Myelomeningocele may be associated with other neural and extraneural anomalies. Authors present association of metopic suture abnormality, an interfrontal encephalocele with widening of metopic suture and abnormal shape frontal bones in the forehead in those associated with hydrocephalus. Authors describes two neonates with interfrontal encephalocele, representing first series reporting in neonate. Management and pertinent literature is briefly discussed

Alvis-Miranda, Hernando Raphael

Calderon-Miranda, Willem Guillermo

Maraby, Johana

Mo-Carrascal, Joulen

Moscote-Salazar, uis Rafael

Padilla-Zambrano, Huber

Ramos-Villegas, Yan Carlos

Satyarthee, Guru Dutta

London Academic Publishing Ltd.: Arts & Humanities Journals

Romanian Neurosurgery  |  Volume XXXI  |  Number 2 |  2017  |  April-June  Article   Association of abnormal metopic suture causing hypertelorism, interfrontal encephalocele with craniofacial cosmetic deformity associated with myelomeningocele: management literature review  Willem Guillermo Calderon-Miranda, Guru Dutta Satyarthee, Huber Padilla-Zambrano, YanCarlos Ramos-Villegas, Hernando Raphael Alvis-Miranda, Joulen Mo-Carrascal, Johana Maraby, Luis Rafael Moscote-Salazar  MEXICO, INDIA, COLOMBIA    DOI: 10.1515/romneu-2017-0028       182 | Calderon-Miranda et al – Encephalocele associated with myelomeningocele        DOI: 10.1515/romneu-2017-0028   Association of abnormal metopic suture causing hypertelorism, interfrontal encephalocele with craniofacial cosmetic deformity associated with myelomeningocele: management literature review Willem Guillermo Calderon-Miranda1, Guru Dutta Satyarthee2, Huber Padilla-Zambrano3, YanCarlos Ramos-Villegas3, Hernando Raphael Alvis-Miranda4, Joulen Mo-Carrascal5, Johana Maraby5, Luis Rafael Moscote-Salazar6 1Resident of Radiology, Universidad Nacional Autonoma de Mexico, Ciudad de Mexico, MEXICO 2Neurosurgeon, Department of neurosurgery, Neurosciences Cengtre, AIIMS New Delhi, INDIA 3University of Cartagena, Cartagena de Indias, COLOMBIA 4Resident of Neurosurgery, University of Cartagena, Cartagena de Indias, COLOMBIA 5Physician, University of Cartagena, Cartagena de Indias, COLOMBIA 6Neurosurgeon, RED LATINO, Latin American Trauma & Intensive Neuro-Care Organization, Bogota, COLOMBIA  Abstract: Myelomeningocele may be associated with other neural and extraneural anomalies. Authors present association of metopic suture abnormality, an interfrontal encephalocele with widening of metopic suture and abnormal   shape frontal bones   in the forehead in those associated with hydrocephalus.  Authors describes two neonates with interfrontal encephalocele, representing first series reporting in neonate. Management and pertinent literature is briefly discussed.    Key words: metopic sututre abnormality, hypertelorism, meningomyelocele, hydrocephalus, neonate  Introduction The meningomyelocele may be associated with Chiari malformation, hydrocephalus, and corpus callosal agenesis. The hydrocephalus may produce large bulging forehead with sunset sign. (1-5) However, abnormal persistence of widely spaced unfused metopic suture is extremely uncommon. The widely opened metopic suture extends superiorly to the anterior fontanelle and inferiorly also extends to the roots of nose causing      Romanian Neurosurgery (2017) XXXI 2: 182 - 185 | 183        hypertelorism and moulding of frontal bones. (4-8)    through the metopic suture defect, the brain parenchyma may herniates and herniated brain may remain vulnerable to external injury and associated consequences. The hydrocephalus is postulated to potentially aggravate further herniation of brain.  These calvarial and brain parenchymal abnormity cases may not require surgery, and the repair of meningocele with ventriculoperitoneal shunt surgery may be sufficient.  Such cases were reported in late childhood.     (8) However, both of our current cases were in the neonatal age, representing first of it’s in the western literature.     Author reports two interesting cases. Here, we present series of five MMC children with interfrontal encephalocele and unfused wide metopic suture and describe the typical feature of this rare abnormality, the probable differences with other MMC patients, associated problems, and management. Case illustration  Case #1 Newborn male with evidence of myelomeningocele in sacral region. Born at 32.8 gestation weeks with a weight of 2900 grams and a cephalic perimeter of 53 centimeters, from a 26 years old mother, G6 P3 A3 C3, the six pregnancies with differents fathers, prenatal controls since the first month of pregnancy, attending 8 consultation in all, she performed 4 prenatal ecographies, of which none of them made the diagnosis of myelomeningocele, ambiguous genitals, bilateral varus equine foot and/o metopic suture defect in U form. Serologic evaluation for HIV, syphilis, toxoplasma and HBV were non-reactive. To physical examination feels a distracted metopic suture, observes ambiguous genitals (scrotum bifidus, hypospadia and micropenis), bilateral varus equine foot, bilateral simian fold and myelomeningocele with fistula, which was operated immediately. Simple skull TC with 3D reconstruction evidences metopic suture defect in U form. (Fig. 1). Cerebral and total spine MRI shows inconspicuous asymmetric cerebellar atrophy predominating on the left side, in the right occipital lobe the rotations are disorganized, there is hypoplasia of the corpus callosum, syringomyelia, low tonsils, supratentorial hydrocephalus and sacral meningocele. Correction is performed through resection of ruptured myelomeningocele, dural graft and fasciocutaneous flap, hemodynamically stable evolution after 16 days of prophylactic scheme for neuroinfection is ordered the discharge.   Figure 1 - CT scan showing an abnormal metopic suture      184 | Calderon-Miranda et al – Encephalocele associated with myelomeningocele        Case # 2 Full term newborn male, product of a fifth pregnancy, from a 41 years old mother, apparently healthy. Pregnancy evolved with adequate prenatal care. Born by Caesarea, punctuation Apgar was 9/10 with appropriate weight to the age, (3850 grams). Physical examination was found to be defective in the lumbar region, suggestive of myelomeningocele. In the cephalic region is evidenced dolichocephalic skull shape, with protrusion on the forehead. Brain CT scan was performed which showed partial agenesis of the frontal bone. (Figure 2 A and B). It neurosurgery performed to correction of neural tube defect. The patient presented communicating hydrocephalus a week later which required development ventriculoperitoneal shunt placement, with satisfactory postoperative evolution. Discussion The metopic suture is also called   median frontal suture, often associated with frontal sinus hypoplasia or agenesis.  Metopic suture extends   through   the nasion to the bregma, in the   midline across the frontal bone and often remain incomplete and usually fuses by around nine months after birth. (1,2)  Rarely, the metopic suture can persist as an anatomical variant, which can be mistaken for fracture of the frontal bone, which is differentiated radiologically the metopic sutures lies in the midline and   sutural interdigitations. (3)   A premature fusion of the suture is termed metopic synostosis   which characterically result into trigonocephaly.  (2)     Figure 2 A and B - CT scan showing an abnormal metopic suture  Myelomeningocele   develops during the neurulation phase of embryonic life due to failure in posterior neuropore closure.  Lacunar skull caused by membranous bone dysplasia typically have well-defined lucent parts and other are   frontal dysplasia cranium bifida,   [4, 5, 6]. Nejat et al postulated the persistent of lacuna in frontal bones in the region of metopic suture may cause scalloping of the frontal bone with predisposition to persist      Romanian Neurosurgery (2017) XXXI 2: 182 - 185 | 185        widely opened metopic suture, pulsation of herniated neural tissue causes abnormality in the metopic suture region also causing cosmetic deformity. (7)  The metopic suture abnormality progression is usually halted with ventriculoperitoneal shunt surgery and usually separate surgery for metopic suture is not advocated, however, rarely cases with severe hypertelorism and cosmetic deformity may be advised.  (7) Nejat et al. analyzed five children with mean age of six month and all were male. Meningomyelocele was located in the lumbar and lumbosacral areas. Neurological deficits included a spectrum of presentation from sphincter disturbance to paraplegia. All had associated hydrocephalus and required CSF shunt surgery. Cranial neuroimaging showed   intracranial, interfrontal encephalocele, asymptomatic Chiari malformation, and corpus callosum agenesis   in five, five, and three patients, respectively. These cases had   anterior fontanel anomalies called as interfrontal encephalocele, associated with open metopic suture extending widely to the nasal radix producing hypertelorism accompanied by interfrontal herniation of frontal lobes. The associated hydrocephalus exaggerates the interfrontal encephalocele and shunt surgery ameliorates interfrontal encephalocele. However, some patient may have surgical correction of metopic suture for cosmetic (7). Conclusion Authors reports two cases of forehead interfrontal encephalocele in two neonate, who had associated myelomeningocele.   Patients had   widened metopic suture and   association with herniation of frontal lobe through the midline calvarial defect    interfrontal encephalocele causing hypertelorism.  Correspondence Luis Rafael Moscote-Salazar, RED LATINO Latin American Trauma & Intensive Neuro-Care Organization, Bogota, Colombia Email: mineurocirujano@gmail.com References 1. Vu HL, Panchal J, Parker EE et-al. The timing of physiologic closure of the metopic suture: a review of 159 patients using reconstructed 3D CT scans of the craniofacial region. J Craniofac Surg. 2001;12 (6): 527-32.   2. Weinzweig J, Kirschner RE, Farley A et-al. Metopic synostosis: Defining the temporal sequence of normal suture fusion and differentiating it from synostosis on the basis of computed tomography images. Plast. Reconstr. Surg. 2003;112 (5): 1211-8.   3. Glass RB, Fernbach SK, Norton KI et-al. The infant skull: a vault of information. Radiographics. 2004;24 (2): 507-22.   4. Anegawa S, Hayash T, Torigoe R, Hashimoto T (1993) Myelomeningocele associated with cranium bifidum: rare coexistence of two major malformations. Child Nerv Sys 9:278–281   5. Gilbert JN, Jones KL, Rorke LB, Chernoff GF, James HE (1986) Central nervous system anomalies associated with meningomyelocele, hydrocephalus, and the Arnold–Chiari malformation: reappraisal of theories regarding the pathogenesis of posterior neural tube closure defects. Neurosurgery 18:559–564   6. Nayak PK, Mahapatra AK (2006) Frontal bone agenesis in a patient of spinal dysraphism. Pediatr Neurosurg 42:171–173   7. Nejat F, Kamali S, El Khashab M. Interfrontal encephalocele: a rare feature of forehead in hydrocephalic myelomeningocele patients. Clinical feature, probable mechanisms, and management. Childs Nerv Syst. 2013 Aug;29(8):1349-52.   8. Nicolaides KH, Campbell S, Gabbe SG, Guidetti R    Ultrasound screening for spina bifida: cranial and cerebellar signs. Lancet 1986; 2:72–74  

Association of abnormal metopic suture causing hypertelorism, interfrontal encephalocele with craniofacial cosmetic deformity associated with myelomeningocele: Management literature review

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Association of abnormal metopic suture causing hypertelorism, interfrontal encephalocele with craniofacial cosmetic deformity associated with myelomeningocele: Management literature review

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