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CASE REPORT |
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| Year : 2020 | Volume
: 15
| Issue : 4 | Page : 426-431 |
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A case of multiloculated hydrocephalus
Binoy D Thavara, Bijukrishnan Rajagopalawarrier, Geo S Kidangan
Department of Neurosurgery, Government Medical College, Thrissur, Kerala, India
| Date of Submission | 11-Jun-2019 |
| Date of Decision | 20-Jun-2019 |
| Date of Acceptance | 24-May-2020 |
| Date of Web Publication | 19-Jan-2021 |
Correspondence Address:
Dr. Binoy D Thavara
Department of Neurosurgery, Government Medical College, Thrissur 680596, Kerala.
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/JPN.JPN_80_19
 Abstract | | |
Multiloculated hydrocephalus (MLH) is a disease in which no single treatment has shown to be superior to others. The authors report a pediatric case of postmeningitic MLH diagnosed at the age of 3 months. It was treated with antibiotics and right ventriculoperitoneal (VP) shunt. At 10 months of age, the patient again presented with gross MLH and non-functioning shunt tube. Patient underwent endoscopic fenestration of the multiple cysts along with endoscopic-guided left VP shunt. At 1 year of age, the patient again presented with MLH with large right-sided cyst. Patient again underwent right VP shunt. The child improved in postoperative period and hence discharged. Pediatric MLH is a neurosurgical challenge. The prognosis is guarded and it is not possible to assure the parents regarding the cure of the disease. Decision of treatment options is difficult since there is no end to the treatment of many such cases. Neuroendoscopy has a definite role in MLH.
Keywords: Endoscopic fenestration, multiloculated hydrocephalus, ventriculoperitoneal shunt
How to cite this article:
Thavara BD, Rajagopalawarrier B, Kidangan GS. A case of multiloculated hydrocephalus. J Pediatr Neurosci 2020;15:426-31 |
| Introduction | |  |
Multiloculated hydrocephalus (MLH) is a condition in which patients have multiple, separate abnormal cerebrospinal fluid (CSF) collections with no communication between them. A uniform surgical strategy has not yet been developed. Current treatment options for MLH are microsurgical fenestration of separate compartments by open craniotomy or endoscopy, shunt surgery in which multiple catheters are placed in the compartments, and combinations of these modalities.[1]
MLH is a severe disease in which no single treatment has clearly been shown to be superior. The goal of treatment is to restore communication between isolated intraventricular compartments in order to create the possibility of the implantation of a simple shunt with only one intraventricular catheter. More than improving the quality of life of the patient, the objective is to reduce the number of surgical procedures. Given the complexity of MLH, each patient must be studied individually, and no procedure proposed by the literature should be ruled out, no matter how old fashioned it may appear.[2]
| Case history | | |
A male child was born at 29 weeks of gestation as one of twin babies. This child was born from second delivery to the parents with nonconsangious marriage. The other twin died in neonatal period due to sepsis. This child survived with birth weight of 1.096 kilogram (kg). At 3 months of age, the child was diagnosed to have broncopneumonia and postmeningitic hydrocephalus [Figure 1] and [Figure 2]. It was treated with antibiotics and right ventriculoperitoneal (VP) shunt (Chhabra medium pressure nonprogrammable). The child improved in postoperative period and was discharged.
At 10 months of age, the child was presented with large head with irritability and vomiting of 2 days duration. On examination, the child was drowsy with head circumference of 53cm and tense anterior fontanelle. The child was having developmental delay with sun setting sign of eyes. The chamber of the right VP shunt tube was not functioning. The child’s weight was 6.5kg. Computed tomography (CT) scan showed MLH and non-functioning shunt tube [Figure 3][Figure 4][Figure 5]. CSF was tapped from anterior fontanelle to decrease the intracranial pressure and to rule out infection. CSF protein, sugar, cytology, and culture were normal. The patient underwent endoscopic fenestration of the multiple cysts. Left frontal cyst was entered using zero degree endoscope [Figure 6]. Endoscopic fenestration of two posterior cysts on left side and fenestration of the septum pellucidum was done [Figure 7]. After endoscopic fenestration, endoscopic-guided left VP shunt (Chhabra medium pressure nonprogrammable) with ventricular end in the left frontal horn was done [Figure 8]. The child improved in postoperative period and was discharged [Figure 9]. | Figure 3: Axial computed tomography showing multiloculated hydrocephalus with non-functioning shunt tube tip
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 | Figure 6: Left frontal cyst was entered using zero degree neuroendoscope
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 | Figure 8: Axial computed tomography showing functioning left-sided shunt tube with collapsed multiple cysts and nonfunctioning right-sided shunt tube
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 | Figure 9: Left ventriculoperitoneal shunt surgery scar with lax anterior fontanelle
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At 1 year of age, the child again presented with decreased activity. CT scan showed hydrocephalus with large right-sided cyst [Figure 10]. The patient again underwent right VP shunt (Chhabra medium pressure nonprogrammable) [Figure 11]. Chamber and distal end of old right VP shunt system was removed. Ventricular end was adherent to brain and hence left behind. The child improved in postoperative period and hence discharged. | Figure 10: Axial computed tomography showing large right-sided cyst. Bilateral shunt tube tip is seen
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 | Figure 11: Axial computed tomography showing decreased size of right-sided cyst with shunt tube tip in it. Left-sided shunt tube tip is also seen
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| Discussion | | |
Hydrocephalus is a common clinical problem seen in pediatric neurosurgical practice. Hydrocephalus involves dilatation of the cerebral ventricular system with corresponding, compressive effects on the parenchyma. It can be communicative or obstructive types. Congenital, acquired, infective, and secondary hydrocephalus have different clinical features with different modality of treatments. The presentation of hydrocephalus differs in the case of the neonate and infant compared with the older child or adult. Prior to the closure of the cranial sutures and obliteration of the fontanella, hydrocephalus results in disproportionate head growth. In addition to head size, clinical signs include bulging of the fontanellae, wide separation of the cranial sutures, prominence of scalp veins, and “setting sun” of the eyes. In older children and adults, the classical symptom complex consisting of raised ICP, headache, vomiting, and drowsiness is more likely to herald an underlying diagnosis of hydrocephalus. CT scan or MRI is usually diagnostic. CT ventriculography has been advocated to know the communication between the cysts.[3]
Hydrocephalus with an elevated intracranial pressure can lead to deterioration in motor, intellectual, cognitive, and other neuropsychological functions. Venkataramana and Mukundan[4] did a study by including children with congenital hydrocephalus who were surgically treated by VP shunt. Forty such children were divided into Group A: congenital hydrocephalus (n = 25) alone and Group B: hydrocephalus with myelomeingocoele (n = 15). All children underwent VP shunts (Indian) using medium pressure valve. In Group B, shunt was followed-up by the repair of myelomeningocoele. Progressive increase in head size was the most common symptom of hydrocephalus. Patients were presented with established disease in 92% of Group A and 93.2% of Group B. Mean head circumference in Group A and Group B are 49.8 and 45.97cm, respectively. Linear measurements were used in these patients to assess the degree of ventricular dilatation and cortical mantle thickness. These include cell media index, anterior horn index, and absolute third ventricular diameter. There was a significant improvement of these indices toward normalcy in all the children (P < 0.01) after the shunt. The difference between mean chronological age and the mean mental age becomes very obvious in children presenting beyond 9 months of age to the hospital. The percentage of improvement of mental age was significantly greater when intervention was made at age level of less than 6 months. Postoperative assessment revealed improvement in neuropsychological function in all cases, although normal age-related development was observed only in 33% of cases in hydrocephalic children. About 80% of children with myelomeningocele and hydrocephalus reached normal psychological development due to earlier institution of therapy. The study also revealed that the postoperative increase in cortical mantle was related to improvement in clinical and neuropsychological outcome in all, though to a variable extent. Improvement in outcome of hydrocephalic children to normalcy or near normalcy appears to be possible with early detection and prompt institution of therapy along with serial and periodic follow-up program for diagnosing and treating complications and dysfunctions, which may be detected from time to time.[4]
Eshra[5] studied the role of neuroendoscopy in the management of 14 pediatric septated MLH. All patients passed through the well-known popular scenario of shunts and their problems of infection, obstruction, revision, hemorrhage, and/or shunt removal. The endoscopic intervention was done to connect as many cysts to the cyst harboring the fenestrated end of the shunt. They needed to redo the procedure in 11 patients due to the presence of residual compartments not opened and not drained by the existing shunts. Of those 11 patients with two endoscopic trials, only 3 patients needed adding new shunt application as the endoscopic trials failed to unify all compartments. They concluded that the treatment of loculated hydrocephalus remains a difficult problem in the practice of neurosurgery, yet neuroendoscopy has yielded encouraging results and may be able to dramatically change the prognosis of this troublesome condition in the near future.
Piyachon et al.[6] did a retrospective analysis on 20 patients with MLH who underwent endoscopic cyst fenestration alone or in combination with VP shunt revision or insertion between August 2014 and December 2016 with the extended follow-up period to December 2017. The most common cause of reoperations was new formation of intraventricular fibrosis or compartmentalization causing ventricular catheter blockage. Endoscopic cyst fenestration and endoscopic-assisted VP shunt insertion minimize shunt complications in MLH. Shunt independence is unusual.
In our case, the child was initially presented with MLH and treated with right VP shunt. At 10 months of age, the child developed MLH with raised intracranial pressure. It was treated with endoscopic multiple cysts fenestration and endoscopic-guided left VP shunt. At 1 year of age, child again returned with hydrocephalus and underwent right VP shunt. Everytime the authors wished for the recovery of the child, but the child went home only to come back after few months.
| Conclusion | | |
Pediatric multiloculated hydrocephalus is a neurosurgical challenge. The prognosis of the MLH is guarded and it is not possible to assure the parents regarding the cure of the disease. Decision of treatment options is difficult since there is no end to the treatment of many such cases. Neuroendoscopy has definite role in MLH.
Acknowledgements
The study was done with the support from the Department of Neurosurgery, Government Medical College, Thrissur 680 596, Kerala, India.
| Declaration of patient consent | | |
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Lee Yun Ho, Kwon Young Sub, Yang Kook Hee Multiloculated hydrocephalus: open craniotomy or endoscopy? J Korean Neurosurg Soc 2017;60:301-5.  |
| 2. | Zuccaro G, Ramos JG Multiloculated hydrocephalus. Childs Nerv Syst 2011;27:1609-19. |
| 3. | Venkataramana NK Hydrocephalus Indian scenario—A review. J Pediatr Neurosci 2011;6(Suppl S1):11-22. |
| 4. | Venkataramana NK, Mukundan CR Evaluation of functional outcomes in congenital hydrocephalus. J Pediatr Neurosci 2011;6:4-12. |
| 5. | Eshra MA Endoscopic management of septated, multiloculated hydrocephalus. Alexandria J Med 2014;50:123-6. |
| 6. | Piyachon S, Wittayanakorn N, Kittisangvara L, Tadadontip P Treatment of multi-loculated hydrocephalus using endoscopic cyst fenestration and endoscopic guided VP shunt insertion. Childs Nerv Syst 2019;35:493-9. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11]
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