A Case of Intracranial Dermoid Cyst Associated with the Klippel-Feil Deformity and Recurrent Meningitis

Abstract

Intracranial dermoid cysts are rare: of 2,023 verified intracranial tumours recorded by Cushing (1932) there were 15 examples, including epidermoid cysts. In many cases there is an associated congenital dermal sinus. The following is presented as a typical case.Case Report D.H. is the second child of healthy parents, the elder child being normal. He was first referred to the Children's Hospital in July, 1954, at the age of 4 weeks because of diarrhoea and vomiting. This was thought to be dietetic in origin, and the only abnormal finding was the presence of the Klippel-Feil deformity, which was confirmed by radiological examination (Fig. 1).Ten months later he was admitted with acute bronchitis, and examination at that time revealed a small mobile lump in the occipital region. This was considered to be a fibroma.He then remained well for 15 months until August, 1956, when he was readmitted with a two-day history of refusal to eat, vomiting and drowsiness. He was extremely irritable and crying more than usual, especially if disturbed.On examination owing to his spinal deformity it was not possible to confirm any neck stiffness. There was no papilloedema.Lumbar puncture yielded a cloudy fluid containing 3,250 white cells per c.mm., most of which were polymorphonuclear. The protein content was 700 mg. %, the sugar 6 mg. % and the fluid was sterile on culture.A diagnosis of probable meningococcal meningitis was made: he was treated with penicillin and sulphadimidine and his progress was satisfactory.Soon after returning home vomiting recurred. He was crying continuously, banging his head and he was noticed to have photophobia. Two weeks later he was readmitted and on this occasion his cerebrospinal fluid contained 1,350 cells per c.mm., of which 75% were polymorphonuclear. The protein content was 60 mg. %, sugar 27 mg. %, and again the culture was sterile. No cause for the relapse of his meningitis was found and he was treated with a course of chloromycetin, and later given a course of tetracycline. Serial lumbar punctures showed a progressive improvement in his cerebrospinal fluid, and coincidently his general condition improved.Following discharge he remained well for a further seven months, when he was readmitted with a history of vomiting and crying for five days; on examination he had an upper respiratory tract infection, and the cerebrospinal fluid was found to be normal. He was discharged home but within a few days started vomiting. He again had photophobia, his mother thought he had been having FIG.

Dermoid cyst in the posterior fossa accompanied by Klippel-Feil syndrome

“…Several of those reported have been accompanied by congenital dermal sinus and considered to be a focus of chronic meningitis. In the literature, only nine patients with dermoid cyst accompanied by Klippel-Feil syndrome have been described [3,4,7,8,9,10,16,19,20]; these were seven female and two male patients, and our patient is also a woman, though there is no sex difference in the incidence of dermoid cysts without Klippel-Feil syndrome. The patients' ages ranged from 4 months to 61 years, with a mean of 26.4 years.…”

“…Dermoid cysts are also rare neoplasms and account for 0.1-0.7% of all intracranial tumors [6,8,11,13,16,18]. Most intracranial dermoid cysts arise in the posterior fossa, with a predilection for a midline position in the vermis or adjacent meninges, and lead to clinical symptoms in childhood or early adulthood.…”

Association of craniovertebral junction anomalies, klippel-feil syndrome, ruptured dermoid cyst and mirror movement in one patient: a unique case and literature review

“…This synkinesia manifests as a rare disorder that is usually associated with KFS (31,35). It may represent neurological impairment or agenesis of the pyramidal decussation (44), cerebral cortex, corpus callosum, medulla oblongata or spinal cord (31).…”

Report of Eight Cases of Occipital Dermal Sinus: An Update, and MRI Findings

“…However, especially in very young children, diagnosis and treatment remain difficult. Plain skull lateral views may be normal, even if in 80 % of cases a vertical bone defect is detected [21] and if in some cases associated osseous anomalies can be disclosed, including hemivertebrae, spina bifida occulta or Klippel-Feil malformation [18,21,22,25]. Usually, a CT scan does not allow the visualisation of the sinus tract.…”