Descompresión de fosa posterior sin duroplastia expansiva en adulto con malformación de Chiari tipo 1-siringomielia

Introducción: La malformación de Chiari tipo 1 incluye un grupo heterogéneo de malformaciones congénitas, caracterizadas por el descenso caudal del cerebelo a través del foramen magno. En un 30-70 % de los casos tiene siringomielia asociada. Existen controversias en torno a la técnica quirúrgica ideal. Objetivo: Presentar un caso de MC-1 asociada a siringomielia en el que no se aplica una duroplastia expansiva. Presentación de caso: Paciente femenina de 43 años, con antecedentes de hipertensión arterial. Acudió a consulta neuroquirúrgica por dolor cervical irradiado al miembro superior derecho. Al examen neurológico mostró signos de afectación de primera y segunda motoneurona. La resonancia magnética confirmó el diagnóstico de MC-1. Se intervino quirúrgicamente mediante descompresión de fosa posterior sin duroplastia expansiva. La paciente evolucionó sin complicaciones y egresó a las 48 horas. Durante el seguimiento mejoraron las manifestaciones parestésicas; sin embargo, el examen neurológico se mantuvo igual. A los seis meses, la resonancia magnética indicó una marcada disminución de la siringomielia y la reconformación de la cisterna magna. Hasta los 18 meses del tratamiento, los síntomas no habían empeorado y la capacidad funcional resultaba aceptable (Karnofsky 90/100). Conclusiones: La descompresión de fosa posterior sin duroplastia expansiva, seguida de re-permeabilización microquirúrgica del foramen de Magendie, tuvo resultados favorables en este caso.

Introduction: Chiari malformation type 1 includes a heterogeneous group of congenital malformations, characterized by caudal descent of the cerebellum through the foramen magnum. It has associated syringomyelia in 30-70% of cases. Controversies exist regarding the ideal surgical technique. Objective: To present a case of MC-1 associated with syringomyelia in which an expansive duroplasty is not applied. Case report: Female patient, 43 years old, with history of arterial hypertension. She went to the neurosurgical consultation for cervical pain radiating to the right upper limb. On neurological examination, she showed signs of first and second motor neuron involvement. MRI confirmed the diagnosis of MC-1. She underwent surgery by decompression of the posterior fossa without expansive duroplasty. The patient evolved without complications and she was discharged after 48 hours. During the follow-up, the paresthetic manifestations improved; however, the neurological examination remained the same. At six months, MRI indicated a marked decrease in syringomyelia and reshaping of the cisterna magna. Until 18 months after treatment, symptoms had not worsened and functional capacity was acceptable (Karnofsky 90/100). Conclusions: Posterior fossa decompression without expansive duroplasty, followed by microsurgical re-permeabilization of Magendie's foramen, had favorable results in our case.

Observational outcome in surgery for Chiari malformation patients

Chiari malformation is the commonest anomaly of the craniovertebral junction involving both the skeletal as well as the neural structures. It is congenital anomaly of the hindbrain characterised by downward elongation of the brain stem and cerebellum into the cervical portion of spinal cord. Most common presenting symptoms was pain in the nape of neck with sub-occipital headache and weakness. If not intervened early in these cases they may progress to quadriparesis and respiratory failure. This study includes authors experience of 30 surgical corrections of Chiari malformation performed at civil hospital Ahmedabad from 2017 to 2019. The age and sex of the patient, the presence of syrinx, the type of surgical procedure and the clinical outcome were determined post-operatively and on follow up. Cerebro spinal fluid leak and collection were observed in patient who undergone duroplasty only with no leakage in patient undergone syringo-subarachnoid shunt. Overall, tingling/numbness had best improvement showed improvement in 13 out of 16 patients. Power showed improvement in 20 out of 27 patients and pain showed improvement in 18 patients. Wasting, clawing and cerebellar signs and bony deformity showed no improvement in any of the above procedures. Authors can conclude for Chiari malformation decompression with or without duroplasty with additional procedure with post-operative physiotherapy and analgesia is the suitable treatment.

Posterior Trans-Dural Repair of Iatrogenic Spinal Cord Herniation after Resection of Ossification of Posterior Longitudinal Ligament

Iatrogenic spinal cord herniation is a rare complication following spinal surgery. We introduce a posterior trans-dural repair technique used in a case of thoracic spinal cord herniation through a ventral dural defect following resection of ossification of the posterior longitudinal ligament (OPLL) in the cervicothoracic spine. A 51-year-old female was suffering from paraplegia after laminectomy alone for cervicothoracic OPLL. Magnetic resonance imaging revealed a severely compressed spinal cord with pseudomeningocele identified postoperatively. Cerebrospinal fluid leak and iatrogenic spinal cord herniation persisted despite several operations with duroplasty and sealing agent. Finally, the problems were treated by repair of the ventral dural defect with posterior trans-dural duroplasty. Several months after surgery, the patient could walk independently. This surgical technique can be applied to treat ventral dural defect and spinal cord herniation.

A Case of Acute Severe Transient Thrombocytopenia Immediately after Cranioplasty using an Artificial Dura Mater / 대한수혈학회지

A four year old boy was admitted to the hospital due to an open skull fracture and severe cerebral contusion with acute subdural hematoma caused by head trauma. The patient underwent emergency operation by craniectomy and duroplasty. Five weeks later, he received a second operation in which autologous cranioplasty and duroplasty was applied using artificial dura mater (Redura), but he experienced transient severe thrombocytopenia immediately after the second operation, necessitating that he receive three platelet transfusions. The patient fully recovered from thrombocytopenia six days after operation. Here, we report a case of abnormal response resulting in acute severe transient thrombocytopenia immediately after cranioplasty using artificial dura mater. The experience of this case suggests that close cooperation with the blood bank is required before a cranioplasty, since severe thrombocytopenia may occur during surgery when employing an absorbable artificial dura mater.

Idiopathic Spinal Cord Herniation Presented as Brown-Sequard Syndrome: A Case Report and Surgical Outcome

Spinal cord herniation is a rare condition that has become increasingly recognised in the last few years. The authors report a case of idiopathic spinal cord herniation in a 33 year old woman performed with progressive Brown-Sequard syndrome. The diagnosis was made on MR imaging. After repairing the herniation, the patient made a gradual improvement. Potential causes are discussed, including a possible role of dural defect. In conclusion, idiopathic spinal cord herniation is a potentially treatable condition that should be more readily diagnosed that increasing awareness and improved imaging techniques.

Cirugía descompresiva occipito-cervical en un adulto con osteopetrosis y malformación de chiari I / Occipito-cervical decompressive surgery in an adult with chiari I osteopetrosis and malformation

Introducción: la osteopetrosis es una rara enfermedad de origen genético, causada por una disminución en la resorción del tejido óseo, pudiendo ser autosómica dominante (benigna) o autosómica recesiva (maligna). Por su afectación craneal puede causar un severo compromiso neurológico (ceguera, parálisis facial, hipoacusia, hipertensión intracraneal, entre otros). El tratamiento incluye el uso de Interferon gamma, el transplante de médula ósea y la cirugía.Descripción del caso: presentamos una paciente de 32 años con osteopetrosis de tipo autonómico dominante asociada a malformación de Chiari I. La misma presentaba cefalea, síndrome cerebeloso, cuadriparesia, disartria, hipoacusia e hipertensión arterial, con edema de papila bilateral. Se realizó una cirugía de descompresión suboccipital, con laminectomía de C1 y duroplastia. Resultados: la paciente tuvo una franca mejoría, la cual mantiene tras 5 años de seguimiento. Continúa tatamiento con Interferon Gamma, Calcitriol y Carbamacepina.

Introduction: osteopetrosis is a rare genetic disease, caused by a decrease in bone resorption. It may be autosomal dominant (benign) or autosomal recessive (malignant). Due to cranial involvement it can cause severe neurological deterioration (blindness, facial paralysis, hearing loss, intracranial hypertension). Treatment includes the use of gamma interferon, bone marrow transplantation and surgery. Case Presentation: we present a 32 year old patient with autosomal dominant osteopetrosis type associated with Chiari I Malformation. She had headaches, cerebellar syndrome, quadriparesis, dysarthria, hearing loss and arterial hypertension, with bilateral papilledema. We conducted a suboccipital decompression surgery with C1 laminectomy and duroplasty. Results: the patient had a marked improvement, which remains after 5 years of follow up. She continues treatment with Interferon Gamma, Calcitriol and Carbamazepine.

Dorsal Herniation of Cauda Equina Due to Sequestrated Intradural Disc

Intradural lumbar disc herniation (ILDH) is uncommon pathology. In present report, authors present a case of ILDH associated with dorsal herniation of the cauda equina rootlets in a 30-year-old male laborer who had chronic backache since last two years. To the best of our knowledge we are reporting this for first time. Report demonstrates the natural course of ILDH.