Anesthetic management of a patient with myotonic dystrophy for a Nissen fundoplication and gastrostomy

Jenkins, Jenny; Facer, E

doi:10.1111/j.1460-9592.2004.01280.x

Cited by 12 publications

(5 citation statements)

References 10 publications

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“…Neuraxial blockade (caudal, epidural, and spinal) has been used frequently in DM children, most often in combination with light general anesthesia or sedation . This is an easy and successful way to provide intra‐ and postoperative analgesia with no or minimal doses of opioids.…”

Section: Anesthetic Carementioning

confidence: 99%

Myotonic Dystrophies type 1 and 2: anesthetic care

Veyckemans

Scholtès

2013

Pediatric Anesthesia

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Myotonic dystrophy is classified as one of the myotonic syndromes although myotonia is only a minor characteristic of it. It is, in fact, also a multisystem disease with cardiac, digestive, ocular, and endocrine abnormalities. Two subgroups are currently identified with many similarities: DM1 refers to classic dystrophia myotonica (Steinert disease), while DM2, formerly called proximal myotonic myopathy has a later onset. The congenital form is present only in DM1. The genetic causes of DM1 and 2 are different but end up in a similar way of altering RNAm processing and splicing of other genes. The anesthetic risk is increased in case of DM1 type. This review summarizes current knowledge concerning the pathophysiology and anesthetic management of this disease in children and adults.

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Section: Anesthetic Carementioning

confidence: 99%

Myotonic Dystrophies type 1 and 2: anesthetic care

Veyckemans

Scholtès

2013

Pediatric Anesthesia

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“…The genetic error that leads to DM 1 was identified in 1991 and is the expansion of an unstable trinucleotide repeat [cytosine, thymine, guanine (CTG)] in the 3′ untranslated region of a protein kinase gene on chromosome 19q (6). Four subtypes of DM 1 are described: congenital myotonic dystrophy (CDM 1 ), early‐onset or infantile and juvenile myotonic dystrophy (JDM 1 ), adult‐onset, and late‐onset asymptomatic myotonic dystrophy (6,7).…”

Section: Introductionmentioning

confidence: 99%

“…The most severe form of the disease is CDM 1 , which tends to be transmitted by affected mothers and has a high mortality in the neonatal period (30–40%) (6,8). Neonates may present with hypotonia, feeding difficulty, and respiratory distress after a pregnancy complicated by polyhydramnios and premature labor (3,6,7). Most have facial weakness and the characteristic tent‐shaped mouth.…”

Section: Introductionmentioning

confidence: 99%

Risk factors for perioperative adverse events in children with myotonic dystrophy

Sinclair¹,

Reed

2009

Pediatric Anesthesia

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The MIRS is a statistically significant and clinically useful tool for predicting high perioperative risk. Patients with a high MIRS grade should therefore be considered for postoperative intensive care. The use of muscle relaxant without reversal was also shown to be a significant risk factor. Patients who require morphine infusions postoperatively might also be most safely managed in a high dependency unit.

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“…The laparoscopic technique which is increasingly applied in children with neurological handicaps may reduce operative and postoperative complications, but does not shorten the duration of surgery [9,15,16] . Data about success and complications of surgical antirefl ux therapy in patients with GER and underlying myopathic disease are limited [4,7] . But some experience has been gained in patients with the spinal muscular atrophy (SMA) type I [2,3,20] , which shares similarities with untreated infantile Pompe ' s disease with respect to age at onset of fi rst symptoms, distribution and extent of muscle weakness, degree of fl accidity, and rate of progression.…”

Section: Discussionmentioning

confidence: 99%

Treatment of Gastroesophageal Reflux with Nissen Fundoplication and Gastrostomy Tube Insertion in Infantile Pompe's Disease

Hirschburger¹,

Hecker²,

Padberg³

et al. 2009

Neuropediatrics

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In infantile Pompe's disease, enzyme replacement therapy (ERT) has been shown to reverse cardiomyopathy, improve skeletal muscle strength, and prolong survival. We report on five patients in whom complications related to gastroesophageal reflux (GER) resulted in deterioration of their clinical status despite initial improvement under ERT. Surgical antireflux therapy, performed in four, yielded positive results in two. Three patients experienced severe aspirations related to GER and underwent fundoplication and gastrostomy subsequently. Two did not regain former motor functions and deceased shortly thereafter, while one slowly recuperated and is in a stable state at age 53 months. In a further patient, severe GER prompted fundoplication at age 17 months. No aspirations occurred until the girl deceased probably due to cardiac arrest 20 months later. These cases suggest that infants with Pompe's disease under ERT may benefit from timely performed fundoplication and gastric tube placement.

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Anesthetic management of a patient with myotonic dystrophy for a Nissen fundoplication and gastrostomy

Cited by 12 publications

References 10 publications

Myotonic Dystrophies type 1 and 2: anesthetic care

Myotonic Dystrophies type 1 and 2: anesthetic care

Risk factors for perioperative adverse events in children with myotonic dystrophy

Treatment of Gastroesophageal Reflux with Nissen Fundoplication and Gastrostomy Tube Insertion in Infantile Pompe's Disease

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