Brief Communications
under RA. In emergency situations, airway may be secured with intubating laryngeal mask airway or other supraglottic devices which require less manipulation of cervical joints.
CONCLUSION Regional anaesthesia in a case of KFS can be safely performed in surgeries where low level block is required. This report highlights the need for careful preoperative clinical and radiological spine evaluation to identify patients with potential difficulty and planning to manage the anticipated problems in performing RA.
Sukhyanti Kerai, KN Saxena, Bharti Taneja Department of Anaesthesiology and Critical Care, Maulana Azad Medical College and Associated Lok Nayak Hospital, New Delhi, India Address for correspondence: Dr. Sukhyanti Kerai, H. No. 53, Village Sultanpur Majra, Sultanpuri, New Delhi ‑ 110 086, India. E‑mail: [email protected]
REFERENCES Kaplan KM, Spivak JM, Bendo JA. Embryology of the spine and associated congenital abnormalities. Spine J 2005;5:564‑76. 2. Mahirogullari M, Ozkan H, Yildirim N, Cilli F, Güdemez E. Klippel‑Feil syndrome and associated congenital abnormalities: Evaluation of 23 cases. Acta Orthop Traumatol Turc 2006;40:234‑9. 3. Khawaja OM, Reed JT, Shaefi S, Chitilian HV, Sandberg WS. Crisis resource management of the airway in a patient with Klippel‑Feil syndrome, congenital deafness, and aortic dissection. Anesth Analg 2009;108:1220‑5. 4. Singh M, Prasad R, Jacob R. Anaesthetic challenges in a patient with Klippel-Feil syndrome undergoing surgery. Indian J Anaesth 2005;49:511‑4. 5. Hsu G, Manabat E, Huffnagle S, Huffnagle HJ. Anesthetic management of a parturient with type III Klippel‑Feil syndrome. Int J Obstet Anesth 2011;20:82‑5. 6. O’Connor PJ, Moysa GL, Finucane BT. Thoracic epidural anesthesia for bilateral reduction mammoplasty in a patient with Klippel‑Feil syndrome. Anesth Analg 2001;92:514‑6. 7. Smith KA, Ray AP. Epidural anesthesia for repeat cesarean delivery in a parturient with Klippel‑Feil syndrome. J Anaesthesiol Clin Pharmacol 2011;27:377‑9. 8. Strax TE, Baran E. Traumatic quadriplegia associated with Klippel‑Feil syndrome: Discussion and case reports. Arch Phys Med Rehabil 1975;56:363‑5
Anaesthetic management of a case of hereditary spherocytosis for splenectomy and cholecystectomy INTRODUCTION Hereditary spherocytosis (HS) is a familial haemolytic disorder characterised by the production of red blood cells (RBC) that are sphere‑shaped rather than bi‑concave disk shaped (doughnut‑shaped) and therefore more prone to haemolysis leading to chronic haemolytic anaemia. The incidence in Northern Europe and America varies from 1:2000 to 1:5000.[1] Patients with HS often present for surgery because of gall stones or for splenectomy. Exact incidence of HS in India is not known; however, the case is reported because of the challenges to the anaesthesiologist due to ‘sickling’ based complications. Peri‑operative management of HS should include blood transfusions, prevention of hypoxia, acidosis and temperature regulation.[2]
1.
Access this article online Quick response code Website: www.ijaweb.org
DOI: 10.4103/0019-5049.135081
Indian Journal of Anaesthesia | Vol. 58 | Issue 3 | May-Jun 2014
CASE REPORT We report a case of 28‑year‑old female weighing 40 kg, a known case of HS posted for laparoscopic splenectomy and cholecystectomy. She presented with pain abdomen and fever. She gave a history of jaundice since childhood. On physical examination, she had pallor, icteric sclera and splenomegaly. Rest of the systemic examination was normal. Her haemogram revealed haemoglobin (Hb) level of 6.2 g% and reticulocyte count of 18.5%. Peripheral blood smear showed spherocytes with increased osmotic fragility and Coombs test negative. Unconjugated bilirubin was 5.4 mg/dL, prothrombin time (PT), international normalised ratio (INR) was 27 s, and 2.0, respectively. Platelet count was 1.0 lakh/mm3. Rest of the liver function tests, creatinine and electrolytes revealed no abnormalities. Abdominal ultrasound revealed gall stones and splenomegaly. Patient was immunised with pneumococcal and haemophilus influenza vaccines. She was hydrated with 1.5 L/day for 2 days and received 5 units of packed cells, 5 units of fresh frozen plasma (FFP). Injection vitamin K (10mg, IV BID) was administered preoperatively for two days. Post‑transfusion, Hb was 9.9 g%, platelet count was 1.0 lakh/mm3 and PT, INR was 18.1 s and 343
Brief Communications
1.35, respectively. 3 units of FFP, 2 units of platelet concentrates and 2 units of packed cells volume were kept ready for intraoperative use. On the morning of surgery, venous access was secured with a wide bore 16G intravenous (i.v) canula and infusion of Ringer’s lactate started. She was pre‑medicated with injection glycopyrrolate and fentanyl. After pre‑oxygenation for 3 min, anaesthesia was induced with injection thiopentone sodium and succinylcholine was used to facilitate intubation which was done with 6.5 no. cuffed Portex tube. Anaesthesia was maintained with O2, N2O, fentanyl, isoflurane and vecuronium. End tidal CO2 was maintained between 30 and 35 mmHg. Intra‑operatively hypoxia, acidosis were avoided. Hypothermia was avoided by infusing warm i.v fluids, warm blood and by wrapping the patient’s extremities with cotton. Patient received 3 units of FFP, 2 units of platelet concentrates, 1 unit of packed cells after clamping of splenic vessels. Surgery lasted for 4 h. She received 2 litres of crystalloids and her urine output was 425 ml. Blood loss was 400 ml. At the end of the surgery, port sites were infiltrated with 0.25% bupivacaine. Trachea was extubated at the end of surgery. The patient received 1 unit of packed cells post‑operatively. I V paracetmol infusions and I V fentanyl were used to alleviate post‑operative pain.
DISCUSSION Hereditary spherocytosis is a heterogeneous form of haemolytic anaemia associated with the presence of spherocytes in the peripheral blood. Classically, the inheritance of HS is autosomal dominant and some severe forms are autosomal recessive.[3] In HS, erythrocyte shape changes are caused by defects in membrane protein spectrin resulting in cytoskeleton instability resulting in spherical RBCs. Spherocytic RBCs are removed rapidly from the circulation and destroyed by the spleen leading to anaemia, jaundice, enlarged spleen, and often gallstones. Moderate cases of HS may present in infancy with severe anaemia, hyperbilirubinemia, whereas mild cases are commonly seen in young adults or even later in life. Indeed, it is often the finding of gallstones in a young person that triggers diagnostic investigations. A characteristic feature of HS is an increase in mean corpuscular haemoglobin concentration (MCHC); this is almost the only condition in which an increased MCHC is seen. In most cases, the diagnosis can be made on the basis of 344
red cell morphology and abnormal osmotic fragility, a modified version of which is called the “pink test.” In some cases, a definitive diagnosis can be obtained only by molecular studies demonstrating a mutation in one of the genes underlying HS.[4] The commonly encountered complications of HS are haemolytic crisis, aplastic crisis and megaloblastic crisis. High RBC turnover and heightened erythroid marrow activity in HS makes children vulnerable to develop aplastic crisis due to parvovirus and various other infections. Death may also occur due to severe anaemia, heart failure, and cardiovascular collapse.[5] Folate therapy is recommended in severe and moderate HS in order to prevent megaloblastic crisis. Surgical treatment involves splenectomy, which results in cessation of haemolysis, return of the Hb to normal and clearance of jaundice. Surgery is indicated in all patients with splenomegaly to prevent gallstone formation and haemolytic crisis. Those who are well‑compensated and asymptomatic may be treated conservatively.[6] Immunisation with pneumococcal and haemophilus influenza vaccines should precede splenectomy. If gallstones are present, cholecystectomy may be performed simultaneously or at a later date.
CONCLUSION We could successfully manage the patient with HS posted for laparoscopic splenectomy and cholecystectomy. Peri‑operative management of HS largely depends on the severity of anaemia and the degree of haemolysis. Anaesthetic goals include avoidance of hypoxia, acidosis, and hypothermia. Vaccination before splenectomy is a must to prevent post‑operative infections.
Krishna Chaithanya, P Narasimha Reddy, Sangamitra Gandra, A Srikanth Department of Anaesthesiology, Narayana Medical College Hospital, Nellore, Andhra Pradesh, India Address for correspondence: Dr. Krishna Chaithanya, Department of Anaesthesiology, Narayana Medical College Hospital, Nellore, Andhra Pradesh, India. E‑mail: [email protected]
REFERENCES 1. 2.
Bolton‑Maggs PH, Stevens RF, Dodd NJ, Lamont G, Tittensor P, King MJ, et al. Guidelines for the diagnosis and management of hereditary spherocytosis. Br J Haematol 2004;126:455‑74. Shapiro ND, Poe MF. Sickle‑cell disease: An anesthesiological problem. Anesthesiology 1955;16:771‑80. Indian Journal of Anaesthesia | Vol. 58 | Issue 3 | May-Jun 2014
Brief Communications 3. 4. 5.
6.
Dacie J. The Haemolytic Anaemias. Vol. 86. London: Churchill Livingstone; 1995. p. 4650‑5. Grace RF, Lux SE. Disorders of the red cell membrane. In: Nathan and Oski’s Hematology of Infancy and Childhood. 7th ed. Philadelphia: Saunders; 2009. p. 659‑837. Mariani M, Barcellini W, Vercellati C, Marcello AP, Fermo E, Pedotti P, et al. Clinical and hematologic features of 300 patients affected by hereditary spherocytosis grouped according to the type of the membrane protein defect. Haematologica 2008;93:1310‑7. Firkin F, Chesterman C, Penington D, Rush B. The hemolytic anaemias. De Gruchy’s Clinical Haematology in Medical Practice. 5th ed. London: Blackwell Science; 1991. p. 182‑4. Access this article online Quick response code Website: www.ijaweb.org
DOI: 10.4103/0019-5049.135082
Colostomy in an ischiopagus, 3rd PND conjoined twins with cross‑circulation: Anaesthetic management
were admitted to our hospital for colostomy. They were actively moving limbs with a conjoined‑weight of 4.0 kg. The routine investigations were within normal limits and the clinical examination revealed common male genitalia and no external anal opening. X‑ray showed two separate spines and pelvic bones, which were apparently conjoined at ischii [Figure 2]. Plain and contrast enhanced computed tomography of the abdomen and pelvis suggested that both lung fields and mediastinal shadows were normal and the twins were fused along anterior abdominal wall as well as pelvis and perineum.[4] Right sided liver parenchyma could be seen separately in both twins, but left parenchyma were fused across the midline with contiguous intrahepatic channels. Multiple small bowel loops in continuation across the fused abdomen suggested their communication. Ureters were not visualised with single urinary bladder and normally appearing separate kidneys in both twins. Spinal columns appeared unfused with two separate pelvic bones. The twins in reference were referred to our centre from a peripheral
INTRODUCTION The ischiopagus variety of conjoined‑twins is the most complicated form of twinning, contributing only 6% to all with significant organ sharing[1] [Figure 1]. The type of the organ shared and the degree of cross‑circulation add to the complexity and determine the success rate of the separation surgery, if undertaken and the same factors also determine the success rate of any palliative surgery. As for unknown reasons the foetuses who survive to the point of birth are females and still‑borns are usually males, they follow a female predominance in the order of 3:1.[2] The incidence is 1:50,000-1:2,00,000 in general and is further higher in Indian and African race, that is 1:14,000‑25,000 live‑births.[3] We share our experience of the anaesthetic management for colostomy in tetrapus variety of ischiopagus with cross‑circulation, on their third postnatal day with an aim of thorough discussion of the anaesthetic considerations and implications of such anatomical abnormalities.
Figure 1: Conjoined-twins fused from their front pelvis
CASE REPORT Three day‑old male twins joined from their front pelvis Indian Journal of Anaesthesia | Vol. 58 | Issue 3 | May-Jun 2014
Figure 2: X-ray baby gram of ischiopagus 345
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.
under RA. In emergency situations, airway may be secured with intubating laryngeal mask airway or other supraglottic devices which require less manipulation of cervical joints.
CONCLUSION Regional anaesthesia in a case of KFS can be safely performed in surgeries where low level block is required. This report highlights the need for careful preoperative clinical and radiological spine evaluation to identify patients with potential difficulty and planning to manage the anticipated problems in performing RA.
Sukhyanti Kerai, KN Saxena, Bharti Taneja Department of Anaesthesiology and Critical Care, Maulana Azad Medical College and Associated Lok Nayak Hospital, New Delhi, India Address for correspondence: Dr. Sukhyanti Kerai, H. No. 53, Village Sultanpur Majra, Sultanpuri, New Delhi ‑ 110 086, India. E‑mail: [email protected]
REFERENCES Kaplan KM, Spivak JM, Bendo JA. Embryology of the spine and associated congenital abnormalities. Spine J 2005;5:564‑76. 2. Mahirogullari M, Ozkan H, Yildirim N, Cilli F, Güdemez E. Klippel‑Feil syndrome and associated congenital abnormalities: Evaluation of 23 cases. Acta Orthop Traumatol Turc 2006;40:234‑9. 3. Khawaja OM, Reed JT, Shaefi S, Chitilian HV, Sandberg WS. Crisis resource management of the airway in a patient with Klippel‑Feil syndrome, congenital deafness, and aortic dissection. Anesth Analg 2009;108:1220‑5. 4. Singh M, Prasad R, Jacob R. Anaesthetic challenges in a patient with Klippel-Feil syndrome undergoing surgery. Indian J Anaesth 2005;49:511‑4. 5. Hsu G, Manabat E, Huffnagle S, Huffnagle HJ. Anesthetic management of a parturient with type III Klippel‑Feil syndrome. Int J Obstet Anesth 2011;20:82‑5. 6. O’Connor PJ, Moysa GL, Finucane BT. Thoracic epidural anesthesia for bilateral reduction mammoplasty in a patient with Klippel‑Feil syndrome. Anesth Analg 2001;92:514‑6. 7. Smith KA, Ray AP. Epidural anesthesia for repeat cesarean delivery in a parturient with Klippel‑Feil syndrome. J Anaesthesiol Clin Pharmacol 2011;27:377‑9. 8. Strax TE, Baran E. Traumatic quadriplegia associated with Klippel‑Feil syndrome: Discussion and case reports. Arch Phys Med Rehabil 1975;56:363‑5
Anaesthetic management of a case of hereditary spherocytosis for splenectomy and cholecystectomy INTRODUCTION Hereditary spherocytosis (HS) is a familial haemolytic disorder characterised by the production of red blood cells (RBC) that are sphere‑shaped rather than bi‑concave disk shaped (doughnut‑shaped) and therefore more prone to haemolysis leading to chronic haemolytic anaemia. The incidence in Northern Europe and America varies from 1:2000 to 1:5000.[1] Patients with HS often present for surgery because of gall stones or for splenectomy. Exact incidence of HS in India is not known; however, the case is reported because of the challenges to the anaesthesiologist due to ‘sickling’ based complications. Peri‑operative management of HS should include blood transfusions, prevention of hypoxia, acidosis and temperature regulation.[2]
1.
Access this article online Quick response code Website: www.ijaweb.org
DOI: 10.4103/0019-5049.135081
Indian Journal of Anaesthesia | Vol. 58 | Issue 3 | May-Jun 2014
CASE REPORT We report a case of 28‑year‑old female weighing 40 kg, a known case of HS posted for laparoscopic splenectomy and cholecystectomy. She presented with pain abdomen and fever. She gave a history of jaundice since childhood. On physical examination, she had pallor, icteric sclera and splenomegaly. Rest of the systemic examination was normal. Her haemogram revealed haemoglobin (Hb) level of 6.2 g% and reticulocyte count of 18.5%. Peripheral blood smear showed spherocytes with increased osmotic fragility and Coombs test negative. Unconjugated bilirubin was 5.4 mg/dL, prothrombin time (PT), international normalised ratio (INR) was 27 s, and 2.0, respectively. Platelet count was 1.0 lakh/mm3. Rest of the liver function tests, creatinine and electrolytes revealed no abnormalities. Abdominal ultrasound revealed gall stones and splenomegaly. Patient was immunised with pneumococcal and haemophilus influenza vaccines. She was hydrated with 1.5 L/day for 2 days and received 5 units of packed cells, 5 units of fresh frozen plasma (FFP). Injection vitamin K (10mg, IV BID) was administered preoperatively for two days. Post‑transfusion, Hb was 9.9 g%, platelet count was 1.0 lakh/mm3 and PT, INR was 18.1 s and 343
Brief Communications
1.35, respectively. 3 units of FFP, 2 units of platelet concentrates and 2 units of packed cells volume were kept ready for intraoperative use. On the morning of surgery, venous access was secured with a wide bore 16G intravenous (i.v) canula and infusion of Ringer’s lactate started. She was pre‑medicated with injection glycopyrrolate and fentanyl. After pre‑oxygenation for 3 min, anaesthesia was induced with injection thiopentone sodium and succinylcholine was used to facilitate intubation which was done with 6.5 no. cuffed Portex tube. Anaesthesia was maintained with O2, N2O, fentanyl, isoflurane and vecuronium. End tidal CO2 was maintained between 30 and 35 mmHg. Intra‑operatively hypoxia, acidosis were avoided. Hypothermia was avoided by infusing warm i.v fluids, warm blood and by wrapping the patient’s extremities with cotton. Patient received 3 units of FFP, 2 units of platelet concentrates, 1 unit of packed cells after clamping of splenic vessels. Surgery lasted for 4 h. She received 2 litres of crystalloids and her urine output was 425 ml. Blood loss was 400 ml. At the end of the surgery, port sites were infiltrated with 0.25% bupivacaine. Trachea was extubated at the end of surgery. The patient received 1 unit of packed cells post‑operatively. I V paracetmol infusions and I V fentanyl were used to alleviate post‑operative pain.
DISCUSSION Hereditary spherocytosis is a heterogeneous form of haemolytic anaemia associated with the presence of spherocytes in the peripheral blood. Classically, the inheritance of HS is autosomal dominant and some severe forms are autosomal recessive.[3] In HS, erythrocyte shape changes are caused by defects in membrane protein spectrin resulting in cytoskeleton instability resulting in spherical RBCs. Spherocytic RBCs are removed rapidly from the circulation and destroyed by the spleen leading to anaemia, jaundice, enlarged spleen, and often gallstones. Moderate cases of HS may present in infancy with severe anaemia, hyperbilirubinemia, whereas mild cases are commonly seen in young adults or even later in life. Indeed, it is often the finding of gallstones in a young person that triggers diagnostic investigations. A characteristic feature of HS is an increase in mean corpuscular haemoglobin concentration (MCHC); this is almost the only condition in which an increased MCHC is seen. In most cases, the diagnosis can be made on the basis of 344
red cell morphology and abnormal osmotic fragility, a modified version of which is called the “pink test.” In some cases, a definitive diagnosis can be obtained only by molecular studies demonstrating a mutation in one of the genes underlying HS.[4] The commonly encountered complications of HS are haemolytic crisis, aplastic crisis and megaloblastic crisis. High RBC turnover and heightened erythroid marrow activity in HS makes children vulnerable to develop aplastic crisis due to parvovirus and various other infections. Death may also occur due to severe anaemia, heart failure, and cardiovascular collapse.[5] Folate therapy is recommended in severe and moderate HS in order to prevent megaloblastic crisis. Surgical treatment involves splenectomy, which results in cessation of haemolysis, return of the Hb to normal and clearance of jaundice. Surgery is indicated in all patients with splenomegaly to prevent gallstone formation and haemolytic crisis. Those who are well‑compensated and asymptomatic may be treated conservatively.[6] Immunisation with pneumococcal and haemophilus influenza vaccines should precede splenectomy. If gallstones are present, cholecystectomy may be performed simultaneously or at a later date.
CONCLUSION We could successfully manage the patient with HS posted for laparoscopic splenectomy and cholecystectomy. Peri‑operative management of HS largely depends on the severity of anaemia and the degree of haemolysis. Anaesthetic goals include avoidance of hypoxia, acidosis, and hypothermia. Vaccination before splenectomy is a must to prevent post‑operative infections.
Krishna Chaithanya, P Narasimha Reddy, Sangamitra Gandra, A Srikanth Department of Anaesthesiology, Narayana Medical College Hospital, Nellore, Andhra Pradesh, India Address for correspondence: Dr. Krishna Chaithanya, Department of Anaesthesiology, Narayana Medical College Hospital, Nellore, Andhra Pradesh, India. E‑mail: [email protected]
REFERENCES 1. 2.
Bolton‑Maggs PH, Stevens RF, Dodd NJ, Lamont G, Tittensor P, King MJ, et al. Guidelines for the diagnosis and management of hereditary spherocytosis. Br J Haematol 2004;126:455‑74. Shapiro ND, Poe MF. Sickle‑cell disease: An anesthesiological problem. Anesthesiology 1955;16:771‑80. Indian Journal of Anaesthesia | Vol. 58 | Issue 3 | May-Jun 2014
Brief Communications 3. 4. 5.
6.
Dacie J. The Haemolytic Anaemias. Vol. 86. London: Churchill Livingstone; 1995. p. 4650‑5. Grace RF, Lux SE. Disorders of the red cell membrane. In: Nathan and Oski’s Hematology of Infancy and Childhood. 7th ed. Philadelphia: Saunders; 2009. p. 659‑837. Mariani M, Barcellini W, Vercellati C, Marcello AP, Fermo E, Pedotti P, et al. Clinical and hematologic features of 300 patients affected by hereditary spherocytosis grouped according to the type of the membrane protein defect. Haematologica 2008;93:1310‑7. Firkin F, Chesterman C, Penington D, Rush B. The hemolytic anaemias. De Gruchy’s Clinical Haematology in Medical Practice. 5th ed. London: Blackwell Science; 1991. p. 182‑4. Access this article online Quick response code Website: www.ijaweb.org
DOI: 10.4103/0019-5049.135082
Colostomy in an ischiopagus, 3rd PND conjoined twins with cross‑circulation: Anaesthetic management
were admitted to our hospital for colostomy. They were actively moving limbs with a conjoined‑weight of 4.0 kg. The routine investigations were within normal limits and the clinical examination revealed common male genitalia and no external anal opening. X‑ray showed two separate spines and pelvic bones, which were apparently conjoined at ischii [Figure 2]. Plain and contrast enhanced computed tomography of the abdomen and pelvis suggested that both lung fields and mediastinal shadows were normal and the twins were fused along anterior abdominal wall as well as pelvis and perineum.[4] Right sided liver parenchyma could be seen separately in both twins, but left parenchyma were fused across the midline with contiguous intrahepatic channels. Multiple small bowel loops in continuation across the fused abdomen suggested their communication. Ureters were not visualised with single urinary bladder and normally appearing separate kidneys in both twins. Spinal columns appeared unfused with two separate pelvic bones. The twins in reference were referred to our centre from a peripheral
INTRODUCTION The ischiopagus variety of conjoined‑twins is the most complicated form of twinning, contributing only 6% to all with significant organ sharing[1] [Figure 1]. The type of the organ shared and the degree of cross‑circulation add to the complexity and determine the success rate of the separation surgery, if undertaken and the same factors also determine the success rate of any palliative surgery. As for unknown reasons the foetuses who survive to the point of birth are females and still‑borns are usually males, they follow a female predominance in the order of 3:1.[2] The incidence is 1:50,000-1:2,00,000 in general and is further higher in Indian and African race, that is 1:14,000‑25,000 live‑births.[3] We share our experience of the anaesthetic management for colostomy in tetrapus variety of ischiopagus with cross‑circulation, on their third postnatal day with an aim of thorough discussion of the anaesthetic considerations and implications of such anatomical abnormalities.
Figure 1: Conjoined-twins fused from their front pelvis
CASE REPORT Three day‑old male twins joined from their front pelvis Indian Journal of Anaesthesia | Vol. 58 | Issue 3 | May-Jun 2014
Figure 2: X-ray baby gram of ischiopagus 345
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.
