Congenital meningocele associated with corpus callosum agenesis and midline superior labial cleft due to Tessier 0-14 facial fissure: A case report.

A case of a 31-day-old infant patient with a Tessier 0-14 deformity related to multiple midline deformities is presented. Although Transnasal endoscopic surgery is the mainstay for the treatment of anterior and middle skull base meningoceles, there are complex cases in which a combined and multidisciplinary approach is necessary. The surgical decisions and techniques are described. To date, this is the first patient reported with Tessier 0-14 deformity treated with a combined endoscopic and external surgical approach.

A case of cranium bifidum with meningocele in Ayrshire calf.

BACKGROUND: Congenital cranial bone defects predispose to herniation of meninges, sometimes with brain tissue involvement, to form a cerebrospinal fluid (CSF)-filled cyst in the head. Such defects mainly results from focal failure of neural tube closure during fetal development and has been reported in various species of domestic mammals. CASE PRESENTATION: A one week old Ayrshire calf with a fluctuant swelling on parieto-occipital region of the head was referred to the faculty. The calf was always lying on lateral recumbency and exhibited resistance to deep palpation around the swelling and neck flexion. Embedded to the midline of the dorso-caudal surface of the cyst's wall was a hard longitudinally oriented structure. The case was diagnosed as meningocele by means of radiographic examination. As the likelihood to full recovery was greatly reduced due to the negative impact already meted on brain tissue by intracranial pressure, the calf was euthanized on grounds of animal welfare and the diagnosis confirmed by anatomopathological findings which also revealed a circular bone defect in parieto-occipital region of the skull vault and a flattened bony structure embedded to the cyst's wall. CONCLUSION: Anatomopathological findings confirmed the diagnosis as cranial bifidum with meningocele at the parieto-occipital region of the skull vault. The presence of a bony structure embedded to the wall of meningeal sac was rather unusual and could not be sufficiently explained. It was however thought to, most likely, represent a part of interparietal bone that failed to get incorporated into squamous part of occipital bone as a result of the defect. The report also highlights challenges that work against timely delivery of urgent veterinary interventions in rural set ups of Africa and rest of the developing world, often leaving veterinarians with animal welfare consideration as main determinant of intervention measures.

Association of anterior thoracic meningocele and azygos lobe of the lung.

Here we report an anterior thoracic meningocele case. Twoyears- old female patient was presented with kyphosis. Azygos lobe of the lung was also demonstrated during radiological studies. Posterolateral thoracotomy incision and extralpeural approach was performed for excision of the anterior meningocele to untether the cord. Although both anomalies are related to faulty embryogenesis and it is well known that faulty embryogenesis may also reveal coexisting abnormalities, we could not speculate a common mechanism for anterior thoracic meningocele and azygos lobe of the lung association.

Malformación del cordón espinal hendido con expresión clínica en el período neonatal / Split spinal cord malformation seen at the neonatal stage

La malformación del cordón espinal hendido es una forma rara de disrafia espinal oculta, se reporta que representa el 3 % de los disrafismos ocultos. El mayor porcentaje de ellos es de localización lumbar. En la literatura cubana solo tenemos referencia de 2 pacientes publicados, una adulta y otro caso pediátrico. Se reporta un recién nacido remitido a nuestro centro por presentar meningocele occipital. Se detecta la presencia de una fosita en región sacro-coccígea, hipotonía muscular en miembro inferior derecho, pie varo con poca movilización del miembro, e incontinencia de esfínter vesical y anal. Los estudios de imagen demuestran la presencia de 2 hemimédulas a nivel lumbar, por lo que se confirma que el recién nacido presenta un síndrome de malformación del cordón espinal hendido, una entidad poco común. Presenta, además, la particularidad de haber sido diagnosticada en el período neonatal por exhibir desde su nacimiento manifestaciones clínicas, como consecuencia de esta entidad, aspecto también relevante, pues, habitualmente, la afección expresa la sintomatología más tarde en la vida, o se mantiene asintomática.

Split spinal cord malformation is a rare form of occult spinal dysraphia and is reported in 3 % of occult dysraphisms which are mainly located in the lumbar region. The Cuban literature made reference to two patients with this condition, an adult and a child. This is the case of a male newborn referred to our center because he presented with occipital meningocele. The examination revealed a small fossa in the sacrococcygeal region, muscular hypotonic in his right lower limb, varus foot and little movement as well as anal and vessical sphincter incontinence. Imaging studies show the presence of two hemicords at the lumbar region, so it is confirmed that this newborn infant had split spinal cord malformation syndrome, which is an uncommon entity. It is interesting to note that this malformation was diagnosed at the neonatal phase because of its clinical manifestations, a relevant aspect since this illness generally shows its symptoms in later life or remains asymptomatic.

Malformación del cordón espinal hendido con expresión clínica en el período neonatal / Split spinal cord malformation seen at the neonatal stage

La malformación del cordón espinal hendido es una forma rara de disrafia espinal oculta, se reporta que representa el 3 por ciento de los disrafismos ocultos. El mayor porcentaje de ellos es de localización lumbar. En la literatura cubana solo tenemos referencia de 2 pacientes publicados, una adulta y otro caso pediátrico. Se reporta un recién nacido remitido a nuestro centro por presentar meningocele occipital. Se detecta la presencia de una fosita en región sacro-coccígea, hipotonía muscular en miembro inferior derecho, pie varo con poca movilización del miembro, e incontinencia de esfínter vesical y anal. Los estudios de imagen demuestran la presencia de 2 hemimédulas a nivel lumbar, por lo que se confirma que el recién nacido presenta un síndrome de malformación del cordón espinal hendido, una entidad poco común. Presenta, además, la particularidad de haber sido diagnosticada en el período neonatal por exhibir desde su nacimiento manifestaciones clínicas, como consecuencia de esta entidad, aspecto también relevante, pues, habitualmente, la afección expresa la sintomatología más tarde en la vida, o se mantiene asintomática(AU)

Split spinal cord malformation is a rare form of occult spinal dysraphia and is reported in 3 percent of occult dysraphisms which are mainly located in the lumbar region. The Cuban literature made reference to two patients with this condition, an adult and a child. This is the case of a male newborn referred to our center because he presented with occipital meningocele. The examination revealed a small fossa in the sacrococcygeal region, muscular hypotonic in his right lower limb, varus foot and little movement as well as anal and vessical sphincter incontinence. Imaging studies show the presence of two hemicords at the lumbar region, so it is confirmed that this newborn infant had split spinal cord malformation syndrome, which is an uncommon entity. It is interesting to note that this malformation was diagnosed at the neonatal phase because of its clinical manifestations, a relevant aspect since this illness generally shows its symptoms in later life or remains asymptomatic(AU)

[Acute urinary retention in children: initial manifestation of caudal regression syndrome]. / Akuter Harnverhalt im Kleinkindalter : Erstmanifestation eines kaudalen Regressionssyndroms.

Caudal regression syndrome is a rare and sporadic congenital developmental defect of the lower spinal segments and the neural tube. Movement disorders and sensory neurological deficits of the lower extremities in conjunction with impaired bladder and bowel control are the major symptoms. Abnormal visual aspects of the sacral region in combination with maternal gestational diabetes are further diagnostic indications. This article reports the unusual case of a toddler presenting with acute urinary retention as the initial symptom of caudal regression syndrome.

Endoscopic endonasal management of congenital intranasal meningocele in a 2-month-old infant.

INTRODUCTION: Nasal meningocele, which is usually congenital, is a rare anomaly resulting from meningeal herniation into the nasal cavities through a bone defect in the skull base. CASE REPORT: An 8-day-old boy was referred with respiratory disturbance and nasal obstruction. Examination showed a cyst-like grayish swelling filling the right nasal cavity. CT scan showed opacity, of fluid-like density, filling the right nasal fossa, in contact with a small bony defect in the right cribriform plate. MRI ruled out herniated brain parenchyma and enabled diagnosis of meningocele. The patient was operated on at the age of 2 months through a transnasal endoscopic approach. Immediate postoperative course was favorable. MRI control at 8 months was normal. DISCUSSION: Modern imaging (CT scan and MRI) is of paramount importance in the preoperative evaluation of nasal meningocele. Endoscopic endonasal (EE) surgery is currently the treatment of choice. CONCLUSION: Steady progress in instrumentation, technique and skills will increase the feasibility of skull-base surgery using an endonasal approach in the pediatric population.

Huge anterior sacral meningocele simulating bladder retention.

Anterior sacral meningocele (ASM) is a rare congenital disorder involving herniation of the dural sac through a defect in the anterior surface of the sacrum. We report the case of a young patient with an enormous ASM that simulated bladder retention in terms of symptoms as well as on physical examination and at ultrasonography. After introducing a catheter that excluded urinary retention, computed tomography (CT) scan and magnetic resonance imaging (MRI) showed the ASM. The patient underwent surgical repair of the ASM through a sacral laminectomy and recovered normal lower urinary tract function.

Intranasal meningoencephalocele presenting as a nasal polyp--a case report.

We present a case of an intranasal meningoencephalocele masquerading as an 'intranasal polyp' in a 17-month-old child. Nasal meningoencephaloceles are uncommon anomalies and require a high index of suspicion for their diagnosis. Biopsy of such lesions without prior imaging studies can be detrimental because of the risk of cerebrospinal fluid leak and meningitis, and is therefore contraindicated. This case reiterates the fact that any child with an intranasal mass should undergo appropriate imaging studies prior to excisional biopsy.

Degeneración tumoral en meningocele no intervenido. Descripción de dos casos / Tumoral degeneration occurring over a non-healing meningocele. Report of two cases

Asistir a pacientes que presentan un meningocele gigante y en edad adulta es una rareza clínica, debido en parte a que es una malformación disráfica con una incidencia baja y a que el índice de supervivencia de los recién nacidos con éstas patologías era menor en épocas anteriores, pese a la benignidad de la malformación. Aportamos a la literatura existente los casos de dos pacientes que nacieron con un disrafismo espinal abierto y que no fueron operados en su momento, llegando a la edad adulta con voluminosos sacos meningocutáneos que tuvieron que ser extirpados, por presentar en el caso de más edad, un absceso con fistulización sin salida de LCR que no mejoró con tratamiento tópico y antibiótico. En el otro caso por sospecha de malignización debido a crecimiento progresivo de la malformación durante el último año, endurecimiento de la piel y dolor. El estudio anatomopatológico de los sacos resecados demostró la existencia de una degeneración carcinomatosa. En nuestros casos y en los pocos casos encontrados en la bibliografía parece que la irritación crónica del LCR y la existencia de células multipotenciales en el saco meningocélico pueden favorecer la malignización de los tejidos que recubren el mismo. La posible malignización descrita en la escasa literatura existente, a la que aportamos nuestros dos casos, sugiere como tratamiento de elección la exéresis quirúrgica de estas lesiones congénitas lo más precozmente posible (AU)

A giant meningocelic sac has not been usually described in adult patients, due to the fact that it shows a low incidence and few newborn have survived to date though the malformation is benign. We report two cases of patients born with the described malformation and who were not operated at that time, so they reached adulthood with bigger sacs. They needed surgery to remove the sacs, for a different reason. The older one had a fistulous abcess but the LCR did not come out, and it did not improved by the application of topic and antibiotic treatment. The other patient showed a progressive growth of the malformation during the last year, skin hardening and pain. The histological study of the dried sacs proved the existence of a carcinomatous degeneration. In the patients we have treated, it seems that a chronic irritation of the LCR and the appearance of multipotent cells in the meningocele may favour the malignancy of the tissues surrounding the sac. This possible malignancy, already described in the bibliography, suggests a prompt elective surgical treatment of the patients with these congenital lesions as soon as possible (AU)