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Items 1 - 7 of 7 |
One page. |
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General anaesthesia in a child with Larsen syndrome.
Critchley LA, Chan L.
Department of Anaesthesia and Intensive Care, Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, New Territories, Hong Kong, China.
Larsen syndrome is a rare congenital connective tissue disorder which is characterized by multiple dislocations of major joints, typical facial appearance, cervical spine narrowing and instability and respiratory difficulties secondary to laryngotracheomalacia. A case of general anaesthesia for laparoscopic hernia repair in a male child aged 16 months with this syndrome is presented. Anaesthesia was well tolerated though intubation proved difficult. Cervical spine instability and postoperative respiratory complications are potential problems.
Publication Types:
PMID: 12712791 [PubMed - indexed for MEDLINE]
Larsen syndrome and its anaesthetic considerations.
Malik P, Choudhry DK.
Department of Anesthesia, Thomas Jefferson University Hospital, Philadelphia, PA, USA.
Larsen syndrome is a complex syndrome with genetic heterogeneity, and with both autosomal dominant and autosomal recessive patterns of inheritance. It is characterized by congenital dislocation of joints, flat faces and complicated by issues relating to respiratory, cardiac, musculoskeletal and central nervous systems. This report describes the anaesthetic management of two patients with Larsen syndrome. The first case is a 4-year-old patient who had cervical cord compression secondary to cervical instability and who was scheduled for anterior corpectomy with fusion of cervical vertebrae and placement of halo frame. This patient had transient loss of evoked potentials during positioning and a stormy postoperative course requiring reintubation and a prolonged stay in the intensive care unit. The second case is a 22-month-old child who was scheduled for a repeat posterior cervical spinal fusion due to failure of her initial fusion procedure. This patient had an uneventful perioperative course. Relevant anaesthetic issues in patients with Larsen syndrome are discussed.
Publication Types:
PMID: 12358662 [PubMed - indexed for MEDLINE]
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[Spinal cord injury following knife stab wound]
[Article in Norwegian]
Larsen LB, Tollesson G, Solgaard T.
Nevrokirurgisk avdeling Ulleval sykehus 0407 Oslo.
BACKGROUND: Problems about penetrating injuries are well known, but spinal cord damage is rare. Stab wounds to the spinal cord may be a new type of injury in our society. MATERIAL AND METHODS: We describe two patients brought to our hospital with stab wounds to the cervicothoracic region and major neurologic injury. One was treated initially only with cleaning and primary closure of the skin. After two weeks the intraspinal damage was repaired in our neurosurgical unit. The second patient was immediately brought to neurosurgery. The intraspinal damage was explored and the dural tear was closed. RESULTS: The first patient got a superficial infection and spinal fluid leakage after initial treatment. This resolved when the dura was closed. There were no wound complications in the second patient. Both demonstrated Brown-Sequard's syndrome. Neurologic recovery was much better in the first than in the second patient. INTERPRETATION: Minor penetrating wounds in the neck region may represent damage to the spinal cord with major neurologic injury. Further investigation with MR is preferable and we recommend immediate surgical treatment with closure of the dural tear.
Publication Types:
PMID: 11255857 [PubMed - indexed for MEDLINE]
Comment on:
Cervical kyphosis in patients who have Larsen syndrome.
Johnston CE 2nd, Schoenecker PL.
Publication Types:
- Case Reports
- Comment
- Letter
PMID: 9378746 [PubMed - indexed for MEDLINE]
Comment in:
Cervical kyphosis in patients who have Larsen syndrome.
Johnston CE 2nd, Birch JG, Daniels JL.
Texas Scottish Rite Hospital for Children, Dallas, Texas 75219, USA.
Four patients who had Larsen syndrome and cervical kyphosis were managed operatively and followed for an average of seventy months (range, forty to ninety-two months). The preoperative cervical kyphosis ranged from 35 to 65 degrees. The patients had had a posterior cervical arthrodesis alone when they were infants, at an average age fo fourteen months (range, ten to sixteen months). In three infants, the kyphosis either stabilized (one patient) or reversed into lordosis (two patients). Thus, the kyphosis corrected gradually by continued anterior growth in the presence of a solid posterior fusion. In the fourth infant, the kyphosis progressed to 110 degrees because of pseudarthrosis. This child had anterior decompression and arthrodesis for an acute neurological deficit. We believe that cervical kyphosis is sometimes present but not diagnosed in patients who have Larsen syndrome. Early diagnosis followed by operative stabilization should help such patients avoid neurological deficits. Posterior cervical arthrodesis alone, performed in infancy, provided stability and the opportunity for the gradual correction of the deformity by continued anterior growth in three of our four patients.
Publication Types:
PMID: 8609132 [PubMed - indexed for MEDLINE]
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Prenatal diagnosis and obstetric management of Larsen syndrome.
Rochelson B, Petrikovsky B, Shmoys S.
State University of New York, Stony Brook.
BACKGROUND: Larsen syndrome is a disease of generalized defect in collagen formation including multiple disorders of the joints and cardiac anomalies. A review of the literature revealed no previous reported cases in pregnancy. CASE: A 29-year-old woman with known Larsen syndrome thought to be of the autosomal recessive type presented in pregnancy; second-trimester ultrasound suggested fetal involvement with Larsen syndrome. The patient was followed with serial ultrasounds, and she had pediatric and anesthesiologic consultations. She was delivered by cesarean of a female infant whose neck was immediately stabilized. The infant was then taken to the neonatal intensive care unit, where a diagnosis of Larsen syndrome was confirmed. CONCLUSIONS: Larsen syndrome, which may be diagnosed prenatally, is a rare and unique condition that requires multidisciplinary care. Obstetric management must take into account the increased anesthetic and surgical risks to the mother and the risk of fetal injury including cervical spine instability. The genetics of Larsen syndrome are also discussed.
Publication Types:
PMID: 8469494 [PubMed - indexed for MEDLINE]
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[Dislocation of the superior cervical spine in the Larsen syndrome]
[Article in French]
Lefort G, Mourad H, de Niscault G, Daoud S.
The discovery of a dislocation of the upper cervical rachis through corporeal hypoplasia of the C3 ans listhesis of the C2 gives rise, at the age of 18 months, to a stabilization by means of surgery due to the threat of compression myelithis. A review of relevant literature brings to emphasis the ill-knowledge of spinal damage and the wide variety of its different types in cases of the Larsen syndrome.
Publication Types:
PMID: 6627541 [PubMed - indexed for MEDLINE]
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