Association Of Abnormal Metopic Suture Causing .

Romanian Neurosurgery Volume XXXI Number 2 2017 April-JuneArticleAssociation of abnormal metopic suture causinghypertelorism, interfrontal encephalocele withcraniofacial cosmetic deformity associated withmyelomeningocele: management literaturereviewWillem Guillermo Calderon-Miranda, Guru Dutta Satyarthee, Huber Padilla-Zambrano, YanCarlos RamosVillegas, Hernando Raphael Alvis-Miranda, Joulen Mo-Carrascal, Johana Maraby, Luis Rafael Moscote-SalazarMEXICO, INDIA, COLOMBIADOI: 10.1515/romneu-2017-0028

182 Calderon-Miranda et al – Encephalocele associated with myelomeningoceleDOI: 10.1515/romneu-2017-0028Association of abnormal metopic suture causinghypertelorism, interfrontal encephalocele withcraniofacial cosmetic deformity associated withmyelomeningocele: management literature reviewWillem Guillermo Calderon-Miranda1, Guru Dutta Satyarthee2,Huber Padilla-Zambrano3, YanCarlos Ramos-Villegas3, HernandoRaphael Alvis-Miranda4, Joulen Mo-Carrascal5, Johana Maraby5,Luis Rafael Moscote-Salazar6Resident of Radiology, Universidad Nacional Autonoma de Mexico, Ciudad de Mexico,MEXICO2Neurosurgeon, Department of neurosurgery, Neurosciences Cengtre, AIIMS New Delhi, INDIA3University of Cartagena, Cartagena de Indias, COLOMBIA4Resident of Neurosurgery, University of Cartagena, Cartagena de Indias, COLOMBIA5Physician, University of Cartagena, Cartagena de Indias, COLOMBIA6Neurosurgeon, RED LATINO, Latin American Trauma & Intensive Neuro-Care Organization,Bogota, COLOMBIA1Abstract: Myelomeningocele may be associated with other neural and extraneuralanomalies. Authors present association of metopic suture abnormality, an interfrontalencephalocele with widening of metopic suture and abnormal shape frontal bones inthe forehead in those associated with hydrocephalus. Authors describes two neonateswith interfrontal encephalocele, representing first series reporting in neonate.Management and pertinent literature is briefly discussed.Key words: metopic sututre abnormality, hypertelorism, meningomyelocele,hydrocephalus, neonateIntroductionThe meningomyelocele may be associatedwith Chiari malformation, hydrocephalus, andcorpus callosal agenesis. The hydrocephalusmay produce large bulging forehead withsunset sign. (1-5) However, abnormalpersistence of widely spaced unfused metopicsuture is extremely uncommon. The widelyopened metopic suture extends superiorly tothe anterior fontanelle and inferiorly alsoextends to the roots of nose causing

Romanian Neurosurgery (2017) XXXI 2: 182 - 185 183hypertelorism and moulding of frontal bones.(4-8) through the metopic suture defect, thebrain parenchyma may herniates andherniated brain may remain vulnerable toexternal injury and associated consequences.The hydrocephalus is postulated to potentiallyaggravate further herniation of brain. Thesecalvarial and brain parenchymal abnormitycases may not require surgery, and the repairof meningocele with ventriculoperitonealshunt surgery may be sufficient. Such caseswere reported in late childhood.(8)However, both of our current cases were in theneonatal age, representing first of it’s in thewestern literature.Author reports twointeresting cases.Here, we present series of five MMCchildren with interfrontal encephalocele andunfused wide metopic suture and describe thetypical feature of this rare abnormality, theprobable differences with other MMCpatients,associatedproblems,andmanagement.for HIV, syphilis, toxoplasma and HBV werenon-reactive.To physical examination feels a distractedmetopic suture, observes ambiguous genitals(scrotumbifidus,hypospadiaandmicropenis), bilateral varus equine foot,bilateral simian fold and myelomeningocelewith fistula, which was operated immediately.Simple skull TC with 3D reconstructionevidences metopic suture defect in U form.(Fig. 1). Cerebral and total spine MRI showsinconspicuous asymmetric cerebellar atrophypredominating on the left side, in the rightoccipital lobe the rotations are disorganized,there is hypoplasia of the corpus callosum,syringomyelia, low tonsils, supratentorialhydrocephalus and sacral meningocele.Correction is performed through resection ofruptured myelomeningocele, dural graft andfasciocutaneous flap, hemodynamically stableevolution after 16 days of prophylactic schemefor neuroinfection is ordered the discharge.Case illustrationCase #1Newborn male with evidence ofmyelomeningocele in sacral region. Born at32.8 gestation weeks with a weight of 2900grams and a cephalic perimeter of 53centimeters, from a 26 years old mother, G6 P3A3 C3, the six pregnancies with differentsfathers, prenatal controls since the first monthof pregnancy, attending 8 consultation in all,she performed 4 prenatal ecographies, ofwhich none of them made the diagnosis ofmyelomeningocele, ambiguous genitals,bilateral varus equine foot and/o metopicsuture defect in U form. Serologic evaluationFigure 1 - CT scan showing an abnormal metopic suture

184 Calderon-Miranda et al – Encephalocele associated with myelomeningoceleCase # 2Full term newborn male, product of a fifthpregnancy, from a 41 years old mother,apparently healthy. Pregnancy evolved withadequate prenatal care. Born by Caesarea,punctuation Apgar was 9/10 with appropriateweight to the age, (3850 grams). Physicalexamination was found to be defective in thelumbarregion,suggestiveofmyelomeningocele. In the cephalic region isevidenced dolichocephalic skull shape, withprotrusion on the forehead. Brain CT scan wasperformed which showed partial agenesis ofthe frontal bone. (Figure 2 A and B). Itneurosurgery performed to correction ofneural tube defect. The patient presentedcommunicating hydrocephalus a week laterwhichrequireddevelopmentventriculoperitoneal shunt placement, withsatisfactory postoperative evolution.DiscussionThe metopic suture is also called medianfrontal suture, often associated with frontalsinus hypoplasia or agenesis. Metopic sutureextends through the nasion to the bregma,in the midline across the frontal bone andoften remain incomplete and usually fuses byaround nine months after birth. (1,2)Rarely, the metopic suture can persist as ananatomical variant, which can be mistaken forfracture of the frontal bone, which isdifferentiated radiologically the metopicsutures lies in the midline andsuturalinterdigitations. (3) A premature fusion of thesuture is termed metopic synostosis whichcharacterically result into trigonocephaly. (2)Figure 2 A and B - CT scan showing an abnormalmetopic sutureMyelomeningocele develops during theneurulation phase of embryonic life due tofailure in posterior neuropore closure.Lacunar skull caused by membranous bonedysplasia typically have well-defined lucentparts and other are frontal dysplasia craniumbifida, [4, 5, 6].Nejat et al postulated the persistent oflacuna in frontal bones in the region ofmetopic suture may cause scalloping of thefrontal bone with predisposition to persist

Romanian Neurosurgery (2017) XXXI 2: 182 - 185 185widely opened metopic suture, pulsation ofherniated neural tissue causes abnormality inthe metopic suture region also causingcosmetic deformity. haltedwithventriculoperitoneal shunt surgery and usuallyseparate surgery for metopic suture is notadvocated, however, rarely cases with severehypertelorism and cosmetic deformity may beadvised. (7)Nejat et al. analyzed five children withmean age of six month and all were male.Meningomyelocele was located in the lumbarand lumbosacral areas. Neurological deficitsincluded a spectrum of presentation fromsphincter disturbance to paraplegia. All hadassociated hydrocephalus and required CSFshunt surgery. Cranial neuroimaging tomatic Chiari malformation, andcorpus callosum agenesis in five, five, andthree patients, respectively. These cases hadanterior fontanel anomalies called asinterfrontal encephalocele, associated withopen metopic suture extending widely to thenasalradixproducinghypertelorismaccompanied by interfrontal herniation offrontal lobes. The associated hydrocephalusexaggerates the interfrontal encephalocele andshunt surgery ameliorates interfrontalencephalocele. However, some patient mayhave surgical correction of metopic suture forcosmetic (7).ConclusionAuthors reports two cases of foreheadinterfrontal encephalocele in two neonate,who had associated myelomeningocele.Patients had widened metopic suture andassociation with herniation of frontal lobethrough the midline calvarial m.CorrespondenceLuis Rafael Moscote-Salazar, RED LATINOLatin American Trauma & Intensive Neuro-CareOrganization, Bogota, ColombiaEmail: mineurocirujano@gmail.comReferences1. Vu HL, Panchal J, Parker EE et-al. The timing ofphysiologic closure of the metopic suture: a review of 159patients using reconstructed 3D CT scans of thecraniofacial region. J Craniofac Surg. 2001;12 (6): 527-32.2. Weinzweig J, Kirschner RE, Farley A et-al. Metopicsynostosis: Defining the temporal sequence of normalsuture fusion and differentiating it from synostosis on thebasis of computed tomography images. Plast. Reconstr.Surg. 2003;112 (5): 1211-8.3. Glass RB, Fernbach SK, Norton KI et-al. The infantskull: 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: rarecoexistence of two major malformations. Child Nerv Sys9:278–2815. Gilbert JN, Jones KL, Rorke LB, Chernoff GF, James HE(1986) Central nervous system anomalies associated withmeningomyelocele, hydrocephalus, and the Arnold–Chiari malformation: reappraisal of theories regardingthe pathogenesis of posterior neural tube closure defects.Neurosurgery 18:559–5646. Nayak PK, Mahapatra AK (2006) Frontal bone agenesisin a patient of spinal dysraphism. Pediatr Neurosurg42:171–1737. Nejat F, Kamali S, El Khashab M. Interfrontalencephalocele: a rare feature of forehead in hydrocephalicmyelomeningocele patients. Clinical feature, probablemechanisms, and management. Childs Nerv Syst. 2013Aug;29(8):1349-52.8. Nicolaides KH, Campbell S, Gabbe SG, Guidetti RUltrasound screening for spina bifida: cranial andcerebellar signs. Lancet 1986; 2:72–74

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) Rare