- Department of Surgery, College of Medicine, University of Ibadan, Ibadan, Nigeria
- Department of Anaesthesia, College of Medicine, University of Ibadan, Ibadan, Nigeria
- Department of Haematology, College of Medicine, University of Ibadan, Ibadan, Nigeria
Matthew T. Shokunbi
Department of Surgery, College of Medicine, University of Ibadan, Ibadan, Nigeria
DOI:10.4103/sni.sni_180_18Copyright: © 2019 Surgical Neurology International This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
How to cite this article: Oluwakemi A. Badejo, Olusola K. Idowu, James A. Balogun, Wuraola A. Shokunbi, Simbo D. Amanor-Boadu, Matthew T. Shokunbi. Outcome of cranial surgery in Nigerian patients with hemoglobinopathies: A retrospective study. 06-Feb-2019;10:16
How to cite this URL: Oluwakemi A. Badejo, Olusola K. Idowu, James A. Balogun, Wuraola A. Shokunbi, Simbo D. Amanor-Boadu, Matthew T. Shokunbi. Outcome of cranial surgery in Nigerian patients with hemoglobinopathies: A retrospective study. 06-Feb-2019;10:16. Available from: http://surgicalneurologyint.com/surgicalint-articles/9200/
Background:Surgical intervention in patients with hemoglobinopathies has been extensively reviewed in the literature, but information on the outcome of cranial surgery in this patient population in sub-Saharan Africa is limited.
Methods:This is a retrospective study of patients with hemoglobinopathies, who underwent brain surgery in our facility. The review covered a 5-year period. We examined patient- and surgery-related variables and described the surgical complications as well as the 60-day mortality.
Results:A total of nine procedures (eight under general anesthesia and one under local anesthesia) were performed on seven patients with hemoglobinopathy during the study period. Eight (88.9%) of these were done in female patients and one (11.1%) in a male patient. Six (66.7%) were performed in patients with no previous history of blood transfusion. Hb SC accounted for five (55.6%), Hb SS for three (33.3%), and Hb CC for one (11.1%) procedure, respectively. Three (33.3%) of these procedures were brain tumor-related, three (33.3%) trauma-related, one (11.1%) cosmetic, one (11.1%) vascular, and one for a postoperative complication. Only one (11.1%) procedure was associated with preoperative blood transfusion, whereas there was a need for blood transfusion following five (55.6%) of the procedures. There was a mortality rate of 11.1% (1 case). Other complications were recorded after three (33.3%) of the procedures and none with five (55.6%) of the procedures.
Conclusion:Neurosurgery is possible and safe in patients with hemoglobin disorders. Adequate preoperative preparation, proper anesthetic techniques, meticulous surgery, and excellent postoperative care can help optimize outcome of surgical intervention in this patient population.
Keywords: Blood transfusion, hemoglobinopathy, neurosurgery, sickle cell disease
Hemoglobinopathies are autosomal recessive hematological genetic disorders characterized by production of abnormal hemoglobin. These include structural defects of the beta globin chain (hemoglobin variants) and quantitative abnormalities of alpha or beta-globin chains (thalassemia syndromes).[
This group of patients may require surgical intervention during the course of their lifetime, either as a direct consequence of their disease or from unrelated causes. The surgical management of patients with sickle cell disease poses a daunting challenge because of a variety of factors. These include low blood reserve, reduced oxygen-carrying capacity of the abnormal red blood cells under anesthesia, increased susceptibility to infections, the high tendency for thromboembolic events, and the potential for precipitating sickle cell crisis.[
We performed a retrospective review of all patients with hemoglobinopathy, who underwent cranial neurosurgical procedures in our facility between January 1, 2013 and January 1, 2018. Data extracted from their medical records include their biodata, type of hemoglobinopathy, history of previous surgeries, comorbidities, past blood transfusion, previous sickle cell crisis, and form. Compliance with routine medications, neurosurgical diagnosis, steady-state packed cell volume, type of anesthesia, and neurosurgical procedure done were recorded. We also documented the estimated intraoperative blood loss, pre/postoperative packed cell volume, intra/postoperative blood transfusion, duration of surgery, the period of intensive care unit admission if any, length of hospitalization, complications, and outcome. These were all recorded and analyzed. The primary outcome measure was the 60-day mortality, and the secondary outcome measures were the procedure-related complications.
We performed 1245 neurosurgical procedures during the study period. There were seven patients with hemoglobin disorders, who had nine (0.72%) procedures during this period.
Right temporal tumor: axial cranial images. (a) Preoperative: cranial magnetic resonance imaging (T1, contrast-enhanced) showing lobulated, partly cystic tumor. Histologically diagnosed as anaplastic astrocytoma. (b) Postoperative: cranial computerized tomography scan (contrast-enhanced); gross near-total resection
Right cerebral hemispheric chronic subdural hematoma; axial images, cranial computerized tomography scan. (a) Preoperative: mixed hypo- and isodense subdural collection (white arrow) with substantial mass effect (white arrow head). (b) Postoperative (6 weeks later): total evacuation of hematoma and restoration of brain midline (white arrow head)
Hemoglobinopathy remains a disease of global health importance.[
Great care must be taken to avoid factors that can predispose patients to sickle cell crisis in the preoperative period. These include hypoxia, acidosis, and hypothermia.[
Based on our experience with our patients, we suggest that preoperative workup of patients with sickle cell disease includes complete blood count, serum electrolytes, coagulation profile, and adequate hydration (to prevent vaso-occlusive crisis). Preoperative blood transfusion should be reserved for patients with hematocrits below their known steady-state levels or less than 30% in those whose steady-state levels are unknown. These patients should have preoxygenation to 100% prior to commencement of induction of anesthesia and adequate oxygenation must be maintained throughout the surgery. Intraoperative measures should include blood transfusion if blood loss exceeds 500 ml or if there is evidence of hemodynamic instability. It is important to prevent hypothermia and achieve optimal pain control in these patients to forestall sickle cell disease-related crises. Given the hypercoagulable states of these patients and the increased risk of thromboembolism, we advocate institution of nonpharmacologic deep venous thrombosis prophylaxis techniques and early ambulation when not contraindicated. Prophylactic administration of broad spectrum intravenous antibiotics and (intravenous) antibiotics coverage continued up to 48 h after surgery should be employed to prevent postoperative sepsis.
Various publications have shed light on the surgical outcomes in patients with sickle cell disease, with most of these involving orthopedic, general surgical, and otorhinolaryngological procedures.[
All the nondeath complications in our patients were successfully managed. Only one patient in our series had postoperative sepsis, which was related to a preoperative infected, plated, and malunited right femoral fracture. He subsequently had implant removal and modified Belfast procedure by the orthopedic team. The presence of comorbidities did not reflect negatively on the outcome in our series. One of the patients had vaso-occlusive crisis following debridement and elevation of an open depressed right frontal fracture, which was secondary to preoperative sepsis. She in fact had a vaso-occlusive crisis in the immediate preoperative period. The mortality was in a 50-year-old Hb SC patient, with a giant pituitary tumor who had an endoscopic transsphenoidal resection, which was terminated on account of excessive intraoperative blood loss (1500 ml). She was transfused with three units of whole blood and a unit of fresh frozen plasma. The patient's preoperative and postoperative packed cell volumes were 34% and 35%, respectively. She had transient diabetes insipidus postoperatively but remained neurologically intact. The patient had a sudden neurologic deterioration 17 h later, with no intracranial complication on cranial computerized tomography scan. This necessitated endotracheal intubation and mechanical ventilation. The neurologic status worsened progressively, and she died on the fifth-day postoperation. An autopsy revealed residual pituitary tumor, lobar pneumonia, benign nephrosclerosis, hepatomegaly, fatty liver, and splenomegaly (with acute congestion). Could the hemorrhagic complication have been caused by sickle cell hepatopathy or was it purely a surgical complication? The mortality may be as much related to the procedure as it was to the patient's sickle cell disease. The only other type of hemoglobinopathy in our study aside sickle cell disease was Hb CC, which constituted 0.08% of all cases done over the study period. At follow-up, which ranged between 2 months and 5 years, six of the patients representing eight (88.9%) of the procedures were alive and well.
Neurosurgery is possible and safe in patients with hemoglobin disorders. Proper preoperative preparation, meticulous anesthesia/surgery, and excellent postoperation care can help improve outcome.
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Conflicts of interest
There are no conflicts of interest.
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