Author: Joshua Alexander, MD
INTRODUCTION
Children with cerebral palsy often have many associated conditions which increase their risk of respiratory illness.Examples of these conditions include: a history of prematurity associated with bronchopulmonary dysplasia; low central tone contributing to gastroesophageal reflux disease which can exacerbate underlying reactive airway disease; and dysphagia leading to aspiration pneumonia. This report presents an unusual cause of respiratory illness in a child with cerebral palsy.
CASE REPORT
A boy with spastic tetraplegic cerebral palsy was referred for recent onset of respiratory difficulties. He presented with a 6-month history of increasing shortness of breath at rest. He denied any fevers, chest pain, cough, or nighttime waking. There was no reported abdominal pain, vomiting, or straining with bowel movements.
MEDICAL HISTORY
He was a former 28 week, 709 gram twin born to a 17 year old primagravida female. Neonatal course included ventilator dependence for bronchopulmonary dysplasia, apnea of prematurity, intraventricular hemorrhage, and hyperbilirubinemia. He went home at 45 days of age. Although he had undergone multiple orthopedic surgeries in the past, his medical history was unremarkable. There was no history of acute trauma. He had no known allergies and was on no medications.
FUNCTIONAL HISTORY
Included the use of a manual wheelchair for household and community mobility. He was independent in communication, and required minimal assistance with bed mobility. He reported complete dependence in transfers, describing the transfers as being slung "like a sack of potatoes" up onto his parents' shoulders.
PHYSICAL EXAMINATION
revealed an obese white male in mild respiratory distress. Temperature was 36.2 degrees Celsius, blood pressure was 94/60 mmHg, and respiratory rate was 24. HEENT demonstrated open mouthed breathing, but no nasal flaring. Heart sounds were distant throughout, but without murmur or rub. Breath sounds were diminished on the entire left side. Abdomen was obese, but non-tender. Extremities showed no cyanosis, clubbing or edema.
X-RAYS
A review of a scoliosis series performed 11 days previously showed opacities in both lung fields with scattered rounded lucencies on the left. An AP and lateral chest x-ray was ordered and showed bilateral opacities and scattered cystic lucencies on the left with deviation of the heart and trachea to the right. A tentative diagnosis was made and surgical consultation was requested.
A barium enema showed normal position of the colon distal to the splenic flexure, with the mid-transverse section passing through a defect into the thoracic cavity. Several loops of bowel were seen within the left thoracic cavity with the ascending colon lying in the right hemithorax.
TREATMENT
The patient was taken to surgery where a Morgagni diaphragmatic hernia with sac was noted. A chest tube was placed in the apex of the left chest, the normal appearing colon was placed back into the abdomen, and the diaphragmatic defect was repaired. At discharge home, the patient was breathing without difficulty, tolerating a regular diet and having normal bowel movements. An upper GI showed normal alignment of the colon with no obstruction. His parents were instructed in proper transfer technique and he had an uneventful recovery with resolution of symptoms.
DISCUSSION
Congenital diaphragmatic hernia is rarely diagnosed outside the neonatal period. When symptoms develop later in life; there is often a delay in diagnosis, with many misdiagnoses leading to inappropriate and unsuccessful treatment. Although not as life threatening as congenital diaphragmatic hernia that presents at birth, a late appearing diaphragmatic hernia can cause morbidity and mortality if left untreated (Delport, 1996; Newman, et al. 1986). The majority of congenital diaphragmatic hernias occur on the left side and are posterolateral (Bochdalek) in position. Morgagni (anterior) hernia is a more rare occurrence (Booker et al. 1981) In this case, the presence of a sac noted at surgery indicated that this patient likely had a small and asymptomatic hernia defect since birth, but a chest x-ray at age four demonstrated no herniation. One possible explanation for this patient's late appearing illness is that the small diaphragmatic defect was asymptomatic until an increased intraabdominal pressure pushed the abdominal contents up through the defect and into the thorax (Burge & Samuel, 1998).While this patient denied any history of strong coughs, straining with bowel movements, or severe trauma, his functional history did offer a possible scenario for increased intraabdominal pressure. The improper transfer technique of swinging the patient up onto his parents' shoulders might have led to herniation of his colon through the diaphragmatic defect.
Children with cerebral palsy have many complex primary and secondary conditions that make their medical care challenging to both general and specialist physicians. A diagnosis of diaphragmatic hernia was made here because of a complete history and physical examination coupled with appropriate radiological studies.
REFERENCES
Booker P, Meerstadt P, Bush G.(1981) Congenital diaphragmatic hernia in the older child. Archives of Disease in Childhood 56:253-257.
Burge DM & Samuel M. (1998) Congenital diaphragmatic hernia. In: Atwell JD, editor. Paediatric Surgery. New York: Oxford University Press. pp.155-165.
Delport S. (1996) Aftermath of failed diagnosis of late-presenting congenital diaphragmatic hernias. South African Journal of Surgery 34:69-72.
Newman BM, Afshani E, Karp MP, Jewett TC, Cooney DR. (I 986) Presentation of congenital diaphragmatic hernia past the neonatal period. Archives of Surgery 121:813-816.
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