Duraplasty is one of the most common neurosurgical procedures which complications include iatrogenic pseudomeningocele, which is common, but ossification of pseudomeningocele following cranial surgery is a rare event. We present a case of a 2-year-old male patient who came to our hospital with a huge bulge in his head and weakness in the right arm and leg. He had a history of sagittal craniosynostosis with a postoperative cranioplasty complication of left parital pseudomeningocele. He underwent a duraplasty, but the bulge recurred with failed cerebrospinal fluid aspiration and external ventricular drain, changing in size periodically. Computed tomography showed that the bulge was a median and left paramedian parital encephalocele, so encephalocele with ossification was diagnosed and a cranioplasty was done. This case highlights that iatrogenic encephaloceles with ossification can develop after duraplasty repair in the parital region. Also, if a postoperative pseudomeningocele changes in size or consistency, clinicians should look for ossification.

The uncommon, non-hereditary congenital abnormalities known as nasal glial heterotopias (NGH) are composed of heterotopic neuroglial tissue. Typically, NGH manifests in infancy, but occasionally it can also be seen in older children and adults. To rule out intracranial extension, magnetic resonance imaging (MRI) and computed tomography (CT) scans should be performed. Numerous cases have been documented where NGH was mistakenly identified as encephaloceles, teratomas, dermoid cysts, capillary haemangiomas, and even desmoids. A proper clinical, sonological, and even CT and MRI evaluation can lead to a near-final diagnosis; nonetheless, surgical excision and histological confirmation are the gold standards. We report a rare case of a firm, subcutaneous, non-tender, non-reducible midline 2 x 2 x 1 cm swelling with bluish-red skin near the root of the nose that was not affected by posture or pressure. Encephalocele, NGH, and dermoid were the differential diagnoses made based on the oedema found on CT and MRI scans. Histopathology provided a conclusive NGH diagnosis. The instance illustrates the significance of histology as the gold standard for NGH diagnosis.

OBJECTIVE: To estimate the prevalence of neural tube defects among all birth outcomes in Odisha during 2016-2022. Additionally, to estimate the identification rate of neural tube defects during Pradhan Mantri Surakshit Matritva Abhiyan sessions.
METHODS: A population-based cross-sectional study with a household survey for neural tube defects using pictorial card as well as a hospital-based study for antenatal ultrasonography data.
METHODS: The sample population was selected through multistage random sampling. In the first stage, one district from each zone was selected randomly. In the second stage, using simple random sampling, one community health centre and one urban primary health centre were selected from each district. In the third stage, the population from a block and ward were picked from the selected rural and urban settings, respectively.
METHODS: All married women in the reproductive age group (18-49 years) residing in these cluster villages in the selected districts were enrolled.
RESULTS: The study surveyed 49 215 women and recorded 50 196 birth outcomes, including 49 174 live births, 890 stillbirths and 132 medical terminations of pregnancy. A total of 30 neural tube defect cases were detected. The overall prevalence rate of neural tube defect was 0.59 per 1000 birth outcomes. Spina bifida was the most prevalent neural tube defect with the prevalence of, followed by anencephaly and encephalocele. Despite 26 860 mothers receiving antenatal ultrasonography Pradhan Mantri Surakshit Matritva Abhiyan session, data on neural tube defects and other birth defects detected through these scans is unavailable.
CONCLUSIONS: This study found a low prevalence of neural tube defect in Odisha, which is far lower compared with the older studies from India. There is an urgent need to strengthen the quality of antenatal care services provided under Pradhan Mantri Surakshit Matritva Abhiyan through better training regarding anomaly scans and better data keeping at public healthcare facilities.
BACKGROUND: CTRI/2021/06/034487.

Introduction  The middle fossa craniotomy (MFCs) is commonly utilized for spontaneous cerebrospinal fluid (CSF) leaks, encephaloceles, and superior semicircular canal dehiscence (SSCD). This study compares postoperative outcomes of MFCs with and without LD use. Methods  A retrospective cohort study of adults over the age of 18 years presenting for the repair of nonneoplastic CSF leak, encephalocele, or SSCD via MFC from 2009 to 2021 was conducted. The main exposure of interest was the placement of an LD. The primary outcome was the presence of postoperative complications (acute/delayed neurologic deficit, meningitis, intracranial hemorrhage, and stroke). Secondary outcomes included operating room (OR) time, length of stay, recurrence, and need for reoperation. Results  In total, 172 patients were included, 96 of whom received an LD and 76 who did not. Patients not receiving an LD were more likely to receive intraoperative mannitol ( n  = 24, 31.6% vs. n  = 16, 16.7%, p  = 0.02). On univariate logistic regression, LD placement did not influence overall postoperative complications (OR: 0.38, 95% confidence interval [CI]: 0.05-2.02, p  = 0.28), CSF leak recurrence (OR: 0.75, 95% CI: 0.25-2.29, p  = 0.61), or need for reoperation (OR: 1.47, 95% CI: 0.48-4.96, p  = 0.51). While OR time was shorter for patients not receiving LD (349 ± 71 vs. 372 ± 85 minutes), this difference was not statistically significant ( p  = 0.07). Conclusion  No difference in postoperative outcomes was observed in patients who had an intraoperative LD placed compared to those without LD. Operative times were increased in the LD cohort, but this difference was not statistically significant. Given the similar outcomes, we conclude that LD is not necessary to facilitate safe MCF for nonneoplastic skull base pathologies.

UNASSIGNED: Encephalocele is a rare medical condition where certain parts of the central nervous system protrude through a skull defect, resulting in a deformity where the head size is smaller than the protrusion. This condition is relatively uncommon, and only a few cases have been reported worldwide.
METHODS: We present a case of a 13-day-old neonate with a giant occipital encephalocele who underwent a successful surgical intervention in a resource-limited setting.
UNASSIGNED: The diagnosis of encephaloceles is frequently by clinical examination, although sonography could be helpful before birth. It is crucial that this patient receives immediate surgical intervention. In cases where hydrocephalus and ventriculomegaly are absent, we predict a better prognosis. The prone position is preferred in these operations, and Anesthesia is a real challenge.
CONCLUSIONS: Congenital giant occipital encephaloceles can be identified clinically shortly after birth. They cause a substantial surgical challenge due to their massive size. Surgical repair must be performed as early as possible.

Frontoethmoidal encephalocele represents protrusion of meninges and brain in a sac through a defect in the anterior skull base, mostly as swelling over the nose. Rarely it is associated with facial dysmorphism and palatal cleft. There are various perioperative concerns like airway difficulties, leaking from the swelling causing fluid and electrolyte disturbances, risk of infection, compression of the swelling causing a rise in intracranial pressure, bleeding, hypothermia, etc., In neonates, these challenges rise exponentially because of the inherent difficulties in dealing with this group of patients. Frontoethmoidal encephaloceles are common in low socioeconomic strata and are often missed in the antenatal period. We are reporting a rare case of frontoethmoidal encephalocele, with a huge swelling protruding through the cleft palate and occupying more than 50% of the face and oral cavity, making mask ventilation impossible.

An encephalocele is a congenital malformation characterized by protrusion of the intracranial contents through a cranial defect. We report that a fetus of a pregnant mother who had two consecutive pregnancies with ultrasound-detected encephalocele carried compound heterozygous variants in B3GALNT2 NM_152490.5:c.[1423C > T (p.Gln475Ter)]; [261-2A > G] of maternal and paternal origins, respectively, as confirmed by exome sequencing followed by Sanger sequencing validation. The present case implies that mutations in B3GALNT2, a well-known dystroglycanopathy causative gene, may result in a phenotype of neural tube defect, providing new insights into the clinical spectrum of B3GALNT2-related disorders. Our study may contribute to prenatal screening/diagnosis and genetic counseling of congenital brain malformations.

BACKGROUND: Knobloch syndrome (KNO, OMIM # 267,750) is a rare ciliopathy group sydrome characterized by a collagen synthesis disorder. It represents an uncommon cause of pediatric retinal detachment. This report presents two cases with different COL18A1 gene mutations, complicated by retinal detachment.
METHODS: Both cases exhibited high myopia and various degrees of occipital skull defect. The first case, a female, had bilateral congenital retinal detachment, posterior embryotoxon, and strabismus. The second case, a male, had unilateral congenital retinal detachment and neuromotor developmental delay. The first case, diagnosed in the early months of life, underwent successful retinal reattachment surgery. However, surgery was not performed on the second case, who presented with late-stage unilateral retinal detachment and pre-phthisis.
CONCLUSIONS: The report describes two patients with Knobloch syndrome, one of whom responded favorably to surgery for retinal detachment in both eyes. Successful anatomical results were achieved with early surgical interventions. It is essential to recognize the phenotypic and genetic heterogeneity within KNO.

UNASSIGNED: Symptoms of temporal encephalocele or cerebrospinal fluid (CSF) leak causing middle ear effusion or otorrhea can be nonspecific and mistaken for other common diagnoses, leading to delays in diagnosis, failed treatments, and a risk of meningitis. This study sought to investigate the association between symptomatology and time to definitive surgical management.
UNASSIGNED: Retrospective cohort.
UNASSIGNED: Single tertiary care academic medical center.
UNASSIGNED: Adults treated surgically for temporal encephalocele or CSF leak. Revision cases were excluded.
UNASSIGNED: Chart review was performed to identify pertinent symptoms at presentation. Multivariable regression was performed to analyze the association between symptoms and time to definitive management.
UNASSIGNED: Otologic and related symptoms present prior to middle cranial fossa (MCF). Time between symptom onset and surgical treatment.
UNASSIGNED: Thirty-four patients had symptoms present a median of 15.5 months (interquartile range, 8-35 months; range, 1 month to 12 years) prior to surgery. The most common symptoms were subjective hearing loss in the affected ear (76.5%) and aural fullness (73.5%). Otorrhea was present in 55.9%, and 42.9% had a history of otorrhea after myringotomy with or without tube insertion. Meningitis occurred in 5 patients (14.7%). Only the absence of otalgia was statistically significantly associated with decreased time between symptoms onset and surgery (P = 0.01).
UNASSIGNED: Encephalocele and CSF leak were most commonly associated with aural fullness and hearing loss. Medical treatment for presumed Eustachian tube dysfunction or chronic ear disease were commonly observed. Patients had symptoms for a median of almost 1 and a half years prior to surgical management.

Congenital anomalies and defects of the skull base and calvarium encompass a broad and complex spectrum of pathologies. The clinical presentation is highly variable, and these anomalies may be discovered incidentally in asymptomatic individuals. Radiological assessment plays a pivotal role in precisely characterizing these abnormalities, facilitating the diagnostic process, and assisting in any preoperative preparation.