Unsuccessful lumbar puncture in a paediatric patient with achondroplasia.
SUMMARYWe present a cast' of an unsuccessful lumbar puncture performed on an anaesthetised 17-year-old girl with achondroplasia who was diagnosed with and being treated for acute lymphoblastic leukaemia. Magnetic resonance imaging (MRI) subsequently showed spinal stenosis and no observable cerebrospinal fluid around the nerve roots at the levels of the lumbar puncture and dish A recommendation is made to obtain MRI scans before proceeding with lumbar puncture and/or Spinal anaesthesia in this patient group to ensure that the anatomical features of the insertion site are favourable to a successful outcome
Key Words: lumbar puncture, dural puncture, achondroplasia, leukaemia, paediatric, magnetic resonance imaging, spinal stenosis
Achondroplasia presents a number of important challenges to the anaesthetist because of characteristic physical and anatomical abnormalities. Since difficult intubation has been reported (1) and obstructive sleep apnoea and restrictive lung disease have been described (2), regional anaesthesia may be considered an attractive option for patients with achondroplasia. We describe a case of unsuccessful lumbar puncture in a patient with achondroplasia and review the anatomical and radiological abnormalities observed for the patient. Institutional Review Board approval and parental consent were obtained to present the following information as a case report.
CASE HISTORY
A 17-year-old girl with achondroplasia was preliminarily diagnosed with B -cell acute lymphoblastic leukaemia (ALL) (six days prior to the events described herein) after developing fatigue, fever, night sweats and bone pain. Her medical history included chronic otitis media, frequent pneumonias and an underlying restrictive lung disease.
Anaesthesia was scheduled to allow clinicians to perform bilateral bone marrow aspirates and biopsies, and a lumbar puncture for cytology and biochemistry. The patient was also scheduled to receive intrathecal chemotherapy.
She had previously undergone two uneventful anaesthetic procedures to correct fibular overgrowth at the age of 11 years and for insertion of tympanostomy tubes at age 16. On examination her vital signs were: HR 116, BP 130/73 mmHg, RR 21 bpm, T 37.9°C and Sp[O.sub.2] 95%. Her physical features were remarkable for the typical appearance of achondroplasia. She weighed 46 kg and was 124 cm tall (< third centile) with a body mass index of 29.8. Her head circumference was 57.5 cm (98th centile) and she had a prominent forehead, large body and short limbs. Chest examination was unremarkable. Airway examination demonstrateed a good range of movement at the temporo-mandibular joint, good neck extension, a Mallampati score of 1 and minimal pectus carinatum. Her investigations showed the following: haemoglobin 9.4 g/dl, WCC 1.4, ANC 800 and platelets 105/[mm.sup.3]. Electrolytes were all within the normal range.
Anaesthesia was provided outside the operating room in a dedicated minor procedure area. Monitoring consisted of non-invasive blood pressure measurement every three minutes, continuous pulse oximetry with heart rate monitoring and capnography via nasal cannulae. The patient was preoxygenated and then anaesthesia was induced intravenously using a peripherally inserted central cannula. Fentanyl was titrated to a total dose of 75 leg, followed by propofol boluses of 1 mg/kg every two to three minutes as required to prevent movement and maintain spontaneous ventilation. There was no incidence of airway obstruction and no airway device was used.
Despite several attempts by three experienced practitioners, lumbar puncture was unsuccessful. A magnetic resonance imaging (MRI) scan was ordered to evaluate whether abnormal spinal anatomy might be the cause for the inability to obtain a successful lumbar puncture.
This patient was able to undergo an MRI of the brain and spine one day after unsuccessful lumbar puncture, without additional use of anaesthesia. Brain imaging showed many typical features of achondroplasia, but was notable for the absence of hydrocephalus and obstruction of the foramen magnum.
Imaging of the spine was completed in standard fashion using sagittal pre-contrast T1- and T2-weighted imaging with axial pre-contrast T1- and T2-weighted images obtained through the lumbar cistern. Post-contrast T1-weighted images were performed to exclude tumour involvement within the thecal sac (Figure 1 A-D). The pedicles of the lumbar spine and posterior arches were hypoplastic, causing spinal stenosis. A characteristic gibbus, kyphotic deformity was present at the L1-2 level. The presence of very mild disc protrusions and hypertrophy of the posterior longitudinal ligament and ligamentum flavum near the lumbar discs exacerbated the lumbar spinal stenosis. Axial T2-weighted images showed a markedly reduced size of the spinal canal with essentially no observable CSF around the nerve roots at the levels of the lumbar pedicles and discs. At the L1-2 disc, the spinal canal measured 6 mm by 9 mm. It was apparent from the signal and patchy enhancement of the central portions of the vertebral bodies that leukaemia extensively involved bone marrow of the spinal vertebral bodies. While posterior scalloping of the vertebral bodies in the lumbar region was noted, no pathologic fracture was identified. Epidural and subdural haemorrhages were not identified.
A neurosurgeon was consulted and was able to successfully place an Ommaya reservoir into the frontal horn of the right lateral ventricle of the brain, four days later, without complication. A nuclear medicine cisternogram was performed postoperatively using Indium 111 DTPA (0.404 mCi) (Figure 1 E). It showed good egress of radiotracer from the ventricles of the brain, with even distribution over the cerebral convexities and into the cerebrospinal fluid (CSF) spaces of the spinal canal, including the lumbar cistern. The patient was able to receive intrathecal chemotherapy without further attempts at lumbar puncture.
DISCUSSION
We present a case of a 17-year-old female patient with achondroplasia, ALL and asymptomatic spinal stenosis, from whom we were unsuccessful in collecting spinal fluid via a lumbar puncture. An MRI subsequently confirmed severe spinal stenosis with decreased CSF spaces around the nerve roots at the levels of the lumbar pedicles and discs. Experience in treating leukaemia patients with intrathecal chemotherapy is extensive at St. Jude Children's Research Hospital. Yet failure to access the lumbar cistern can occur in a variety of clinical situations, including the development of subdural haemorrhages in the lumbar cistern, the presence of epidural lipomatosis and spinal stenosis. In this patient, anatomical alterations in achondroplasia precluded diagnostic lumbar puncture. Since administration of intrathecal chemotherapy is an important component of the treatment of ALL (3), an Ommaya reservoir was subsequently inserted to allow the patient to complete therapy.
Achondroplasia is an autosomal dominant disorder that occurs at a rate of 0.5 to 1.5 per 10,000 live births(4). Failure of endochondral ossification of the skull base, vertebral bodies and long bones results in rhizomelic limb shortening (short proximal segments), macrocephaly, frontal bossing and midface hypoplasia (5).
Lumbar stenosis is common in patients with achondroplasia (6). Reduced spinal canal cross-sectional area is due to early fusion of the pedicles to the vertebral bodies causing shortened pedicles and decreased interpedicular distance (7). There is therefore a reduction in both the antero-posterior and transverse dimensions. Frequent occurrence of vertebral disc herniation as well as hypertrophy of the ligamentum flavum may also contribute to symptomatology (8).
A retrospective study found that 38% of adult patients with achondroplasia had clinical symptoms of spinal claudication and/or objective neurological abnormalities, and that the average age of onset of symptoms was 22 years (6). In another small study, the average age at the time of decompressive laminectomy was 33 years (9). Although our patient at 17 years of age was somewhat young to be developing this complication, surgery has been undertaken in patients as young as 10 years of ages (10).
There have been many reports of epidural anaesthesia in patients with achondroplasia (11-18), with some advocating a reduction in the volume of anaesthetic administered (13,18,19). Successful spinal anaesthesia has not been reported as many times as epidural anaesthesia. In one case report a spinal anaesthetic for caesarean section resulted in a partially successful block, requiring some supplementation after delivery of the baby (20). In that case, the patient had previously had lumbar laminectomy without fusion for the treatment of spinal stenosis. In a similar case, a patient having cesarean section was successfully given spinal anaesthesia using a microcatheter technique for titration of local anaesthetic dose (21). There has also been a report of a single-shot spinal anaesthetic using a standard volume and dose of local anaesthetic (14). On the other hand, one case series reported five spinal anaesthetics with one failure due to anatomical difficulties (22).
One study reported in the anaesthetic literature used MRI to examine conditions that may be associated with difficult neuraxial blockade. That study confirmed that the subarachnoid space may be difficult to identify on MRI in patients with spinal stenosis (23). The same study opined that not only may it be difficult to locate the subarachnoid space in such patients via lumbar puncture, but also that the cauda equina may be more easily damaged due to the reduced volume of surrounding CSF. Based on this information and the case we have described, we believe that MRI should be considered prior to attempting dural puncture in patients with achondroplasia, especially those with signs or symptoms of spinal stenosis.
We have presented a case of an achondroplastic patient with spinal stenosis so severe that no CSF was discernible around the nerve roots at the levels of the lumbar pedicles and discs upon MRI scan. We were unable to obtain CSF from this patient via lumbar puncture. We therefore recommend that if a lumbar puncture for the administration of spinal anaesthesia or for the recovery of clinical samples is contemplated for a patient with achondroplasia, that an MRI scan be performed prior to the procedure to ensure that the anatomical features of the insertion site are favourable to a successful outcome.
ACKNOWLEDGEMENTS
St. Jude Children's Research Hospital is funded by the American Lebanese Syrian Associated Charities. We wish to thank Dr Donald Samulack for editorial expertise, and Julie Groff for image preparation.
Accepted for publication on April 27, 2007.
REFERENCES
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(8.) Ferrante L, Acqui M, Mastronardi L, Celli P, Fortuna A. Stenosis of the spinal canal in achondroplasia. Ital J Neurol Sci 1991; 12:371-375.
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(19.) Brimacombe JR Caunt JA. Anaesthesia in a gravid achondroplastic dwarf. Anaesthesia 1990; 45:132-134.
(20.) DeRenzo JS, Vallejo MC, Ramanathan S. Failed regional anesthesia with reduced spinal bupivacaine dosage in a parturient with achondroplasia presenting for urgent cesarean section. Int J Obstet Anesth 2005; 14:175-178.
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L. L. BURGOYNE *, F. LANINGHAM (†), J. T. ZERO (‡), G. B. BIKHAZI (§), L. A. PEREIRAS **
Division of Anesthesiology, St. Jude Children's Research Hospital, Memphis Tennessee United States of America
* B.M., B.S., F.A.N.Z.C.A., Staff Physician.
(†) M.D., Assistant Member, Diagnostic Imaging, St. Jude Children's Research Hospital.
(‡) Student.
(§) M.D., Member.
** M.D., Assistant Member.
Address for reprints: Dr L. L. Burgoyne, Division of Anesthesiology, St Jude Children's Research Hospital, 332 N. Lauderdale St, Memphis, TN 38105-2794, U.S.A.
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| Author: | Burgoyne, L.L.; Laningham, F.; Zero, J.T.; Bikhazi, G.B.; Pereiras, L.A. |
|---|---|
| Publication: | Anaesthesia and Intensive Care |
| Article Type: | Clinical report |
| Geographic Code: | 1USA |
| Date: | Oct 1, 2007 |
| Words: | 2213 |
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