Download as pdf or txt
Download as pdf or txt
You are on page 1of 4

Child's Nervous System

https://doi.org/10.1007/s00381-023-05881-9

TECHNICAL NOTE

Endoscopic fenestration of the superior medullary velum


for the treatment of a trapped fourth ventricle—technical note
Pasquale Gallo1 · Desiderio Rodrigues1 · Fardad T. Afshari1

Received: 1 July 2022 / Accepted: 11 February 2023


© The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature 2023

Abstract
A trapped fourth ventricle is a clinic-radiological entity characterised by progressive neurological symptoms due to an
enlargement of the fourth ventricle secondary to obstruction to its outflow. This condition is most commonly observed in
ex-preterm patients shunted for a post-haemorrhagic or post-infective hydrocephalus. Until the introduction of endoscopic
aqueductoplasty and stent placement, through a supratentorial or an infratentorial approach, treatment of trapped fourth
ventricle entailed high rates of complications, repeated procedures and consequent morbidity. We describe the first case
of successful treatment of trapped fourth ventricle by fenestration of superior medullary velum through an infratentorial
approach in a 20-month-old child with a functional supratentorial ventriculoperitoneal shunt and an aqueductal anatomy
not favourable for stenting. To the best of our knowledge, this is the first reported case of utilisation of this technique in a
patient with a trapped fourth ventricle, and we wish to highlight this new alternative approach in cases where conventional
aqueductoplasty and stenting may not be feasible.

Keywords  Aqueductoplasty · Endoscopy · Hydrocephalus · Trapped fourth ventricle

Introduction techniques employed before the advent of neuro-endoscopy


such as shunting of the fourth ventricle or microsurgical pos-
Trapped fourth ventricle (TFV) is a challenging surgical entity terior fossa arachnoidolysis/fenestration of the fourth ven-
most commonly observed in paediatric patients shunted in tricle outlets. These procedures are known to be associated
infancy for the treatment of post-infective or post-haemorrhagic with higher rates of complications and repeated procedures
hydrocephalus [1, 2]. Endoscopic techniques, including aque- [4].
ductoplasty and stenting, either through a supratentorial or a We describe here a case of a 20-month-old child presenting
suboccipital route, have revolutionised the treatment of this with symptomatic progressive trapped fourth ventricle on the
challenging entity providing a safe, effective and long-term background of a previous ventriculoperitoneal shunt with slit
solution [3] compared to the previous surgical approaches ventricles and an unfavourable anatomy of the aqueduct of
associated with high morbidity, risk of recurrence and repeated Sylvius, which was managed successfully with the endoscopic
procedures [4]. Performing an isolated aqueductaplasty is asso- fenestration of the superior medullary velum into the quadri-
ciated with the recurrence of stenosis, and therefore stents fol- geminal cistern through a posterior fossa approach.
lowing aqueductoplasty are commonly utilised [3, 5, 6].
Nonetheless, in cases where the anatomical shape of the
aqueduct precludes performing safe aqueductoplasty and Case history
stenting, the only option remains to resort to the classic
We report on a case of a premature baby male born at 27 weeks
and 5 days. The weight at birth was 1034 g, and ultrasound
demonstrated evidence of grade 3 intraventricular haemor-
* Pasquale Gallo
pasquale.gallo@nhs.net rhage. He was initially monitored in the neonatal intensive care
unit in the local hospital. Repeat cranial ultrasound over time
1
Department of Neurosurgery, Birmingham Women’s demonstrated increasing ventricular indices with clinical evi-
and Children’s NHS Foundation Trust, Steelhouse Lane, dence of bulging anterior fontanelle, prominent scalp veins and
Birmingham B4 6NH, England

13
Vol.:(0123456789)
Child's Nervous System

intermittent episodes of desaturation. In light of deterioration, of the fourth ventricle versus an attempt of endoscopic fen-
he was transferred to our neurosurgical unit for further man- estration of the superior medullary velum. On the balance of
agement and treatment. He underwent a right-sided ventricu- risks versus benefits and following discussion in a multidis-
loperitoneal shunt insertion (Delta 1.5 valve, Medtronic) at the ciplinary meeting, the decision was made for an endoscopic
corrected age of 34 weeks and 2 days. He was subsequently approach and fenestration of the superior medullary velum.
followed up in the outpatient clinic every 3 months.
The patient was represented acutely at 20 months of age Surgical technique
with a week’s history of progressive ataxia, regression in
his motor milestones, vomiting, drowsiness and a left VI The patient was placed in a prone position with the head in
nerve palsy. An urgent MRI brain demonstrated evidence of Concorde position in a 3-point fixation using a paediatric
supratentorial slit ventricles and a trapped 4th ventricle with Mayfield clamp. Following minimal hair shave, the skin was
compression of the brainstem and the cerebellar structures prep and draped. Following a straight (3 cm) skin incision over
(Fig. 1A–D). The aqueductal anatomy was found to be non- the cranio-cervical junction, muscles were dissected laterally.
favourable for an aqueductoplasty owing to the length of Minimal bony decompression of Foramen magnum along with
obstruction and the curved shape of the aqueduct; the scan partial C1 laminectomy was performed using Kerrison ron-
showed instead a remarkable bowing conformation of the geurs to improve the working angle. Under endoscope-assisted
superior medullary velum (SMV). visualisation, a 1 cm dura incision was made. Arachnoid adhe-
Possible treatment options included a microscopic approach sions at the level of the foramen of Magendie were fenestrated
with an opening of the IV ventricular outlets or direct shunting and a 30 degrees rigid endoscope (B-braun Miniop) was

Fig. 1  Post-shunting coronal
MRI brain T2 sequence dem-
onstrating large fourth ventricle
with ballooned SMV (A). Post-
shunting MRI brain axial view,
T2 sequence demonstrating slit
ventricles (B). Sagittal (C) and
axial (D) MRI T2 brain demon-
strating trapped fourth ventricle
at age of 20 months following
the symptomatic presentation

13
Child's Nervous System

Fig. 2  Endoscopic view of superior medullary velum pre-fenestration grasper forceps (B). Dilatation of stoma using neuro balloon (C). Created
(A). Asterix points to the location of the abnormal aqueduct. Endoscopic stoma following deflation of balloon (D). Magnified view of the stoma at
view following perforation of the superior medullary velum using blunt the end of the procedure (E)

advanced into the fourth ventricle. A safe area in the superior Closure of muscle and fascia was done in a layered fash-
medullary velum was carefully identified (Fig. 2A), a stoma ion (muscle closure with 2/0 Vicryl; fascia and subcuticular
was created by perforating the velum and then neuro-balloon layers with 3/0 Vicryl). The skin was closed with running
was utilised to enlarge the fenestration (Fig. 2B–C). Once sat- 4/0 Monocryl. Postoperatively, the patient made an une-
isfactory communication with the quadrigeminal cistern was ventful recovery with rapid resolution of the preoperative
established (Fig. 2D–E), the dura was closed in a watertight symptoms. Post-operative MRI at 4 months (Fig. 3A–B) and
fashion with 5/0 Prolene. 12 months (Fig. 3C–D) demonstrated decompression of the

Fig. 3  Coronal (A) and axial


(B) MRI T2 brain demonstrat-
ing reduction in the size of the
trapped fourth ventricle and
deflated SMV at 4 months follow-
ing fenestration. Sagittal (C) and
axial (D) MRI brain T2 sequence
demonstrating a further reduction
in the size and decompression
of trapped fourth ventricle at
12 months post fenestration

13
Child's Nervous System

fourth ventricle and stoma patency. The last follow-up clinic Availability of data and Material  Not applicable.
assessment at 24 months post-surgery has confirmed full
resolution of preoperative signs and symptoms. Declarations 
Ethics approval and consent to participate  Appropriate consent has
been obtained.
Discussion
Consent to participate  Appropriate consent has been obtained.
Management of TFV is challenging and dictated by the size Conflict of interest  The authors declare no competing interests.
of the supratentorial ventricles, the anatomy of the aque-
duct and whether there is already an associated functional
supratentorial shunt in place [1, 3, 7].
In cases with viable lateral ventricles, endoscopic aqueduc- References
toplasty can be performed from a supratentorial approach with
1. Panagopoulos D, Karydakis P, Themistocleous M (2021) The entity
additional stent placement fixed to an Ommaya/Rickham res- of the trapped fourth ventricle: a review of its history, pathophysiol-
ervoir. Alternatively, in cases with slit ventricles and short seg- ogy, and treatment options. Brain Circ 7(3):147–158
ment obstruction of the aqueduct, aqueductoplasty and stenting 2. Pomeraniec IJ, Ksendzovsky A, Ellis S, Roberts SE, Jane JA Jr (2016)
through an infratentorial approach are the favoured option [8]. Frequency and long-term follow-up of trapped fourth ventricle fol-
lowing neonatal posthemorrhagic hydrocephalus. J Neurosurg Pediatr
In cases where aqueductal anatomy is non-favourable for 17(5):552–557
aqueductoplasty or there is long segment obstruction, fourth 3. Imperato A, Almaguer Ascencio LM, Ruggiero C, Spennato P, Di
ventricular shunting or open posterior fossa approaches for Martino G, Aliberti F, Mirone G, Cinalli G (2021) Endoscopic aque-
microscopic fenestration of fourth ventricular outlets can ductoplasty and stenting in the treatment of isolated fourth ventri-
cle in children: 20-year institutional experience. Childs Nerv Syst
be attempted [4, 7, 9, 10]. Nonetheless, these techniques are 37(5):1587–1596
associated with a higher risk of failure, the need for other 4. Lee M, Leahu D, Weiner HL, Abbott R, Wisoff JH, Epstein FJ
procedures and consequent morbidity [4, 7]. (1995) Complications of fourth-ventricular shunts. Pediatr Neuro-
Endoscopic approach and fenestration of the superior med- surg 22:309–314
5. Teo C, Burson T, Misra S (1999) Endoscopic treatment of the trapped
ullar velum through a right supratentorial burr hole has been fourth ventricle. Neurosurgery 44(6):1257–61; discussion 1261–2
previously described in 10 adult cadavers [11]; however, it has 6. Fallah A, Wang AC, Weil AG, Ibrahim GM, Mansouri A, Bhatia, (2016)
not been reported in adults nor in paediatric patients. Our case Predictors of outcome following cerebral aqueductoplasty: an indi-
represents the first such case in a patient with TFV. vidual participant data meta-analysis. Neurosurgery 78(2):285–296
7. Harter DH (2004) Management strategies for treatment of the
This technique has the added benefit of being less invasive trapped fourth ventricle. Childs Nerv Syst 20:710–716
compared to open microscopic approaches or shunting of the 8. Gallo P, Szathmari A, Simon E, Ricci-Franchi AC, Rousselle C,
fourth ventricle. The main limitations include a steep learning Hermier M, Mottolese C (2012) The endoscopic trans-fourth ventri-
curve and familiarity for IV ventricular endoscopic approach. cle aqueductoplasty and stentplacement for the treatment of trapped
fourth ventricle: long-term results in a series of 18 consecutive
Long-term follow-ups and a larger cohort of patients are required patients. Neurol India 60(3):271–277
to further validate long-term patency of the stoma and outcomes. 9. Udayakumaran S, Biyani N, Rosenbaum DP, Ben-Sira L, Constantini S,
Beni-Adani LJ (2011) Posterior fossa craniotomy for trapped fourth
ventricle in shunt-treated hydrocephalic children: long-term outcome.
Neurosurg Pediatr 7(1):52–63
Conclusions 10. Villavicencio AT, Wellons JC 3rd, George TM (1998) Avoiding
complicated shunt systems by open fenestration of symptomatic
Endoscopic fenestration of the superior medullary represents a fourth ventricular cysts associated with hydrocephalus. Pediatr
viable and effective surgical option in cases where aqueductal Neurosurg 29:314–319
11. Tubbs RS, Wellons JC 3rd, Salter G, Oakes WJ (2004) Fenestration
anatomy prevents the use of other endoscopic approaches. of the superior medullary velum as treatment for a trapped fourth
ventricle: a feasibility study. Clin Anat 17(2):82–87
Abbreviations  CSF: Cerebrospinal fluid; MRI: Magnetic resonance
imaging; TFV: Trapped fourth ventricle Publisher's Note Springer Nature remains neutral with regard to
jurisdictional claims in published maps and institutional affiliations.
Author contribution  PG has conceived and performed the procedure;
PG and FA have prepared the article with further input from DR.

13

You might also like