Successful epidural anaesthesia for Caesarean section in a patient with spondyloepiphyseal dysplasia

H.D. de Boer, A. Hemelaar, R. van Dongen and M. J. M. Gielen

Department of Anesthesiology, University Medical Center St Radboud, PO Box 9101, 6500 HB Nijmegen, The Netherlands*Corresponding author

Accepted for publication: July 19, 2000


    Abstract
 Top
 Abstract
 Introduction
 Case report
 Discussion
 References
 
Spondyloepiphyseal dysplasia congenita is a rare genetic entity in which it is very important to involve anaesthetists early on to discuss the possible anaesthetic complications for both general or regional anaesthesia. A case is described of a patient with spondyloepiphyseal dysplasia and multifetal pregnancy in which successful epidural anaesthesia for caesarean section was performed.

Br J Anaesth 2001; 86: 133–4

Keywords: complications, spondyloepiphyseal dysplasia congenita; anaesthetic techniques, epidural; anaesthesia, obstetric


    Introduction
 Top
 Abstract
 Introduction
 Case report
 Discussion
 References
 
Spondyloepiphyseal dysplasia congenita (SDC) is a rare entity characterized by pathological changes in the vertebral column and the epiphyses of long bones, which results in dwarfism.1 Its precise incidence is unknown, but may be 1–2 per million.2 In pregnant patients with SDC, Caesarean section is indicated because of the small pelvis. Early discussion with anaesthetists is very important because of the possible anaesthetic complications of both general and regional anaesthesia in such patients.35 Only one case has been reported in the English literature on anaesthetic management in a pregnant patient with SDC, but none in patients with SDC and multifetal pregnancy.5 The present report describes a case of a patient with SDC and multifetal pregnancy in which succesful epidural anaesthesia for Caesarean section was performed.


    Case report
 Top
 Abstract
 Introduction
 Case report
 Discussion
 References
 
A 39 yr old nulliparous patient with SDC was admitted for elective Caesarean section. The patient’s medical history revealed SDC diagnosed in late childhood. The patient had been wheelchair dependent since her 25th year as a result of bilateral hip dislocation. In another hospital, she had undergone an in vitro fertilization procedure because of idiopathic infertility; this resulted in a twin pregnancy.

Physical examination showed that the patient had a short trunk with a reduced xiphoid-to-pubic distance, marked lumbar kyphoscoliosis and shortened limbs. Her height was 134 cm and her weight 75 kg. Extension of the neck was normal, as were jaw movements. No neurological symptoms were found. Lung function test results were as follows (normal values in brackets): vital capacity, 2.40 litres (3.40 litres); total lung capacity, 3.45 litres (4.97 litres); functional residual capacity, 1.00 litre (1.60 litres); forced expiratory volume in 1 s, 2.10 litres (2.90 litres), showing a mild restrictive defect. Radiographic examination revealed severe lumbar kyphoscoliosis, flattening of the vertebral bodies at all levels and an inadequate pelvic outlet. However, x-rays of the cervical spine were normal.

As a result of these findings, Caesarean section was indicated, so preoperative consultation with an anaesthetist was performed early, at 14 weeks’ gestation. Repeat lung function tests showed no further deterioration of the restrictive function, so Caesarean section was delayed until 37 weeks’ gestation. Caesarean section under epidural anaesthesia was planned. Premedication was with ranitidine 150 mg orally on the night before and on the morning of the operation. Standard procedures, including non-invasive automatic monitoring of arterial pressure, oxygen saturation and electrocardiography, were used for monitoring the patient in theatre. Before surgery, a 1500 ml preload of Ringer’s solution was administered. As a result of the lumbar kyphoscoliosis, the most comfortable position for the patient during epidural puncture was lying on her left side. After extensive palpation, an intervertebral space was found, which was considered to be the interspace L3–L4. After infiltration with local anaesthetic, a standard 18-gauge Tuohy needle was introduced, using the loss of resistance to air technique. The epidural space was identified 3 cm from the skin and a catheter was inserted 4 cm into the extradural space. After a test dose of 3 ml of 0.5% bupivacaine with 1:200 000 epinephrine showed no symptoms of subarachnoid or intravascular injection, 5 ml of 0.5% bupivacaine with epinephrine was injected. After 20 min, the upper level of sensory block, tested by pin-prick in the mid axillary line, was T5. The patient was turned supine with left lateral tilt. Mild hypotension was treated with intravenous ephedrine. Surgery proceeded uneventfully. The first child was a 2.7 kg male infant (Apgar scores 6 and 9) and the second a 2.8 kg female infant (Apgar scores 6 and 9). Oxytocin 5 IU was given at delivery. Postoperatively the epidural catheter was removed because of the unusual vertebral column anatomy of the patient. Total blood loss was estimated as 1500 ml. After anaesthesia, the course was uneventful and postoperative pain management was achieved with patient-controlled analgesia. Mother and children were discharged 5 days after delivery.


    Discussion
 Top
 Abstract
 Introduction
 Case report
 Discussion
 References
 
SDC is a rare entity, which arises spontaneously or from X-linked or autosomal dominant inheritance.1 The diagnosis is made at birth or in late childhood. SDC is characterized by pathological changes in the vertebral column, resulting in progressive kyphoscoliosis, platyspondyly, central anterior pointing of the vertebral bodies and atlantoaxial instability.1 35 Laryngotracheal stenosis has also been reported in patients with SDC.3 Kyphoscoliosis can impair respiration; in pregnant patients, this, combined with diaphragmatic splinting from the gravid uterus, may lead to severe respiratory problems. Our patient had marked lumbar kyphoscoliosis and was carrying more than one fetus, but only mild restrictive function disturbances were found.

At 14 weeks’ gestation, advice was sought regarding potential problems with anaesthesia in this patient, including the possibility of laryngotracheal stenosis and a high risk of aspiration resulting from a combination of skeletal anomalies and compromised respiratory status. The decision was made to perform epidural anaesthesia. We also considered that, in case of accidental dural puncture, the Caesarean section would be performed under continuous spinal anaesthesia using a spinal catheter. A continuous regional technique was preferred to a ‘single-shot’ technique because of the anatomical abnormalities of the vertebral column and the unpredictable spread of local anaesthetic.5 In our patient, the epidural space was entered with no problem. An appropriate sensory block was achieved with a low volume of local anaesthetic. The epidural catheter was removed because the unusual vertebral column anatomy increased the risk of unpredictable spread of local anaesthetics by continuous infusion.

In summary, we describe a case of a patient with SDC and multifetal pregnancy in which succesful epidural anaesthesia for Caesarean section was performed. The importance of early anaesthetic involvement in such patients is stressed.


    References
 Top
 Abstract
 Introduction
 Case report
 Discussion
 References
 
1 Jones KL Jr, Carden AB. Dysmorphic syndromes and their significance to anesthesiologists. In: Sterling LC, Zaunder HL, eds. Anesthetic Implications of Congenital Anomalies in Children. New York: Appelton–Century–Crofts, 1990; 9–22

2 Wynne-Davies R, Hall CM, Apley AG. Spondylo-epiphyseal dysplasia congenita with severe coxa vara. In: Wynne-Davies R, ed. Atlas of Skeletal Dysplasias. Edinburgh: Churchill Livingstone, 1985; 61

3 Myer CM, Cotton RT. Laryngotracheal stenosis in spondyloepiphyseal dysplasia. Laryngoscope 1985; 95: 3–5[ISI][Medline]

4 Mogera C, Muralidhar V. Spondyloepiphyseal dysplasia congenita syndrome: anesthetic implications. Anesth Analg 1996; 83: 433–42

5 Rodney GE, Callander CC, Harmer M. Spondyloepiphyseal dysplasia: Caeserean section under epidural anesthesia. Anaesthesia 1991; 46: 648–50[ISI][Medline]

6 Wardell GJ, Frame WT. Extradural anaesthesia for caesarean section in achondroplasia. Br J Anaesth 1990; 64: 367–70[Abstract]





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