Case Study: Management of a Complex CSF Leak Causing Recurrent Meningitis (Cranial Base Program)

By Randall Bly, MD

Summary

A 6-year-old male was referred to Seattle Children’s by his primary care provider (PCP) for an evaluation of meningoencephalocele and probable cerebrospinal fluid (CSF) leak. The patient received state-of-the-art care at Seattle Children’s from a multidisciplinary care team of physicians and surgeons from the Cranial Base Program, which led to a carefully planned, complex surgery that repaired the leak and resolved his accompanying health problems.

Patient History

TB is a 6-year old male with a complex medical history, including repairs of his cleft lip and palate from an outside hospital, meningoencephalocele, recurrent meningitis requiring multiple hospital admissions, conductive hearing loss and velopharyngeal insufficiency. His PCP referred him to Seattle Children’s for suspected CSF leak due to his clear nasal drainage, headaches and recurrent meningitis. His episodes of meningitis had been challenging to treat, requiring multiple days of IV antibiotics.

Patient Diagnosis

TB was found to have a CSF leak from the meningoencephalocele in the anterior cranial fossa. This was visualized on nasal endoscopy in clinic.

MRI and CT scans identified extensive craniofacial abnormalities. There was bone dehiscence in the anterior cranial fossa with soft tissue density herniating into the nasal cavity. There was also a malformation of the hard palate connecting to the sphenoid and partial opacification of ethmoid cells in the fovea ethmoidalis region.

Treatment and Discussion

The severity of TB’s craniofacial abnormalities required surgery that was complex both in planning and execution. TB’s findings were reviewed by a multidisciplinary team of providers, including specialists from Otolaryngology, Craniofacial Surgery and Neurosurgery. Drs. Randall Bly (Otolaryngology), Amy Lee (Neurosurgery) and Kris Moe (Otolaryngology) performed advanced surgical planning to determine the best surgical approach(es) to access the lesion. These options ranged from a transnasal endoscopic approach to an open craniotomy approach. The latter option is likely to have been adopted by many other institutions, accompanied by brain retraction and removal of part of the skull to bring in tissue that is more conventionally used for such repairs. In an effort to minimize risks from the surgery, the patient, family and surgical team elected to proceed with endoscopic repair because the planning demonstrated adequate access without needing to make large incisions or retract the brain.

The surgical team proceeded with an endoscopic transnasal resection of the meningoencephalocele and repair of the CSF leak. The reconstruction was with a hinged nasal septal flap. Placement of a lumbar drain was used to divert CSF pressure. Repair of defects in this location are often done with a nasoseptal flap; however, the reconstructive technique chosen was not standard. TB’s septum was severely abnormal due to his underlying problems and his multiple prior surgeries. The surgical team had to design the reconstruction with the limited adjacent tissue that was available.

Outcome

TB recovered well after surgery and was excited to get back to fishing! Follow-up imaging confirmed successful repair. In the three years since his surgery, TB has had no signs or symptoms of CSF leak, no meningitis, no headaches and no evidence of clear nasal drainage. He continues to be followed at Seattle Children’s Cranial Base Program and Craniofacial Center for ongoing management of his velopharyngeal insufficiency and conductive hearing loss.

Seattle Children’s Cranial Base Program is the only one in the country where a multidisciplinary team of pediatric specialists treats children with cranial base lesions. Meet the team.