it in his mouth. He took 3–4 spoonfuls very slowly before becoming agitated and refusing further food. he took the bottle-fed milk in bursts, stopping and starting throughout the feeding. Examination revealed that his chest was clinically clear and his abdomen was soft. He did not have a jaw jerk, but patella and biceps reflexes were present and symmetrical. He crawled in a reciprocal pattern around the room and walked with his knees and hips flexed with support but not independently.

Summary of Findings Both the history and the clinical examination suggested that the etiology of the presenting feeding problems were likely to be multifactorial. First, we were aware that feeding difficulties (chewing and swallowing difficulties) were commonly associated with Cornelia de Lange. Second, gastroesophageal reflux and projectile vomiting may have affected his appetite and desire to eat. Third, it was not clear what effect structural anomalies, such as the unrepaired cleft palate and shape and size of his oral cavity, might have on the ability to feed orally. Fourth, he had received prolonged periods of nasogastric tube feeding and had subsequently missed sensitive periods for the introduction of different food tastes and textures. Finally, he had significant developmental delay in both cognitive and motor functioning. His ability to manage more solid foodstuffs certainly needed to be considered within the context of his overall developmental levels. His gradual deterioration in feeding warranted further investigation as a priority. A two-stage approach was agreed upon with his parents. Initially, a barium swallow would be performed to assess the oral, pharyngeal, and esophageal stages of the digestive process, followed by 24-hour pH monitoring if indicated.

Clinical Examination

Results of Investigations

On examination, he was found to be responsive and interactive. Classical features associated with Cornelia de Lange, including short stature [weight ! 18.2 kg, length ! 83 cm (both well below third centile)] were present. He was noted to have a small narrow tongue and a high arched palate and was heard to grind his teeth excessively. A typical feeding was observed and he drank

The barium swallow and modified barium swallow revealed foreign bodies in the proximal esophagus (Fig. 1). An esophageal stricture was noted below the foreign bodies (Fig. 2) and gross gastroesophageal reflux to the level of the stricture was noted (Fig. 3). As a result, he was seen by the surgical team and five foreign bodies (coin, earring clasp, one other small piece of metal, and

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Fig. 1. Chest x-ray showing foreign body in the proximal oesophagus.

two small plastic objects) were removed. Dilatation of the stricture was also undertaken at the same time. In the meantime he was referred to the pediatric surgeons about management of his ongoing reflux and to the dietitians about nutritional needs. One month later a gastrostomy and a Thal fundoplication were performed as recommended by the surgical team. He recovered well, although he did suffer from dumping and diarrhea (requiring hospitalization on one occasion) and some gastrostomy site granulation. Follow up Three months later he was admitted to the hospital with a gastrostomy site infection, severe diarrhea, fever, and a

report of blood-stained vomit and coffee-ground aspirate on one occasion. A chest x-ray showed shadows of a dense object in the upper mediastinum thought to be a foreign body. An esophagoscopy was performed and a small piece of plastic was removed from the esophagus. Approximately three months later, he presented once again to the surgeons with a history of increasing difficulty with feeding over a 4–5-week period with decreasing tolerance of bottle feeds and increased gagging and coughing. He was also reported to be very irritable and the gastrostomy site was inflamed. Esophagoscopy revealed the presence of foreign bodies (a piece of a sweet packet, a piece of plastic, and some cardboard) which were removed from the esophagus. An esophageal stricture was dilated.


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Fig. 2. Barium swallow illustrating the presence of an esophageal stricture below the foreign bodies.

Discussion More than three-quarters of all FBI cases in children occur under the age of 6 years [3]. While the majority of foreign bodies pass through the digestive system uneventfully in 4–7 days [4], impaction may occur in the esophagus. The ingestion of foreign bodies by SDD children carries a high morbidity and mortality rate [5].

A number of reasons have been proposed to explain why children with SDD might be at a higher risk of FBI: 1. Impaired or delayed fine and gross motor skills may result in poor control of hand-to-mouth activity and exploration of objects. 2. Children with severe developmental delay often have a prolonged oral phase involving constant mouthing

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Fig. 3. Barium swallow showing contrast pooled in the ballooned esophagus above the stricture and gross gastroesophageal reflux to the level of the stricture.

of objects. Pica, an inability to discriminate between edible and nonedible substances, is also reported to occur more frequently in children with severe cognitive impairment [6]. 3. Oral and pharyngeal dysphagia limits control over objects placed in the oral cavity [7], and reduced intraoral and pharyngeal sensation may further contribute to FBI.

4. Impaired protective mechanisms, such as frequent gagging, coughing and choking episodes, occur in children with SDD. A sudden episode, therefore, may not provoke additional anxiety in the child’s caregivers. 5. Communication impairment in individuals with SDD can result in an inability to communicate even the


most basic signals, i.e., that they had swallowed something unusual! Dallal et al. [2] reported examining an adult patient with SDD (unable to communicate verbally) whose inability to open his mouth voluntarily for an endoscopic examination was considered behavioral. Subsequent examination revealed that the patient had FBI. Alterations in state and behavior (whether related to feeding or not) are often ignored in people with SDD, and behavioral changes are attributed to nonorganic causes. We believe that many, if not all, of the above factors contributed to this patient’s repeated FBI. In addition, the presence of an unrepaired cleft palate, a history of prolonged nasogastric tube feeding, and the presence of severe gastroesophageal reflux (requiring fundoplication) were also contributory factors. Clinicians should have a high index of suspicion in individuals with SDD who present with feeding difficulties. This patient had been under the care of a craniofacial team since birth and had seen a number of specialists because of his multiple and complex disabilities. However, prior to referral he had not been seen by a multidisciplinary dysphagia team. The feeding difficulties he had were attributed to being “part of the SDD” and, therefore, other causes had not been considered. Challenging feeding behavior does coexist in people with SDD; however, careful history and thorough clinical examination should be made before a nonorganic cause is attributed to such patients. Factors that should raise a high level of suspicion in individuals with SDD include: sudden refusal to eat or drink sudden loss of appetite or weight gradual change in eating habits or patterns gradual exclusion of foods from the diet until the child/adult is taking a restricted range of solids or existing solely on a liquid diet • gradual or sudden onset of “difficult” mealtime behavior

• • • •

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These factors have implications for caregivers and the staff in schools, day care centers, and residential homes that care for individuals with SDD. The individual’s behavior should be monitored (a diary of events can be helpful), and referral to a multidisciplinary dysphagia team should be expedited. Many people with SDD will not have had a dysphagia assessment despite the fact that more than half are underweight and have significant coexisting feeding problems [2,8,9]. As the case we have described illustrates, regular review of feeding status in individuals with SDD is essential. At the last followup visit, this patient was feeding well; however, his parents and local team are aware of his tendency to ingest foreign objects despite his parents vigilance. They recognize the warning signs that suggested foreign body ingestion in the past and are able to act accordingly. References 1.

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7. 8.

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