Achalasia cardia presenting with Marasmic Kwashiorkor
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《美国医学杂志》
Department of Pediatrics, Lokmanya Tilak Municipal Medical College & General Hospital, Sion, Mumbai 400 022, India
A one-year, nine-month-old girl presented to us with non-projectile vomiting, fever and cough with breathlessness, since one month. There was a history of regurgitation of feeds since the last three months, for which a milk scan and an ultrasound of the abdomen were done, both reported as normal. There was a history of failure to thrive since birth. The child was the first of two children, the second being a 6 months old female who was normal. The patient had been delivered normally at term, with a birth weight of 1.75 kg. She was exclusively breast fed till one year of age, when weaning was started which was inadequate due to poor intake. Normal tear formation was present.
On examination, there was generalized wasting, with loss of subcutaneous fat. The child was febrile with a respiratory rate of 50/min. Her weight was 3.64 Kg, length 60 cm and head circumference 40.5cm, all less than the fifth percentile for age. Scorbutic beading, mild pallor and pitting pedal edema were present. Respiratory system examination revealed intercostal retractions and bilateral fine crepitations. Other systems were essentially normal.
Investigations at admission, revealed a random blood sugar of 60mg/dl. The hemoglobin was 9.6 g/dl and the total WBC count 10,000/cumm with a differential of polymorphs 67%, lymphocytes 29%, and monocytes 4%. The serum total proteins were 4.8 g/dl, with a serum albumin of 1.8 g/dl. Other liver function tests were normal as were the renal function tests, arterial blood gases and serum electrolytes. The chest X-ray Figure1 showed bilateral patchy opacities suggestive of bronchopneumonia and an absence of the fundic air bubble. A diagnosis of bronchopneumonia with marasmic kwashiorkor was made. With a history of regurgitation and an X -ray chest showing an absence of the fundic air bubble, a possibility of achalasia cardia was considered.
Pneumonia was treated with intravenous amoxicillin-clavulinic acid combination and amikacin. The child also received oxygen, nebulisation and chest physiotherapy. Nasogastric feeding was started once the respiratory distress had settled. Over a period of two weeks there was a loss of edema followed by a steady weight gain up to 4.14 kg. A barium swallow done showed dilatation of the esophagus with hold-up of the contrast material and narrowing of the distal esophagus with the typical bird's beak appearance described in achalasia cardia Figure2. An esophagoscopy revealed resistance at the lower esophageal sphincter.
A modified Heller's myotomy was done. At follow-up 8 months later, the weight was 7.8 kg and the patient was asymptomatic.
Achalasia is a primary, esophageal motility disorder caused by failure of the lower esophageal sphincter to relax and an absence of esophageal peristalsis leading to a functional obstruction at the gastro-esophageal junction. Only 4%-5% of patients with achalasia are symptomatic prior to 15 years of age.[1] Achalasia cardia could occur in isolation, or as part of Allgrove's syndrome, where it is associated with alacrima and adrenocorticotrophic hormone insensitivity.[2] Weight loss, emesis, dysphagia, recurrent lower respiratory tract infections, chest pain and heart burn are the common modes of presentation.[3] The vomiting may be mistaken for gastro-esophageal reflux and may delay diagnosis. Although the incidence of achalasia in childhood is extremely low, the retardation of growth and development, as well as the severity of pulmonary symptoms are profound hence early diagnosis and treatment are important. A history of vomiting uncurdled milk and an absence of the fundic air bubble on chest X- ray should alert us to the possibility of achalasia cardia.[4]
Acknowledgement
We would like to thank our Dean, Dr. M.E. Yeolekar for giving us permission to publish this article.
References
1. Olsen AM, Harrington SW, Moersch HJ et al. The treatment of cardiospasm: Analysis of a twelve year experience. J Thorac Sur 1951; 22 : 164-187.
2. Allgrove J, Clayden GS, Grant DB. Familial glucocorticoid deficiency with achalasia of the cardia and deficient tear production. Lancet 1978; 1 : 1284-1286.
3. Myers NA, Jolley SG, Taylor R. Achalasia of the cardia in children: A worldwide survey. J Pediatr Surg 1994; 29:1375-1379.
4. Thomas R J, Sen S, Zachariah N et al. Achalasia cardia in infancy and childhood : an Indian experience. JR Coll Surg Edind 1998; 43(2) : 103-104.(More Vaishali, Shanbag Pr)
A one-year, nine-month-old girl presented to us with non-projectile vomiting, fever and cough with breathlessness, since one month. There was a history of regurgitation of feeds since the last three months, for which a milk scan and an ultrasound of the abdomen were done, both reported as normal. There was a history of failure to thrive since birth. The child was the first of two children, the second being a 6 months old female who was normal. The patient had been delivered normally at term, with a birth weight of 1.75 kg. She was exclusively breast fed till one year of age, when weaning was started which was inadequate due to poor intake. Normal tear formation was present.
On examination, there was generalized wasting, with loss of subcutaneous fat. The child was febrile with a respiratory rate of 50/min. Her weight was 3.64 Kg, length 60 cm and head circumference 40.5cm, all less than the fifth percentile for age. Scorbutic beading, mild pallor and pitting pedal edema were present. Respiratory system examination revealed intercostal retractions and bilateral fine crepitations. Other systems were essentially normal.
Investigations at admission, revealed a random blood sugar of 60mg/dl. The hemoglobin was 9.6 g/dl and the total WBC count 10,000/cumm with a differential of polymorphs 67%, lymphocytes 29%, and monocytes 4%. The serum total proteins were 4.8 g/dl, with a serum albumin of 1.8 g/dl. Other liver function tests were normal as were the renal function tests, arterial blood gases and serum electrolytes. The chest X-ray Figure1 showed bilateral patchy opacities suggestive of bronchopneumonia and an absence of the fundic air bubble. A diagnosis of bronchopneumonia with marasmic kwashiorkor was made. With a history of regurgitation and an X -ray chest showing an absence of the fundic air bubble, a possibility of achalasia cardia was considered.
Pneumonia was treated with intravenous amoxicillin-clavulinic acid combination and amikacin. The child also received oxygen, nebulisation and chest physiotherapy. Nasogastric feeding was started once the respiratory distress had settled. Over a period of two weeks there was a loss of edema followed by a steady weight gain up to 4.14 kg. A barium swallow done showed dilatation of the esophagus with hold-up of the contrast material and narrowing of the distal esophagus with the typical bird's beak appearance described in achalasia cardia Figure2. An esophagoscopy revealed resistance at the lower esophageal sphincter.
A modified Heller's myotomy was done. At follow-up 8 months later, the weight was 7.8 kg and the patient was asymptomatic.
Achalasia is a primary, esophageal motility disorder caused by failure of the lower esophageal sphincter to relax and an absence of esophageal peristalsis leading to a functional obstruction at the gastro-esophageal junction. Only 4%-5% of patients with achalasia are symptomatic prior to 15 years of age.[1] Achalasia cardia could occur in isolation, or as part of Allgrove's syndrome, where it is associated with alacrima and adrenocorticotrophic hormone insensitivity.[2] Weight loss, emesis, dysphagia, recurrent lower respiratory tract infections, chest pain and heart burn are the common modes of presentation.[3] The vomiting may be mistaken for gastro-esophageal reflux and may delay diagnosis. Although the incidence of achalasia in childhood is extremely low, the retardation of growth and development, as well as the severity of pulmonary symptoms are profound hence early diagnosis and treatment are important. A history of vomiting uncurdled milk and an absence of the fundic air bubble on chest X- ray should alert us to the possibility of achalasia cardia.[4]
Acknowledgement
We would like to thank our Dean, Dr. M.E. Yeolekar for giving us permission to publish this article.
References
1. Olsen AM, Harrington SW, Moersch HJ et al. The treatment of cardiospasm: Analysis of a twelve year experience. J Thorac Sur 1951; 22 : 164-187.
2. Allgrove J, Clayden GS, Grant DB. Familial glucocorticoid deficiency with achalasia of the cardia and deficient tear production. Lancet 1978; 1 : 1284-1286.
3. Myers NA, Jolley SG, Taylor R. Achalasia of the cardia in children: A worldwide survey. J Pediatr Surg 1994; 29:1375-1379.
4. Thomas R J, Sen S, Zachariah N et al. Achalasia cardia in infancy and childhood : an Indian experience. JR Coll Surg Edind 1998; 43(2) : 103-104.(More Vaishali, Shanbag Pr)