Letters to Editor
Anaesthetic management of a patient with Jarcho–Levin syndrome Sir, Jarcho–Levin syndrome (JLS) is a rare, congenital, inherited costovertebral dysplasia with an estimated global incidence of 1/40,000 births.[1,2] Though exact incidence in Indian context is not known, there are few case reports describing it in Indian population.[3] We report a case of JLS, associated anaesthetic challenges and their management. A 12‑month‑old male child with JLS presented with left inguinal hernia and was scheduled for herniotomy. The history was significant, with presence of abnormal position of lower limbs, protuberant abdomen at birth, recurrent chest infections and cyanosis on crying. The child had short stature with normal limb length, kyphoscoliosis and shield‑like chest. His height was 60 cm, arm span 66 cm, upper segment to lower segment ratio 1.2, weight 6.8 kg, length/age below 3 standard deviation (SD) and weight/height within 1 SD. On examination, there was no upper chest movement and slight (1–1.2 cm) lower chest movement with respiration. Mouth opening was 2 cm with bulky tongue, short neck, and restricted neck mobility. Radiography showed fused cervical vertebrae [Figure 1], crowded ribs (crab‑like or fan‑like) due to posterior fusion, without any intrinsic rib abnormality [Figure 2a] and hemivertebrae (pebble beach) in thoracolumbar spine [Figure 2b]. Abdominopelvic ultrasonography and two‑dimensional echocardiogram were normal. Other investigations were within normal limits.
Patient was posted for herniotomy; in view of vertebral anomalies, neuraxial blockade was ruled out, and general anaesthesia was planned. Since difficult intubation was anticipated, difficult airway cart was kept ready. Pre‑operative oxygen saturation (SpO2) on room air was 90%, pulse rate 130/min and blood pressure 96/70 mm Hg. Intravenous tramadol 12 mg was given as pre‑medication through a 24‑gauge cannula already in situ. Patient was pre‑oxygenated, and SpO2 of 100% was achieved, followed by inhalational induction with halothane and O2. Laryngoscopy was attempted with spontaneous respiration and Cormack and Lehane grade 3 was noted. Patient could be intubated with a 4 mm uncuffed endotracheal tube using stylet; bilateral air entry and tube placement (by capnography) were confirmed. Patient was kept on volume controlled ventilation, and anaesthesia was maintained with O2 and isoflurane. Injection atracurium was used for muscle relaxation. Intraoperative vitals remained stable. After completion of surgery, patient was reversed with injection neostigmine and glycopyrrolate and extubated after return of airway reflexes and adequate muscle power. Post‑operative SpO2 was 100% on oxygen by ventimask. Rest of the post‑operative period was uneventful and patient was discharged on 3rd post‑operative day. On the follow‑up after 2 months patient was healthy. Jarcho–Levin syndrome is skeletal dysplasia with a short trunk. It is of two subtypes, spondylothoracic dysostosis (STD) and spondylocostal dysostosis (SCD). Associated anomalies are hernias, neural tube defects, urogenital and anal anomalies. [4] Patients with STD have vertebral anomalies with a ‘fan‑like’ or ‘crab‑like’ rib configuration, a higher incidence of neural tube defects and a higher mortality
a
Figure 1: X‑ray neck lateral view showing fused cervical vertebrae 322
b
Figure 2: (a) Chest X‑ray PA view showing crowding of ribs characteristic ‘crab‑like’ appearance of ribs. (b) X‑ray thoracic and lumbar spine showing ‘pebble beach’ appearance due to hemi vertebrae Indian Journal of Anaesthesia | Vol. 59 | Issue 5 | May 2015
Letters to Editor
rate and inherited in autosomal recessive manner. SCD patients have vertebral anomalies with marked intrinsic rib abnormalities such as absent ribs, abnormal orientation, irregularity of shape and size, bifurcation, broadening, fusion of ribs, etc. They are often short statured, have higher survival rate, less likely to have associated neural tube defects and inherited in either autosomal recessive or autosomal dominant manner.[5] There is no definitive treatment of this syndrome and management is directed towards prevention of respiratory infection and symptomatic support. Our patient had features of STD. This syndrome may pose multiple challenges to anaesthesiologist such as difficulty in airway management, respiratory compromise and problems posed by associated anomalies. As these patients have recurrent respiratory infections, perioperative assessment and management of these complications is of prime importance.[6] Cyanosis while crying in the absence of cyanotic heart disease may be seen due to collapse of the trachea owing to the absence of tracheal cartilaginous rings.[4] Fusion of cervical vertebra and kyphoscoliosis pose a special challenge during airway management and contribute to intubation difficulty. Supraglottic airway devices can be of great help specially in centres without facility for fiberoptic intubation.[7] As we were able to intubate the patient in first go, we did not use supraglottic device. Inhalational induction is preferred as it allows intubation in spontaneously breathing patients. Pre‑operative sedatives and opioid analgesics should not be used to avoid respiratory depression. We avoided the use of nitrous oxide as these patients may have associated pulmonary hypertension.[8] Post‑operative mechanical ventilation may be needed because of the presence of kyphoscoliosis and decreased chest wall compliance. The perioperative morbidity and mortality of these patients is mainly related to pulmonary consequences of thoracic skeletal dysplasia and associated anomalies. Adequate care during pre‑operative period, anaesthetic management and post‑operative period are imperative in successful outcomes in patients with Jarcho–Levin syndrome. [This article is devoted to the memory of Dr. Priya Verma.]
Neena Jain, Pooja Mathur, Priya Verma, Arvind Khare Department of Anaesthesiology, Jawaharlal Nehru Medical College, Ajmer, Rajasthan, India Indian Journal of Anaesthesia | Vol. 59 | Issue 5 | May 2015
Address for correspondence: Dr. Pooja Mathur, Assistant Professor, Department of Anaesthesiology, Jawaharlal Nehru Medical College, Ajmer ‑ 305 001, Rajasthan, India. E‑mail: [email protected]
REFERENCES 1. Odéhouri‑Koudou TH, Yaokreh JB, Tembély S, Dick RK. Sporadic occurrence of Jarcho‑Levin syndrome in an ivorian newborn. Case Rep Orthop 2013;2013:129625. 2. Jarcho S, Levin PM. Hereditary malformations of the vertebral bodies. Bull Johns Hopkins Hosp 1938;62:216‑26. 3. Kansal R, Mahore A, Kukreja S. Jarcho‑Levin syndrome with diastematomyelia: A case report and review of literature. J Pediatr Neurosci 2011;6:141‑3. 4. Schulman M, Gonzalez MT, Bye MR. Airway abnormalities in Jarcho‑Levin syndrome: A report of two cases. J Med Genet 1993;30:875‑6. 5. Butler MG, Hayes BG, Hathaway MM, Begleiter ML. Specific genetic diseases at risk for sedation/anesthesia complications. Anesth Analg 2000;91:837‑55. 6. Kulkarni ML, Navaz SR, Vani HN, Manjunath KS, Matani D. Jarcho‑Levin syndrome. Indian J Pediatr 2006;73:245‑7. 7. Ramesh S, Jayanthi R. Supraglottic airway devices in children. Indian J Anaesth 2011;55:476‑82. 8. Rastogi D, Rosenzweig EB, Koumbourlis A. Pulmonary hypertension in Jarcho‑Levin syndrome. Am J Med Genet 2002;107:250‑2. Access this article online Quick response code Website: www.ijaweb.org
DOI: 10.4103/0019-5049.156892
Cisatracurium degradation: Intravenous fluid warmer the culprit? Sir, Cisatracurium is a non‑depolarising muscle relaxant that undergoes degradation in plasma at physiological pH and temperature by organ‑independent Hofmann elimination.[1] This accounts for 77% of its overall elimination.[2,3] Its metabolites (laudanosine and a monoquaternary acrylate) do not possess any intrinsic neuromuscular blocking property.[1] Cisatracurium is not hydrolysed by plasma esterase directly.[4] It has been noted that the rate of cisatracurium degradation increases 6.5 fold with an increase in pH from 6.4 to 7.8.[4] 323
Copyright of Indian Journal of Anaesthesia is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Anaesthetic management of a patient with Jarcho–Levin syndrome Sir, Jarcho–Levin syndrome (JLS) is a rare, congenital, inherited costovertebral dysplasia with an estimated global incidence of 1/40,000 births.[1,2] Though exact incidence in Indian context is not known, there are few case reports describing it in Indian population.[3] We report a case of JLS, associated anaesthetic challenges and their management. A 12‑month‑old male child with JLS presented with left inguinal hernia and was scheduled for herniotomy. The history was significant, with presence of abnormal position of lower limbs, protuberant abdomen at birth, recurrent chest infections and cyanosis on crying. The child had short stature with normal limb length, kyphoscoliosis and shield‑like chest. His height was 60 cm, arm span 66 cm, upper segment to lower segment ratio 1.2, weight 6.8 kg, length/age below 3 standard deviation (SD) and weight/height within 1 SD. On examination, there was no upper chest movement and slight (1–1.2 cm) lower chest movement with respiration. Mouth opening was 2 cm with bulky tongue, short neck, and restricted neck mobility. Radiography showed fused cervical vertebrae [Figure 1], crowded ribs (crab‑like or fan‑like) due to posterior fusion, without any intrinsic rib abnormality [Figure 2a] and hemivertebrae (pebble beach) in thoracolumbar spine [Figure 2b]. Abdominopelvic ultrasonography and two‑dimensional echocardiogram were normal. Other investigations were within normal limits.
Patient was posted for herniotomy; in view of vertebral anomalies, neuraxial blockade was ruled out, and general anaesthesia was planned. Since difficult intubation was anticipated, difficult airway cart was kept ready. Pre‑operative oxygen saturation (SpO2) on room air was 90%, pulse rate 130/min and blood pressure 96/70 mm Hg. Intravenous tramadol 12 mg was given as pre‑medication through a 24‑gauge cannula already in situ. Patient was pre‑oxygenated, and SpO2 of 100% was achieved, followed by inhalational induction with halothane and O2. Laryngoscopy was attempted with spontaneous respiration and Cormack and Lehane grade 3 was noted. Patient could be intubated with a 4 mm uncuffed endotracheal tube using stylet; bilateral air entry and tube placement (by capnography) were confirmed. Patient was kept on volume controlled ventilation, and anaesthesia was maintained with O2 and isoflurane. Injection atracurium was used for muscle relaxation. Intraoperative vitals remained stable. After completion of surgery, patient was reversed with injection neostigmine and glycopyrrolate and extubated after return of airway reflexes and adequate muscle power. Post‑operative SpO2 was 100% on oxygen by ventimask. Rest of the post‑operative period was uneventful and patient was discharged on 3rd post‑operative day. On the follow‑up after 2 months patient was healthy. Jarcho–Levin syndrome is skeletal dysplasia with a short trunk. It is of two subtypes, spondylothoracic dysostosis (STD) and spondylocostal dysostosis (SCD). Associated anomalies are hernias, neural tube defects, urogenital and anal anomalies. [4] Patients with STD have vertebral anomalies with a ‘fan‑like’ or ‘crab‑like’ rib configuration, a higher incidence of neural tube defects and a higher mortality
a
Figure 1: X‑ray neck lateral view showing fused cervical vertebrae 322
b
Figure 2: (a) Chest X‑ray PA view showing crowding of ribs characteristic ‘crab‑like’ appearance of ribs. (b) X‑ray thoracic and lumbar spine showing ‘pebble beach’ appearance due to hemi vertebrae Indian Journal of Anaesthesia | Vol. 59 | Issue 5 | May 2015
Letters to Editor
rate and inherited in autosomal recessive manner. SCD patients have vertebral anomalies with marked intrinsic rib abnormalities such as absent ribs, abnormal orientation, irregularity of shape and size, bifurcation, broadening, fusion of ribs, etc. They are often short statured, have higher survival rate, less likely to have associated neural tube defects and inherited in either autosomal recessive or autosomal dominant manner.[5] There is no definitive treatment of this syndrome and management is directed towards prevention of respiratory infection and symptomatic support. Our patient had features of STD. This syndrome may pose multiple challenges to anaesthesiologist such as difficulty in airway management, respiratory compromise and problems posed by associated anomalies. As these patients have recurrent respiratory infections, perioperative assessment and management of these complications is of prime importance.[6] Cyanosis while crying in the absence of cyanotic heart disease may be seen due to collapse of the trachea owing to the absence of tracheal cartilaginous rings.[4] Fusion of cervical vertebra and kyphoscoliosis pose a special challenge during airway management and contribute to intubation difficulty. Supraglottic airway devices can be of great help specially in centres without facility for fiberoptic intubation.[7] As we were able to intubate the patient in first go, we did not use supraglottic device. Inhalational induction is preferred as it allows intubation in spontaneously breathing patients. Pre‑operative sedatives and opioid analgesics should not be used to avoid respiratory depression. We avoided the use of nitrous oxide as these patients may have associated pulmonary hypertension.[8] Post‑operative mechanical ventilation may be needed because of the presence of kyphoscoliosis and decreased chest wall compliance. The perioperative morbidity and mortality of these patients is mainly related to pulmonary consequences of thoracic skeletal dysplasia and associated anomalies. Adequate care during pre‑operative period, anaesthetic management and post‑operative period are imperative in successful outcomes in patients with Jarcho–Levin syndrome. [This article is devoted to the memory of Dr. Priya Verma.]
Neena Jain, Pooja Mathur, Priya Verma, Arvind Khare Department of Anaesthesiology, Jawaharlal Nehru Medical College, Ajmer, Rajasthan, India Indian Journal of Anaesthesia | Vol. 59 | Issue 5 | May 2015
Address for correspondence: Dr. Pooja Mathur, Assistant Professor, Department of Anaesthesiology, Jawaharlal Nehru Medical College, Ajmer ‑ 305 001, Rajasthan, India. E‑mail: [email protected]
REFERENCES 1. Odéhouri‑Koudou TH, Yaokreh JB, Tembély S, Dick RK. Sporadic occurrence of Jarcho‑Levin syndrome in an ivorian newborn. Case Rep Orthop 2013;2013:129625. 2. Jarcho S, Levin PM. Hereditary malformations of the vertebral bodies. Bull Johns Hopkins Hosp 1938;62:216‑26. 3. Kansal R, Mahore A, Kukreja S. Jarcho‑Levin syndrome with diastematomyelia: A case report and review of literature. J Pediatr Neurosci 2011;6:141‑3. 4. Schulman M, Gonzalez MT, Bye MR. Airway abnormalities in Jarcho‑Levin syndrome: A report of two cases. J Med Genet 1993;30:875‑6. 5. Butler MG, Hayes BG, Hathaway MM, Begleiter ML. Specific genetic diseases at risk for sedation/anesthesia complications. Anesth Analg 2000;91:837‑55. 6. Kulkarni ML, Navaz SR, Vani HN, Manjunath KS, Matani D. Jarcho‑Levin syndrome. Indian J Pediatr 2006;73:245‑7. 7. Ramesh S, Jayanthi R. Supraglottic airway devices in children. Indian J Anaesth 2011;55:476‑82. 8. Rastogi D, Rosenzweig EB, Koumbourlis A. Pulmonary hypertension in Jarcho‑Levin syndrome. Am J Med Genet 2002;107:250‑2. Access this article online Quick response code Website: www.ijaweb.org
DOI: 10.4103/0019-5049.156892
Cisatracurium degradation: Intravenous fluid warmer the culprit? Sir, Cisatracurium is a non‑depolarising muscle relaxant that undergoes degradation in plasma at physiological pH and temperature by organ‑independent Hofmann elimination.[1] This accounts for 77% of its overall elimination.[2,3] Its metabolites (laudanosine and a monoquaternary acrylate) do not possess any intrinsic neuromuscular blocking property.[1] Cisatracurium is not hydrolysed by plasma esterase directly.[4] It has been noted that the rate of cisatracurium degradation increases 6.5 fold with an increase in pH from 6.4 to 7.8.[4] 323
Copyright of Indian Journal of Anaesthesia is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
