About the Health Information

In addition to housing the brain in a separate compartment, the base of the skull protects it
from the sinuses, nasal cavity, and other facial features. In extremely unusual cases, birth defects in this area of the skull can cause the meninges to herniate into the nasal cavity. Nasal meningoencephalocele and nasal encephalocele are terminology used to describe the same medical illness, however the particular names vary depending on which components are afflicted.

The loss of cerebrospinal fluid (CSF), a transparent fluid that typically surrounds and shields The brain and spinal cord is a common consequence of this illness. Meningitis, respiratory difficulties, and developmental delays can all be caused by cerebrospinal fluid (CSF) spilling into neighboring cavities such as the nose due to a ruptured or inadequate CSF barrier. A multidisciplinary team of neurosurgeons and ENT specialists is essential for accurate diagnosis and effective treatment.

The following symptoms were reported by a one-year-old child from Odisha who was
brought to MGM Healthcare in Chennai:

• Months of “brain fever” diagnoses
• Difficulty breathing due to a protracted nasal obstruction
• A constant and transparent discharge from the nose.
• Anorexia and poor eating habits.

The child’s condition persisted even after a battery of conservative treatments and several hospital stays. The baby’s issues were initially thought to be caused by common neonatal nasal congestion reasons including enlarged adenoids or respiratory infections, according to much research. However, the symptoms persisted and even worsened with time.

A congenital skull base defect allowed a part of the brain and meninges to herniate into the nasal cavity, as was discovered after thorough imaging testing, which included MRI and CT scans. Additional signs of the abnormality’s chronic cerebrospinal fluid leak included nasal discharge and repeated infections.

This highly rare illness affects approximately one baby out of ten thousand. Many parents or caregivers fail to recognize the signs and symptoms of this condition in their children because they are similar to those of other prevalent pediatric ENT illnesses.

The child was evaluated by a multidisciplinary team headed by Prof. Dr. Sanjeev Mohanty, a
senior consultant and department head at MGM Healthcare’s Institute of ENT. Given the child’s age, size, and anatomical fragility, standard open surgery posed an exceptionally high risk to the child.

A transnasal endoscopic treatment was instead chosen as a less intrusive option in order to accomplish the following:

• Locate and fix the defect at the base of the skull
• Stop the CSF leak
• Bring the nasal airway back to operation

The surgical team used Duragen, a bioengineered collagen-based substance that acts as an artificial dura mater, instead of extracting tissue from another part of the child’s body. Taking away an extra surgical site shortened the surgery and reduced the trauma, which is very important for newborns that are weak and underweight.

Better navigation and more accurate imaging: The staff expertly guided the child’s narrow nasal passages with cutting-edge, high-definition endoscopic tools. This method is meant to reduce damage to tissue and problems after surgery.

Knowledge Across Fields: A team of neurosurgeons, ear, nose, and throat (ENT) surgeons, pediatric anesthesiologists, and critical care specialists worked together to make sure the youngster was safe and got better quickly.

• Had no further episodes of cerebrospinal fluid leakage or fever
• Demonstrated rapid improvement in breathing and comfort
• Discharged from the hospital 48 hours after surgery.
• Minimized the likelihood of infection and scarring by eliminating the need for
  external incisions

This case shows a significant advance in pediatric skull base surgery, given the child’s age and the severity of their sickness.

Surgeons face significant challenges when trying to access a high-risk skull base malformation in a one-year-old infant through a narrow transnasal passage. Conventional open surgery carries a high risk of complications in these cases. By integrating endoscopic procedures with novel biomaterials, they were able to achieve a safer outcome with faster recovery, according to Prof. Dr. Sanjeev Mohanty, Senior Consultant and Head, ENT, Head and Neck Surgery at MGM Healthcare.

This shows how important it is to quickly and accurately identify congenital skull base deformities, since the patient had symptoms for months before a diagnosis was made. A full review is needed after all other steps have been attempted to help the baby’s chronic nasal discharge, recurring fever, or blocked nose.

• MRI and CT scans to visualise problems with bones and soft tissues
• An endoscopic look into the nasal passages
• The CSF beta-2 transferrin test, which shows that CSF is present in nasal discharge

Post-surgery for skull base defect repair, the child will require:

• Regular consultations with an otolaryngologist (ENT) and a neurosurgeon (NS).
• Seeking indications of a cerebrospinal fluid leak or a recurrence of the infection
• Methods to limit physical activity during the healing process

Parents need not be concerned, since the long-term prognosis is favorable with appropriate
treatment.

An endoscopic operation was successfully completed by MGM Healthcare on a one-year-old infant who had a rare congenital malformation of the skull base.This shows how important it is to act quickly. It shows how collaborative care, improved biomaterials, and less invasive procedures have changed the results of pediatric neurosurgery and ENT surgeries.

If your child has symptoms like persistent nasal congestion, recurring infections, or trouble breathing, you should see our pediatric otolaryngologist (ENT) right once. Quick identification is very important for survival.