Surgical management of Chiari malformation type II
- PDF / 7,049,584 Bytes
- 14 Pages / 595.276 x 790.866 pts Page_size
- 13 Downloads / 220 Views
REVIEW ARTICLE
Surgical management of Chiari malformation type II Giuseppe Talamonti 1 Giuseppe D’Aliberti 1
&
Eleonora Marcati 1 & Lara Mastino 1,2 & Giulia Meccariello 1,3 & Marco Picano 1 &
Received: 25 April 2020 / Accepted: 11 May 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Background Chiari malformation type II is present in almost all patients with myelomeningocele but usually remains asymptomatic. Symptoms are generally more severe in neonates, who have the worst prognosis. The association symptoms/ hydrocephalus is well known, and first treatment usually consists of ensuring adequate ventricular drainage. Craniovertebral decompression may be required in patients who do not improve after drainage. However, mechanisms of symptom development are not yet completely understood, timing and techniques of surgery are not codified, long-term evolution is poorly reported, and there are few paper reporting clinical onset and treatment in older patients. Methods We reviewed our personal series of 42 consecutive symptomatic patients that required surgical treatment. Age at surgery ranged from 1 week to 44 years (mean 6.6 years). Surgical timing strictly depended on clinical conditions: urgent management in the more compromised patients (usually infants) and elective treatment before severe deterioration in patients with less severe conditions. All patients first underwent external ventricular drainage, which resolved the symptomatology in 17 cases (40%). Craniocervical decompression was required by 25 patients (60%) who received no benefit from the ventricular drainage. Results Early mortality (2 cases = 4.7%) occurred only in neonates. Clinical improvement was achieved in 37 of 40 survivors (92%). During a follow-up ranging from 2 to 20 years (mean 10.3 years), late mortality consisted of 4 cases (10%), mainly due to cardio-respiratory arrest. Twenty-two patients (55%) required surgery for shunt malfunction and 4 for cord detethering. Six patients (15%) required reoperation owing to symptom recurrence. Conclusion Early treatment of symptomatic Chiari II malformations may warrant satisfactory results in a significant number of patients, even in neonates. Nevertheless, overall mortality remains relatively high, throughout the patient life. Formal transition programs and adult spina bifida care processes have become crucial. Keywords Chiari II malformation . Craniocervical decompression . Myelomeningocele . Ventriculoperitoneal shunt . Spina bifida
Chiari 2 malformation (CM-II) is a myelomeningocele (MMC) associated anomaly consisting of downward displacement of hindbrain [4, 10, 13, 17, 18, 22, 30, 32, 37]. It is
* Giuseppe Talamonti [email protected]; [email protected] 1
Department of Neurosurgery, ASST Niguarda, P.za Ospedale Maggiore 3, 20162 Milan, Italy
2
Department of Neurology and Psychiatry, Division of Neurosurgery, La Sapienza University, Rome, Italy
3
Università degli Studi di Milano, Milan, Italy
symptomatic in 10–30% of patients and symptoms a
Data Loading...
