ABSTRACT
Background: Meningocele and meningoencephalocele are rare congenital deformities characterized by herniation of the meninges and of the meninges and brain tissue through a bone defect in the skull (cranioschisis), respectively. Descriptions of these malformations in pigs are scarce in the international literature and non-existent in Brazil, with only one case of cranioschisis with brain exposure (without protrusion of the meninges and covered by skin) reported to date. Here, we report a case of meningocele and another of meningoencephalocele in piglets in the state of Bahia and describe the main related anatomopathological and imaging characteristics (radiographic and ultrasonographic). Case: The congenital malformations occurred in newborn piglets from a farm located in the city of Catu, Bahia, Brazil, which presented with an evident large saccular area positioned dorsally to the head. The animals died 48 h after birth and were referred to the Laboratory of Animal Pathology of the Federal University of Bahia, for the studies and diagnostic conclusion. Postmortem ultrasound examinations showed that the region was filled with fluid and associated with a small brain protrusion in case 1 and a defect in the skull bone, which had its orifice dimensions estimated. Despite the superimposition of structures, the skull malformation was identified on radiographic examination (case 1) and measured on ultrasonographic examination (cases 1 and 2). During necropsy, the piglets presented with a marked marked bulge of saccular appearance under the skin in the frontal region. The saccular formation was floating, internally covered by dura mater and filled with reddish serous liquid (liquor). Discussion: The diagnoses of meningoencephalocele and...(AU)
Subject(s)
Animals , Meningocele/diagnostic imaging , Meningocele/pathology , Meningocele/veterinary , Encephalocele/diagnostic imaging , Encephalocele/pathology , Encephalocele/veterinary , Swine , Congenital Abnormalities/veterinary , Ultrasonography/veterinary , Autopsy/veterinaryABSTRACT
Background: Meningocele and meningoencephalocele are rare congenital deformities characterized by herniation of the meninges and of the meninges and brain tissue through a bone defect in the skull (cranioschisis), respectively. Descriptions of these malformations in pigs are scarce in the international literature and non-existent in Brazil, with only one case of cranioschisis with brain exposure (without protrusion of the meninges and covered by skin) reported to date. Here, we report a case of meningocele and another of meningoencephalocele in piglets in the state of Bahia and describe the main related anatomopathological and imaging characteristics (radiographic and ultrasonographic). Case: The congenital malformations occurred in newborn piglets from a farm located in the city of Catu, Bahia, Brazil, which presented with an evident large saccular area positioned dorsally to the head. The animals died 48 h after birth and were referred to the Laboratory of Animal Pathology of the Federal University of Bahia, for the studies and diagnostic conclusion. Postmortem ultrasound examinations showed that the region was filled with fluid and associated with a small brain protrusion in case 1 and a defect in the skull bone, which had its orifice dimensions estimated. Despite the superimposition of structures, the skull malformation was identified on radiographic examination (case 1) and measured on ultrasonographic examination (cases 1 and 2). During necropsy, the piglets presented with a marked marked bulge of saccular appearance under the skin in the frontal region. The saccular formation was floating, internally covered by dura mater and filled with reddish serous liquid (liquor). Discussion: The diagnoses of meningoencephalocele and...
Subject(s)
Animals , Encephalocele/diagnostic imaging , Encephalocele/pathology , Encephalocele/veterinary , Meningocele/diagnostic imaging , Meningocele/pathology , Meningocele/veterinary , Swine , Congenital Abnormalities/veterinary , Autopsy/veterinary , Ultrasonography/veterinaryABSTRACT
The meningocele is the least common form of spina bifida cystica and represents less than 10% of cases of this disorder. A case of a female patient aged 26 with a history of two pregnancies and two previous cesarean sections, present pregnancy unplanned, uncontrolled during the first trimester prenatal and without supplementation with folic acid. Family history of consanguinity with her husband (second cousins) and psychomotor reassessed in four maternal cousins. The first follow-up visit the patient was at 34 weeks of pregnancy. The ultrasonographic findings were: cervical meningocele posterior filiform connection between the first and second cervical vertebrae, lateral ventriculomegaly and third and fourth ventricles and hydrocephalus secondary. Cesarean section was performed at 37 weeks gestation and was a newborn male 3.000 g, 52 cm, head circumference of 36 cm, Apgar 8/9, Capurro 37 weeks of gestation. In the posterior cervical region tumor was located a soft 5 x 5 cm with intact skin, adhered to deep planes. Movement of all four extremities without neurological involvement. He referred to the department of neurosurgery for shunt placement and subsequently performed surgical excision of the meningocele.
Subject(s)
Cervical Vertebrae/abnormalities , Meningocele/pathology , Adult , Cerebrospinal Fluid Shunts , Cesarean Section, Repeat , Consanguinity , Female , Humans , Hydrocephalus/congenital , Hydrocephalus/etiology , Hydrocephalus/surgery , Infant, Newborn , Magnetic Resonance Imaging , Male , Meningocele/diagnostic imaging , Meningocele/embryology , Meningocele/surgery , Pregnancy , Ultrasonography, PrenatalABSTRACT
PURPOSE: The aim of this study was to highlight the challenges for early diagnosis and the difficulties observed in surgical treatment of patients with transsphenoidal meningoencephalocele associated with cleft lip and/or palate. METHOD: We evaluated six male patients treated over the course of 4 years. Five patients presented encephalic herniation with nonfunctional brain tissue; one of these presented herniation of the pituitary gland and cerebral ventricles. RESULTS: All the patients received surgical treatment for the cleft lip and/or palate. Only one patient underwent repair of the meningoencephalocele, via nasal endoscopy. There were no postprocedural clinical or surgical complications. CONCLUSION: The tendency is to avoid neurosurgery, opting for periodic follow-up with magnetic resonance imaging. In the presence of cleft palate, palatoplasty is essential to protect the meningoencephalocele.
Subject(s)
Encephalocele/diagnosis , Encephalocele/surgery , Meningocele/diagnosis , Meningocele/surgery , Sphenoid Bone/surgery , Cleft Lip/diagnosis , Cleft Lip/pathology , Cleft Lip/surgery , Cleft Palate/diagnosis , Cleft Palate/pathology , Cleft Palate/surgery , Encephalocele/pathology , Endoscopy , Follow-Up Studies , Humans , Magnetic Resonance Imaging , Male , Meningocele/pathology , Sphenoid Bone/pathology , Treatment OutcomeABSTRACT
Atretic cephalocele is a clinicopathological entity, which is different from the common form of cephalocele. Its etiopathogenesis has not been completely explained and there are only two previous reports of familial recurrence. We report a Brazilian family with autosomal dominant inheritance with variable expressivity.
Subject(s)
Encephalocele/genetics , Genes, Dominant , Scalp/abnormalities , Adult , Aged , Alopecia/complications , Brazil , Child , Child, Preschool , Encephalocele/complications , Encephalocele/diagnosis , Family Characteristics , Female , Genetic Variation , Humans , Karyotyping , Male , Meningocele/pathology , Middle Aged , Pedigree , Phenotype , Strabismus/complicationsABSTRACT
The authors review the literature and report a case that illustrate the large spectrum of clinical presentation of the anterior-sacral meningocele. It is analized the presently available diagnostic procedures and it is emphasized the importance of the plain film and mielography in the correct diagnosis of this lesion.