Encephalocele is  a congenital  neural  tube defect  (NTD).  The pathophysiology  of  the NTDs  is  exceedingly complex.  Numerous explanations have been proposed to explain it. Double encephaloceles are highly unusual. There have only been fifteen previously reported cases of double encephalocele in the medical literature, with this index case being the oldest and first from Pakistan. A four-year-old girl presented with two occipital scalp swellings from infancy. The occipital swelling measured about 7x5x3 cm, while the suboccipital swelling measured about 7x9x5 cm. The skin over both  the swellings  was  intact. Following  a  thorough history,  physical  examination, and  radiological  investigations,  surgical excision and repair was performed. Postoperative recovery was uneventful. She did not develop hydrocephalus until the three month follow-up.Double encephalocele is a rare entity. The multisite closure theory appears to be the most plausible explanation for the development of multiple NTDs. The management of double encephalocele requires a case based approach.

An   encephalocele   is  a   congenital   neural  tube   defect  (NTD) caused  by  failure of  the  cranial part  of   the  developing   neural   tube  to   close,   resulting  in  herniation  of cranial  contents  via a  defect  in the  skull.Encephaloceles  are uncommon  NTDs,  affecting one  in  every 5,000  infants  globally, with  70%  being occipital.1   The   pathophysiology   of  the   NTDs   is  extremely complicated, including intricate interactions between  genes, environment,  and  nutrition. Multiple  hypotheses  have been  offered  to explain  neural  tube formation using experimental models.2 Based on defect site,  encephaloceles  are classified  as:  i) occipital,  ii) suboccipital,    iii)   sincipital   (fronto-ethmoidal),    iv)    basal   (trans-sphenoidal,   trans-ethmoidal,   spheno-ethmoidal,   and   spheno-orbital),   and  v)   parietal.3 Double  encephalocele is  extremely  rare; the  majority  of them  involve  the  occipital or  suboccipital  region.4There are  only  fifteen previously  reported  cases of double encephalocele in the medical literature, and this index case is the oldest and the first to be reported from Pakistan. We present the following case in accordance with the CARE-guidelines.

four years old girl came to us in December 2023 as an outpatient with a history of two occipital scalp swellings  since  birth. According  to  her mother,  she  was born  at  term in  a  small local  hospital,  and they  did not seek additional medical care for her occipital swellings.  On   examination,   the  occipital   swelling   measured around 7x5x3 cm, whereas the suboccipital swelling  measured  about 7x9x5  cm.  Overlying skin  was   intact  over   both   the  swellings,   and   a  slight   indentation   was  visible   on   the  bottom   edge   of  the  occipital  swelling. (Fig.1-A,  B,  C&D) A  bony  defect was  palpable  around the  occipital  swelling. Transillumination was negative in both the swellings. Computed  tomography (CT)  brain  plain with  bone  window and magnetic resonance imaging (MRI) brain plain with MR venogram were performed. CT showed both the encephaloceles and the bony defects. (Fig.2-A&B) MRI  brain  showed soft  tissue  details (Fig.3-A,  B&C)  whereas MR  venogram  showed details  of  the dural venous sinuses. The patient underwent surgical excision   and  repair   for   both  the   encephaloceles. 

Fig.1: Double encephalocele, A&C: Superior views, B&D: Right lateral and left lateral views.

15 ml cystic fluid and applied a tight crepe bandage. There  was no  recurrent  collection after  that,  and the  bandage was removed on her second week of follow-up. She did not develop hydrocephalus until the three month follow-up.

Encephaloceles  are uncommon,  with  an incidence  of  0.8 to  5  per 10,000  live  births globally.6,7  Male  and female carried the same incidence.6 Encephaloceles are typically  solitary, with  occipital  encephaloceles  being more common in general.6,8 Double encephaloceles are extremely uncommon, with only a few cases reported in the  literature.6  Our literature  review,  conducted using  the  PubMed and  Google  Scholar databases, identified fifteen cases, as indicated in Table-I, with no cases reported on PakMediNet, making this the first case report of double encephalocele from Pakistan.Neural tube  genesis  and closure  need  complicated cellular,   extracellular,   and  intracellular   processes.   There are  two  basic ideas  on  neural tube  closure.  The commonly  recognized  hypothesis is  that  neural tube  closure  occurs in  a  continuous, bidirectional  process  that begins in the mid-cervical region and advances in

Fig.5: A&B: Dissection of occipital encephalocele, C,D&E: Sac exposure, F: Viable brain tissue inside sac.

a zipper-like pattern both rostrally and caudally, with the  cranial and  caudal  neuropores closing  last.

There are several flaws in this relatively rudimentary 'zipper concept'. This idea suggests that meningomyeloceles are  more   commonly   seen  at   the   most  cranial   or   caudal ends,  however  it does  not  account for  cervical  meningomyelocele,     multiple     NTDs,    or     double    encephalocele.9The   findings   of  multiple   meningoceles   can  be   explained  at different  levels  by the  multisite  closure theory put out by Van Allen et al.10 and  Nakatsu  et al.11  According  to the  multisite  neural tube  closure  model,  human   normal   neural  tubes   have   several  closure points, or "zippers". Presumably, one or more genes   regulate   these  zippers;   mutations   in  these   genes  would result  in  neural tube  abnormalities  in  the vicinity of the affected zipper.9 This could explain why   double  NTDs   develop   in  embryogenesis   at   different locations similar to our index case.The    mainstay    of   treatment    for    encephalocele    is  surgical.   This   surgery  consists   of   incising  the   sac,  amputating the  extra  tissue to  the  level of  the  surrounding skull,  dural  closure, and  skin  closure. In  general,  infants born  with  an occipital  encephalocele  surrounding  the   brain   have  a   poor  prognosis.   In   addition to  the  contents of  the  sac, the  extent  of the  lesion influences the long-term prognosis.6 In our case, parents of  the  girl were  satisfied  with the  surgical treatment of their daughter.

Double   encephalocele   is  a   rare   condition.  The   multisite closure theory appears to be the most plausible explanation for the development of multiple NTDs. The Double encephalocele in a four-year-old girl

requires unique solutions for each instance.Consent  for  publication: Consent  was  obtained from  the father of the girl for publication of this case report and the accompanying images.

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