Management of pregnancy in a patient with beta thalassaemia major

beta thalassaemia is one of the world's most wide-spread monogenetic disorders. Advances in the management of beta thalassaemia major by extensive blood transfusions and chelation therapy have improved survival of patients into adult life. Due to the prolonged life expectancy and improvements in quality of life, pregnancy has now become an important issue for patients and clinicians. We report a case of a pregnant patient with beta thalassaemia major who underwent a successful caesarean section under spinal anaesthesia. The multidisciplinary approach to management of beta thalassaemia major and pregnancy is discussed.     

Prevention of overwhelming postsplenectomy infection in thalassemia patients by partial rather than total splenectomy

Objective

We aimed to evaluate the protective role of partial versus total splenectomy against sepsis in patients with thalassaemia when other preventive measures are not available. Overwhelming postsplenectomy infection is a serious complication of splenectomy in these patients, and most present with pneumococcal septicemia. Pneumococcal vaccine given before surgery is a well-established preventive measure.

Methods

In this study, we compared 2 populations of patients from Iraq and Saudi Arabia, both of whom underwent splenectomy for thalassaemia. All patients from Saudi Arabia were given a preoperative pneumococcal vaccine and underwent total splenectomy after about 4 weeks. Unfortunately, this vaccine was not available for the Iraqi patients. Partial splenectomy was offered to many of these patients as a protective measure against this fatal complication.

Results

A significant difference was found between the total splenectomy fatalities in the 2 groups. There were 5 deaths in the 30 enrolled Iraqi patients over 4 years. One death over a 12-year period was reported in the 22 patients from Saudi Arabia. Partial splenectomy was associated with a dramatic reduction of mortality in the Iraqi patients. None of the 12 patients died during a follow-up period of 4 years.

Conclusions

Pneumovax is a powerful prophylactic tool against overwhelming postsplenectomy infection in patients with thalassaemia and should be used whenever available. In poor or problematic countries with limited health resources, partial rather than total splenectomy could offer an alternative measure to avoid this fatal complication.     

Nodular fasciitis: an unusual cause of extradural spinal cord compression.

A case of nodular fasciitis causing extradural spinal cord compression with a successful outcome following removal is reported. Such a tumor within the spinal cord has never been reported. The pathogenesis of a nodular fasciitis in this situation and its differential diagnosis is discussed.     

Brown-Séquard syndrome caused by a spinal subarachnoid hematoma due to anticoagulant therapy

A case is reported of a patient with a pure Brown-Séquard syndrome caused by a spinal subarachnoid hematoma complicating anticoagulant therapy. None of the seven previously reported cases of spinal subarachnoid hematoma had led to this syndrome. The diagnosis, treatment, prognosis, and etiology of spinal subarachnoid hematoma are discussed.     

Spinal subdural hematoma: case report and review of the literature.

A case of lumbar spinal subdural hematoma in a patient who had been on anticoagulant therapy is reported. Thus far 19 cases of spinal subdural hematoma have been reported in the literature, the majority in patients with a bleeding diathesis and after a lumbar puncture. Our case is the third reported to be in association with anticoagulant therapy. The hematoma was lumbosacral, in contrast to the usual location in the dorsal-lumbar area. A possible mechanism for the production of spinal subdural hematoma after a lumbar puncture is discussed. An early decompressive laminectomy and evacuation of the hematoma is the recommended treatment to obtain the best possible recovery of neurological function. (Neurosurgery, 5: 614--616, 1979).     

Myelopathy due to epidural varicose veins of the cervicothoracic junction. Case report

A 30-year-old man presented with a subacute course of myelopathic signs and symptoms. Magnetic resonance imaging demonstrated an epidural mass lesion of the spinal canal at the cervicothoracic junction causing compression of the spinal cord. Laminectomy with resection of this lesion revealed a large varix with acute and chronic thrombus. Postoperatively, an improvement in neurological function occurred. Spinal epidural varicosities have been reported as an etiological factor in lumbar and sacral radiculopathies. This is the first reported case of spinal cord compression in association with spinal epidural varices. The diagnosis, pathophysiology, and management of this disorder are presented.     

DIFFICULT INTUBATION: A HAZARD IN THALASSAEMIA: A Case Report

A case is described which illustrates how hypertrophy of themaxillary bone marrow in thalassaemia may complicate endotrachealintubation.     

Paraplegia in pregnancy: a case of spinal vascular malformation with klippel-trenaunay syndrome

STUDY DESIGN.: A case report. OBJECTIVE.: To present improvement of paraplegia due to spinal vascular malformation after delivery with no intervention. SUMMARY OF BACKGROUND DATA.: Pregnancy has been reported rarely in patients with Klippel-Trenaunay syndrome (KTS). A combined case of spinal arteriovenous malformations (AVMs) within KTS has not been reported before. METHODS.: A case report of KTS with paraplegia is presented and the pertinent literature is then reviewed. RESULTS.: A pregnant woman who was diagnosed with KTS in childhood presented with newly developed paraplegia due to spinal AVMs. Magnetic resonance image showed a vascular malformation at the T9-T12 levels without evidence of spinal cord hemorrhage. Diagnostic angiography that was performed after delivery revealed a high-flow arteriovenous fistula with AVM. CONCLUSION.: Pregnancy complicated by paraplegia is thought to be secondary to venous engorgement and to the resulting spinal cord ischemia in this case. Her neurological symptoms and signs gradually improved over the few days after cesarean delivery.     

Multiple Cerebral Arteriovenous Malformations (AVMs) Associated with Spinal AVM

Summary  The co-existence of multiple cerebral arteriovenous malformations (AVMs) and a spinal AVM is extremely rare. A 22-year-old man suddenly developed severe headache. Computed tomography (CT) scan showed intracerebral haemorrhage in the left occipital lobe. Cerebral angiography revealed eight AVMs; four were in the right frontal lobe and two each were in the right temporal and left occipital lobe, respectively. A huge high-flow spinal AVM was found incidentally. He had no other vascular lesions such as hereditary haemorrhagic telangiectasia. A left occipital craniotomy was performed and the ruptured left occipital AVMs were removed. Further therapeutic treatment was refused. To our knowledge, except for one autopsy case, this is the first reported patient with multiple cerebral AVMs with a spinal AVM. We discuss the characteristics of this case and review reported cases with cerebral and spinal AVMs.     

Value of magnetic resonance imaging in spontaneous extradural spinal hematoma due to vascular malformation: case report

A case of spinal cord compression due to spontaneous extradural spinal hematoma is reported. A spinal arteriovenous malformation was suspected on the basis of magnetic resonance imaging. Early surgical exploration allowed a complete neurological recovery. The vascular malformation was histopathologically confirmed. The role of magnetic resonance imaging in the evaluation of acute spinal cord compression syndromes is stressed.     

Lumbar spinal stenosis associated with renal osteodystrophy

A case of coexisting lumbar spinal stenosis and renal osteodystrophy is reported. The presence of renal osteodystrophy is known to have predated the onset of neurological complaints. The patient's symptoms were relieved with surgery. Although a causative relationship between renal osteodystrophy and spinal stenosis has not been reported previously, it seems likely in this case.     

Spinal subarachnoid hematoma following spinal anesthesia in a patient with HELLP syndrome

A case of subarachnoid hematoma following spinal anesthesia for cesarean section in a patient with HELLP syndrome is reported. A 39-year-old woman underwent cesarean section under spinal anesthesia for worsening preeclampsia with HELLP syndrome. Despite full recovery from the spinal anesthetic, on the second postoperative day she felt numbness on the posterior aspect of her right leg, noticed she was insensitive to bladder fullness and had mild flaccid paraparesis. Magnetic resonance imaging revealed a spinal subarachnoid hematoma with cauda equina compression. With conservative management she made an almost complete recovery within three months. Serial magnetic resonance imaging showed spontaneous regression of the hematoma. The risk of spinal subarachnoid hematoma following obstetric regional anesthesia is exceedingly small even in a patient with coagulopathy and, to our knowledge, this is only the second reported case following obstetric regional anesthesia. Anesthesia for HELLP syndrome in patients with an adequate platelet count but without disseminated intravascular coagulation is controversial. It is therefore important for clinicians to recognize the symptoms and signs of spinal subarachnoid hematoma to avoid delay in treatment that might result in severe neurological deficit.     

Neurofibromatosis with spinal paralysis due to arteriovenous fistula

Summary A case of neurofibromatosis with spinal paralysis due to arteriovenous fistula is reported. Preoperatively, the appropriate diagnosis was missed because angiography had not been performed. Post-operative angiography disclosed that an arteriovenous fistula formed a tumor-like mass, and that a part of the mass had invaded the spinal canal, compressing the spinal cord. It is concluded that the possibility of an arteriovenous fistula should be kept in mind in neurofibromatosis patients with spinal cord symptoms.     

Subarachnoid hemorrhage due to a spinal neurinoma

A rare observation of spinal neurinoma associated with subarachnoid hemorrhage is reported and the other 12 cases in the literature are reviewed. The extramedullary location of the tumor and the feeble arachnoidal attachments to the spinal cord favor subarachnoidal bleeding in these cases. When the meningeal signs are evident and signs of spinal cord impairment are still unclear, an intracranial origin of the bleeding must be excluded. In doubtful cases, selective spinal angiography is useful in the differential diagnosis between a bleeding spinal tumor and an arteriovenous malformation of the spinal cord.     

Thalassaemia in the Greek community of Cape Town

A survey of the thalassaemias has been undertaken in the Greek community of Cape Town. In a sample of 250 persons 3 were found to have the alpha-thalassaemia trait, and its prevalence can therefore be estimated to be 1,2%. Neither haemoglobin H disease nor homozygous alpha-thalassaemia was encountered, but 2 persons with homozygous beta-thalassaemia (thalassaemia major) who were not part of the survey were traced through the Western Province Blood Transfusion Service. Twenty-three of the 250 subjects were found to have heterozygous beta-thalassaemia (thalassaemia minor), the prevalence of which could be calculated at 9,2%, with a gene frequency of 0,046. The thalassaemias therefore warrant consideration whenever an individual of Greek stock in Cape Town presents with an anaemia.     

Hemangioblastoma of the spinal nerve root

A case of extramedullary spinal root hemangioblastoma is described. A 44-year-old male presented with gait disturbance. Magnetic resonance (MR) imaging revealed a round enhanced mass at the level of T8. Spinal angiography showed a vascular tumor fed by the posterior spinal artery via the left T9 intercostal artery. A laminectomy of T8-T9 was performed, and a reddish brown-colored soft tumor originating in the T9 posterior spinal nerve root was exposed. The postoperative course was uneventful. This is the first reported case of a spinal root hemangioblastoma that was not related with Von Hippel-Lindau disease.     

Postoperative synergistic gangrene after spinal fusion

STUDY DESIGN: A case of synergistic necrotizing gangrenous fasciitis after spinal surgery is reported. OBJECTIVES: To describe this unusual complication, explain the rationale of treatment, and increase awareness of this serious postoperative complication. SUMMARY OF BACKGROUND DATA: Although several cases of postoperative synergistic necrotizing fasciitis have been reported, there are no previously reported cases of this condition after spinal surgery. METHODS: A rapidly progressive necrotizing spinal wound infection after fusion for degenerative disc disease was treated in a 39-year-old man. RESULTS: The infection was successfully treated with serial debridements, appropriate antibiotics, and hyperbaric wound oxygenation. CONCLUSIONS: The authors suggest adherence to the fundamental principles of treatment including radical surgical debridement and appropriate antibiosis for necrotizing gangrene after spinal surgery. In evaluation of aggressive spinal wound infections, diagnosis of synergistic necrotizing fasciitis should be kept in mind. Although hyperbaric wound oxygenation was implemented as an adjunct and appeared to aid in controlling the infection, its effect on outcome is not clear.     

Adult onset of tethered spinal cord syndrome due to fibrous diastematomyelia: case report

Adult onset of the symptoms of tethered spinal cord is a rare entity that is occasionally associated with diastematomyelia. Only one case of fibrous diastematomyelia in an adult has been reported. The fibrous nature of this disease may present a diagnostic difficulty. A 32-year-old man with the adult onset of impairment of sacral functions with lumbar fibrous diastematomyelia is reported. Surgical release of the spinal cord was followed by improvement of the patient's function.     

Solitary osteochondroma presenting as a neck mass with spinal cord compression syndrome.

Osteochondromas are a rare cause of spinal pathology and neurological dysfunction. A case of cervical osteochondroma with spinal cord compression is reported and the pathological, clinical, and radiological features are discussed with a brief review of the literature.     

Spinal cord proliferative sparganosis in Taiwan: a case report

A 43-year-old woman suffered from low back pain and bilateral footdrop. A cisternal myelogram unexpected revealed multiple filing defects in the spinal canal extending from the lower cervical region to the caudal equina. Diagnostic exploration revealed numerous cystic organisms adhering to the spinal cord and nerve roots. Histopathological examination showed these organisms to be proliferative sparganum cestode larvae. Although these cestode larval infections have been reported a dozen times in humans from various parts of the world, this is probably the first reported case of spinal cord infection.