Referred Shoulder Pain Due to the Positioning of the Ventriculoperitoneal Shunt: A Case Report and Literature Review of an Unusual Complication.

We present the case of an 18-year-old male with a ventriculoperitoneal (VP) shunt for hydrocephalus who experienced right shoulder pain. The patient was thoroughly investigated for gastrointestinal disease, including abdominal ultrasound and upper endoscopy, which revealed no abnormalities that could explain his symptoms. X-ray imaging subsequently revealed that the shunt's distal peritoneal tubing was positioned in a supra-hepatic subdiaphragmatic location. Surgical shortening and repositioning of the peritoneal tubing successfully alleviated the patient's shoulder pain. A review of the literature uncovered four articles, comprising a total of six patients, who exhibited similar symptoms of shoulder pain linked to their VP shunts. Given the rarity of this complication, it can be easily overlooked or misdiagnosed. It is crucial for physicians to consider this possibility when evaluating patients with VP shunts who present with shoulder pain to ensure prompt and effective treatment.

Comparing motor development in children with hydrocephalus after treatment with ventriculoperitoneal shunt and endoscopic third ventriculostomy: a cross-sectional study.

Introduction: Ventriculoperitoneal shunt insertion (VPSI) and endoscopic third ventriculostomy (ETV) are the major procedures for treating pediatric hydrocephalus. However, studies comparing motor development following the two treatments are limited. Objective: We aimed to determine motor development outcomes in children with hydrocephalus up to 2 years of age after undergoing VPSI or ETV, to identify which surgical approach yields better motor outcomes and may be more effective for Malawian children. Methods: This was a cross-sectional study where we recruited two groups of participants: one group consisted of children with hydrocephalus treated with VP shunt whilst the other group were treated with ETV, at least 6 months prior to this study. Participants were identified from the hospital records and were called to come for neurodevelopmental assessment using the Malawi Development Assessment Tool (MDAT). Results: A total 152 children treated for hydrocephalus within an 18-month period met the inclusion criteria. Upon follow up and tracing, we recruited 25 children who had been treated: 12 had VPSI and 13 had ETV. MDAT revealed delays in both assessed motor domains: 19 out of the 25 children had delayed gross motor whilst 16 of 25 had delayed fine motor development. There was no significant difference between the shunted and the ETV groups. Conclusion: Children with hydrocephalus demonstrate delays in motor development six to 18 months after treatment with either VPSI or ETV. This may necessitate early and prolonged intensive rehabilitation to restore motor function after surgery. Long-term follow-up studies with bigger sample sizes are required to detect the effect of the two treatment approaches.

Presentation, management, and outcomes of pediatric hydrocephalus in Africa: a systematic review and meta-analysis of 12,355 patients.

OBJECTIVE: The prevalence, management, and outcomes of hydrocephalus remain underexplored in Africa. This study aimed to analyze demographic and clinical features, evaluate treatment strategies, and assess neurological outcomes of pediatric hydrocephalus in Africa. METHODS: A systematic review of the literature using the PubMed, Google Scholar, and Web of Science electronic databases was completed according to the PRISMA guidelines to identify articles describing pediatric patients in Africa with hydrocephalus. RESULTS: Seventy-four retrospective and prospective studies and 33 case reports involving 12,355 patients were included. In 54 retrospective articles reporting patient demographics, 53.8% (3926/7297) were male with a mean age of 12.3 months. Nineteen studies reported macrocephaly (80.2%, 1639/2043) as the most common presentation. The etiology of hydrocephalus was reported as postinfectious (41.0%, 2303/5614) across 27 articles and congenital (48.6%, 1246/2563) in 10 articles. Eleven articles reported 46.7% (609/1305) of patients had communicating hydrocephalus while 53.3% (696/1305) had obstructive hydrocephalus. Diagnostic imaging included CT (76.1%, 2435/3202; n = 29 articles), ultrasonography (72.9%, 2043/2801; n = 15 articles), and MRI (44.8%, 549/1225; n = 11 articles). In 51 articles, 83.1% (7365/8865) of patients had ventriculoperitoneal shunting (VPS) while 33 articles described 54.1% (2795/5169) receiving endoscopic third ventriculostomy (ETV) for hydrocephalus surgical management. Postoperative complications included sepsis (6.9%, 29/421; n = 4 articles), surgical site infections (5.1%, 11/218; n = 4 articles), and CSF leaks (2.0%, 15/748; n = 8 articles). Shunt-related complications included infections (4.3%, 117/2717; n = 21 articles) and blockages (4.1%, 34/829; n = 6 studies). In 15 articles, 9.0% (301/3358) of patients with shunts had revisions. The mean follow-up duration was 18.9 ± 16.7 months with an overall mortality rate of 7.4% (397/5383; n = 29 articles). In the analysis of comparative studies, the 160 patients undergoing ETV demonstrated significantly higher odds of a successful operation (OR 1.54, 95% CI 0.51-4.69; p = 0.03) and neurological improvement at last follow-up (OR 3.36, 95% CI 0.46-24.79; p < 0.01) compared with the 158 who received VPS, but no significant differences were observed for complications and mortality between the two groups (p > 0.05). CONCLUSIONS: This review offers a comprehensive summary of pediatric hydrocephalus in Africa, highlighting shunting as the primary treatment. However, the observed variations across studies highlight the need to establish standardized guidelines for reporting patient characteristics, management strategies, and outcomes to ensure consistency and comparability in articles.

Changes in callosal angle and Evans index after shunt surgery in patients with idiopathic normal pressure hydrocephalus.

BACKGROUND: Idiopathic normal pressure hydrocephalus (iNPH) is a disease characterized by gait disturbance, cognitive impairment and urinary incontinence. For those patients who do not respond to shunt surgery, it lacks objective radiological findings for the diagnosis of shunt malfunction. Here we aimed to evaluate whether Evans index and callosal angle change during a prospective long-term follow-up of patients with iNPH submitted to shunt surgery. METHODS: Clinical (NPH Japanese Scale) and radiological (Evans index, callosal angle) data were collected pre- and postoperatively (3, 6, 12 months) in 19 patients with iNPH. Imaging tests were evaluated by the same neuroradiologist during the follow-up. RESULTS: Patients had lower scores on NPH Japanese Scale over time (p< 0.001). There was no significant difference among Evans index values during the follow-up (p= 0.24). Preoperative average callosal angle was 72 ± 15, which increased to 91 ± 18 in 6 months (p= 0.003). CONCLUSIONS: In this sample, patients with iNPH submitted to a programmable valve shunt had an increase in callosal angle concomitant to neurological improvement. Evans index did not change during follow-up.

Ventriculoperitoneal shunt tube migration into the heart and pulmonary artery: successful tube removal. Illustrative case.

BACKGROUND: Ventriculoperitoneal (VP) shunt tube migration is recognized as an occasional complication; however, migration into the cardiac system is rare. The authors report a case of VP shunt tube migration into the heart and pulmonary artery and the safe removal of the tube. OBSERVATIONS: The patient had a VP shunt implanted for hydrocephalus after a subarachnoid hemorrhage. The screening chest radiograph taken a few months later showed the migration of the abdominal tube into the heart. Examinations revealed that the abdominal tube had migrated from the left subclavian vein, passed through the superior vena cava, right atrium and ventricle, and looped into the pulmonary artery. The patient had no symptoms; however, there were some risks, such as damage to the valves or fatal arrhythmia. The authors therefore decided to remove the tube. To avoid various intraoperative risks, in particular tube knot formation, the tube was removed under fluoroscopy with a guidewire inserted, which was 0.035 inches for the angiography catheter. The tube was successfully removed without any complications. LESSONS: Knot formation can be one of the fatal complications of shunt tube removal. The authors' technique is an effective method for safe removal. https://thejns.org/doi/10.3171/CASE24127.

Ventriculoperitoneal shunt extrusion in pediatric patients, clinical patterns and therapeutic strategies: A scoping review.

Background: Ventriculoperitoneal shunts (VPSs) are frequently employed in neurosurgery to treat hydrocephalus, with a particular focus on pediatric patients. Although VPSs are commonly utilized, they are not exempt from difficulties, such as shunt extrusion. The main aim of this study is to enhance comprehension regarding the occurrence, causes contributing to, and consequences of VPS extrusion in pediatric patients. Methods: A comprehensive search approach was implemented, including electronic databases, including PubMed, Google Scholar, and Scopus, to locate pertinent articles published between January 1950 and May 2023. The utilization of keywords such as "ventriculoperitoneal shunt" and "extrusion," "ventriculoperitoneal shunt" and "migration," and "ventriculoperitoneal shunt" and "perforation" was employed. Data on patient demographics, underlying diseases, origin of extrusion, presenting symptoms, treatment, and follow-up were gathered. Statistical studies were conducted to identify potential risk factors connected with the occurrence of shunt extrusion. Results: A study analyzed 80 studies on 120 individuals with extruded VPS catheters. The majority of patients (55.8%) had symptoms such as cerebrospinal fluid leakage and irritation. Hydrocephalus was categorized into congenital (40%), obstructive (36.7%), and communicating (11.7%) groups. Catheter extrusion sites varied, with most from the anal or rectal site. Preoperative meningitis or peritonitis was present in 20% of patients. Treatments ranged from shunt removal to endoscopic third ventriculostomy, resulting in a 90% recovery rate, 1.7% mortality, and 5% follow-up loss. Conclusion: Extrusion of the distal catheter in VPSs is a critical medical situation that necessitates urgent surgical intervention. The presence of an infection raises the likelihood of complications; hence, it is vital to promptly address the issue through the administration of antibiotics and the replacement of the shunt. Timely intervention enhances results.

To shunt or not to shunt when closing myelomeningocele? A systematic review and meta-analysis of simultaneous versus delayed ventriculoperitoneal shunt placement in neonates undergoing myelomeningocele closure.

OBJECTIVE: Approximately 70%-80% of children born with myelomeningocele develop hydrocephalus and need CSF diversion, commonly a ventriculoperitoneal shunt (VPS) placement. The optimal timing of surgery is not known, but many centers delay VPS placement and perform it in a separate surgery to avoid shunt complications, mainly shunt contamination and infection. This systematic review and meta-analysis aimed to compare shunt-related complications between populations with VPS surgery performed either simultaneously with myelomeningocele closure or with delay. METHODS: The authors searched MEDLINE (PubMed), Scopus, Web of Science, Cochrane Central Register of Controlled Trials, and Cochrane Database of Systematic Reviews databases on December 15, 2022, and November 11, 2023, using a predefined search strategy. Randomized and nonrandomized trials of neonates undergoing postnatal myelomeningocele closure and VPS placement before the age of 29 days were included. Cases with prenatal myelomeningocele closure and hydrocephalus treatment other than a VPS were excluded. All studies were screened and assessed by two independent reviewers. The authors performed a meta-analysis pooling risk ratios (RRs) with a 95% CI using the random-effects model. The quality of studies was assessed using the Newcastle-Ottawa Scale. RESULTS: After screening and a full-text review of 2099 database search results, 12 studies with a total number of 4894 patients were included. All studies were nonrandomized studies. Only 2 studies were ranked as good quality on the Newcastle-Ottawa Scale. No statistically significant differences were found between simultaneous and delayed shunt insertion in terms of shunt infection (RR 0.77, 95% CI 0.41-1.42); shunt revision (RR 0.49, 95% CI 0.19-1.30); overall mortality (RR 0.87, 95% CI 0.09-8.57); wound CSF leak (RR 0.20, 95% CI 0.03-1.23); or myelomeningocele wound dehiscence (RR 0.52, 95% CI 0.07-3.71). In the subgroup analysis of studies conducted in high-income countries, simultaneous shunting was superior to delayed shunting concerning shunt infection (RR 0.49, 95% CI 0.31-0.78) and shunt revision (RR 0.30, 95% CI 0.09-0.95). CONCLUSIONS: This systematic review and meta-analysis found no statistically significant differences in shunt-related complications between simultaneous and delayed VPS surgery in myelomeningocele-related hydrocephalus in neonates. The current literature does not support the common practice of delayed shunting or its alternative, simultaneous shunting.

Re-examining the classification of abdominal pseudocysts as surgical site infections through review of broad-range polymerase chain reaction results.

BACKGROUND: The Hydrocephalus Clinical Research Network-quality group (HCRNq) historically defined all abdominal pseudocysts associated with a ventriculoperitoneal shunt as a surgical site infection regardless of culture result. METHODS: We retrospectively reviewed broad-range polymerase chain reaction (BRPCR) results sent between January 2017 and July 2023 from abdominal pseudocyst fluid sent from hospitals around the country to a reference laboratory to help further characterize these collections. RESULTS: A total of 19 samples were tested via BRPCR between 1/2017 and 7/2023. Two (10.5 %) had organisms identified; one with Staphylococcus epidermidis and one with Candida parapsilosis. No fastidious organisms that would be expected to not grow with typical culture techniques were identified. CONCLUSIONS: Few abdominal pseudocysts had organisms identified by BRPCR, suggesting that not all pseudocysts are due to infectious causes. Consideration should be given to alternate causes of pseudocyst development when cultures are negative.

How do I do it? Real-time three-dimensional robotic C-arm navigation for ventriculoperitoneal shunting.

BACKGROUND: Ventriculoperitoneal (VP) shunts are commonly used for managing hydrocephalus, with mechanical dysfunction being the most common cause of complications that require revision. A VP shunt placed using a real-time three-dimensional (3D) robotic C-arm navigation system may have better outcomes and fewer complications. METHODS: In this technical note, we introduced the workflow of the use of the real-time 3D robotic C-arm navigation system for ventriculoperitoneal shunting. CONCLUSION: The real-time 3D robotic C-arm can provide a more precise approach to the target. Furthermore, this technique may lower the risk of complications and increase the success rate of shunt placements.

Cerebrospinal Fluid Seeding Versus Inflammation in Setting of Ventriculoperitoneal Shunt as a Potential Cause for Distant Recurrence of Glioblastoma.

Glioblastoma multiforme (GBM) is the most common primary brain tumor in adults with a median survival of approximately 15 months, despite treatment, with most patients experiencing recurrence within 9 months of resection. The propensity of recurrence in GBM exemplifies the fatal course of the disease and remains an underlying area of study as novel instances of recurrence are encountered. The authors present a unique case of a 31-year-old male patient with a history of cerebellomedullary junction astrocytoma who later developed a supratentorial GBM followed by recurrence centered around a preexisting ventriculoperitoneal catheter and located in the hemisphere contralateral to his first GBM. Each of these lesions was initially thought to represent de novo glial neoplasms because of the absence of intervening T2 fluid-attenuated inversion recovery signal change between each lesion. However, next-generation sequencing using the GlioSeq™ platform revealed similar mutational profiles in both GBMs, suggesting an alternative method of migration of tumor cells to the shunt catheter site, and a local inflammatory environment likely triggering recurrence. This study concludes that in rare instances, in the presence of dormant glioma cells, intracranial foreign bodies may promote an inflammatory microenvironment that may activate tumorigenesis.

Intracranial vein of Galen malformation and its management: A case report.

Vein of Galen malformation (VOGM) is a rare congenital arteriovenous malformation affecting the pediatric population, characterized by a fistula between the diencephalon and a dilated vein of Galen. The present study reports the case of a 6-month-old male infant referred for developmental delays and abnormal head circumference. A clinical examination revealed macrocephaly, dilated scalp veins and neurological abnormalities. Neuroimaging confirmed a large VOGM with associated hydrocephalus. A multidisciplinary team devised a treatment plan involving endovascular embolization and ventriculoperitoneal shunt placement. The post-operative recovery exhibited an improvement in neurodevelopmental milestones and a reduced head circumference. Generally, the management of VOGM requires a comprehensive approach, including early diagnosis, precise imaging and timely intervention. The case described herein highlights the importance of interventional radiology in planning a management plan and the novel kissing microcatheter endovascular embolization technique.

A Rare Complication of Proteus Brain Abscess Due to Coccidioidomycosis Causing Hydrocephalus.

Proteus mirabilis is a very common gram-negative facultative anaerobe seen in urinary tract infections. This rod-shaped bacterium tends to cause urolithiasis via its ability to alkalinize the urine. However, in some cases, this bacterium has been shown to cause bacteremia as well as other complicated infections. Here we would like to present a rare case of Proteus mirabilis that has invaded the brain in a patient that has a ventriculoperitoneal (VP) shunt placed due to coccidioidal meningitis causing hydrocephalus. We would also like to discuss the importance of the monitoring of VP shunt and discuss their likelihood of infections and the medical as well as surgical management.

A Rare Case of Delayed Ventriculoperitoneal Shunt Catheter Migration Into the Heart: A Case Report.

The treatment of hydrocephalus with a ventriculoperitoneal (VP) shunt can lead to complications such as shunt migration. A 67-year-old male, who had previously undergone VP shunt surgery for normal-pressure hydrocephalus, presented approximately five years later with symptoms of general weakness and abdominal pain. Imaging revealed shunt malpositioning, with the catheter passing through an abnormal route to the heart. The catheter was successfully removed under fluoroscopic guidance while monitoring patient's electrocardiogram to prevent potential secondary complications. Although rare, cardiac migration of VP shunts can lead to life-threatening secondary complications. Our case highlights the possibility of delayed upward migration of the shunt catheter in patients with VP shunts, emphasizing the need for various strategies to address and manage this issue.

Cryptococcal Meningitis in an Immunocompetent Patient With Underlying Risk Factors.

Cryptococcal meningitis, a severe fungal infection of the central nervous system, is usually found in immunocompromised patients, especially those with human immunodeficiency virus/acquired immunodeficiency syndrome. Its occurrence in immunocompetent individuals is rare and the presentation can be nonspecific. We present a case of cryptococcal meningitis in a young, immunocompetent male with a known history of intravenous drug abuse who was also found to have hepatitis C during admission. Induction therapy with amphotericin B and flucytosine was completed for 14 days. This shorter duration was considered as he had a good clinical response with rapid improvement in mental status and intracranial pressure with an extraventricular drain and negative repeat cerebrospinal fluid (CSF) culture. However, during the consolidation phase with fluconazole, the patient developed new neurologic symptoms and the induction phase had to be re-initiated for a total of 28 days. The patient likewise required the re-placement of an extraventricular drain and the creation of a ventriculoperitoneal shunt due to persistent CSF accumulation and increased intracranial pressure. He was eventually discharged on fluconazole for a planned consolidation phase of eight weeks, followed by a prolonged maintenance phase, but the patient was lost to follow-up.

Impact of dual procedures: How combining VP shunt placement for hydrocephalus and myelomeningocele repair in newborns affects complication rates?

To discern the efficacy of simultaneous versus delayed VPS surgery in managing hydrocephalus linked with MMC repair: The debate over the concurrent or deferred placement of ventriculoperitoneal shunts (VPS) during myelomeningocele (MMC) repair in hydrocephalic neonates necessitates a nuanced evaluation of associated risks and benefits. While VPS placement can mitigate cerebrospinal fluid (CSF) leaks and minimize wound dehiscence post-MMC repair, it concurrently introduces potential hazards such as infections and shunt-related malfunctions. This prospective cohort study focused on144 newborns with spinal myelomeningocele and hydrocephalus. Divided into two groups based on the timing of dysraphism repair and VPS placement, 101 children underwent concurrent procedures, while 43 received deferred VPS insertion post-MMC closure. Female patients constituted 60% of the cohort, with lumbar lesions being predominant. The median age for MMC closure was three days. Analysis revealed that the deferred insertion group exhibited higher rates of shunt malfunctions, CSF leaks, and wound dehiscence compared to the concurrent insertion group. Although indications hinted at a potential increase in shunt infections in the immediate insertion group, statistical significance was lacking. The study established a statistically significant association between the timing of shunt insertion during MMC repair and specific outcomes, such as CSF leaks and wound dehiscence. The findings suggest that concurrent shunt insertion during MMC repair may reduce the incidence of these complications compared to deferred insertion. However, no substantial differences emerged in terms of shunt infection and malfunction, emphasizing the persistent challenges associated with these major complications.

Safety Study of Percutaneous Gastroscopic Gastrostomy in Patients After Ventriculoperitoneal Shunt.

OBJECTIVE: To evaluate the safety study of percutaneous gastroscopic gastrostomy in patients after ventriculoperitoneal shunt. METHODS: We conducted a retrospective analysis of neurosurgical patients who underwent VPS and PEG at our hospital between January 2012 and November 2023. Patients were divided into 2 groups: VPS group and VPS followed by PEG gruop. Patients received routine antibiotic prophylaxis before the procedure, continued for 48 hours. Follow-up included monitoring immediate complications, particularly wound infection, intracranial infection, neurologic status deterioration, and shunt dysfunction. Routine follow-up visits were conducted post-discharge. RESULTS: In the VPS group (n = 778), the incidence of intracranial infection was 3.08%. Among patients with PEG after VPS, the time interval between procedures ranged from 13 to 685 days. The mean follow-up period was 22 (1-77) months, with no deaths or further complications. CONCLUSION: Performing PEG more than 13 days after VPS does not significantly increase the risk of intracranial infections or PEG-associated infections, making it a relatively safe procedure.

Antigen titers in cryptococcal meningitis: what determines how fast they fall?

Follow-up of previously healthy patients surviving cryptococcal meningitis found that cryptococcal antigen could be detected for more than one year in serum from 38 of 44 (86%) patients and in CSF from 20 of 31 patients (67%), far beyond the time of culture conversion. The speed of titer decline, measured as the number of days for a two fold drop in titer to occur, was slower in serum than in CSF. Speed of decline of antigen titers was much slower in serum and CSF for patients infected with C. gattii than C. neoformans. The speed of decline in CSF and serum titers was also much slower in patients who had received a ventriculoperitoneal shunt for increased intracranial pressure. The variable and extraordinarily slow rate of clearance in our patients did not appear to reflect differences in disease control but rather differences in species and shunting for increased intracranial pressure.

Obstetric and anesthetic management in parturients with ventriculoperitoneal shunt: a case series.

Further study is needed to determine the safest mode of delivery and anesthetic management for parturients with ventriculoperitoneal shunts (VP). Prior recommendation for delivery in women with ventriculoperitoneal shunts was cesarean delivery. However, both vaginal delivery and neuraxial anesthesia have been shown to be safe in women with appropriately functioning VP shunts. We present a case series of parturients with VP shunt. Parturients with VP shunts were identified and VP shunt placement indications, neurologic symptoms during pregnancy, delivery mode, anesthetic type, and postpartum complications were reviewed. Forty patients were identified, and fifteen women with twenty deliveries were included. Two women experienced neurological symptoms during pregnancy and one required postpartum shunt revision for blurry vision and ataxia. There were ten cesarean deliveries and ten vaginal deliveries (eight normal spontaneous, one vacuum assisted, and one forceps assisted). Assisted vaginal deliveries were performed to decrease Valsalva including the patient with neurological symptoms related to shunt malfunction. Of the vaginal deliveries, six (60%) had epidural analgesia. Anesthesia for cesarean delivery included neuraxial anesthesia (n = 5) and general anesthesia (n = 5). In our cohort, women with VP shunt received neuraxial blockade without complication. Neuraxial techniques should be offered to women with appropriately functioning VP shunt.

Hyperacute epidural hematoma following ventriculoperitoneal shunt placement: A fatal case presentation and discussion of management strategies.

INTRODUCTION: An Epidural hematoma following a Ventriculoperitoneal shunt is a critically rare complication. Due to that rarity and potential dangers, we present a case where the patient became symptomatic 8 h after shunting requiring craniotomy for evacuation of the hematoma. The literature and treatment options were discussed. CASE PRESENTATION: A 40-year-old male arrived to the emergency department in a comatose state with dilated pupils, 8 h post-ventriculoperitoneal shunt placement. Computed tomography showed a massive hyperacute epidural hematoma causing severe midline deviation and subfalcine herniation, leading to emergency craniotomy. Despite surgical intervention and intensive care, the patient's condition did not improve, resulting in his death. DISCUSSION: Epidural hematomas (EDHs) are an infrequent complication of ventricular drainage procedures, occurring at a documented frequency of 0.4 %, often presenting with symptoms mimicking shunt malfunction or mass lesions. Diagnosis involves computed tomography (CT) scans, but magnetic resonance imaging (MRI) may be more sensitive. Treatment options include conservative management and surgical intervention, with prognosis heavily dependent on the patient's neurological status at the time of surgery, with mortality rates varying based on level of consciousness. CONCLUSION: This study presents a rare case of fatal hyperacute epidural hematoma following Ventriculoperitoneal shunt (VPS) placement in a 40-year-old man, highlighting the importance of early detection and intervention. The hypotheses suggests overloaded pressure on the dura during ventricular end insertion. Despite available treatment options, patient prognosis depends on neurological status at the time of surgery, emphasizing the need for further research into effective management strategies for this complication.

Congenital hydrocephalus and ligneous conjunctivitis in two children with severe type I plasminogen deficiency: A case report and literature review.

Type I plasminogen (PLG I) deficiency is a genetic disorder inherited in an autosomal recessive mode and carries high mortality and morbidity. This case report discusses two babies, aged 2 and 3 months, who were diagnosed with ligneous conjunctivitis and congenital hydrocephalus. They had progressive macrocephaly, which led to the insertion of a ventriculoperitoneal shunt. However, there was no significant improvement. During the course of the disease, they underwent genetic testing and were diagnosed with PLG I deficiency. One of the babies underwent ventriculocholecystic shunt insertion as part of palliative care and management, since this disease has poor absorption in the peritoneal cavity. Unfortunately, there was no improvement observed, and he died at 18 months. The other baby received intravenous plasma (10 ml/kg) three times a week, plus using several eye drops daily, with moderate improvement. Promising results are expected with the approved plasminogen, human-tvmh, by the Food and Drug Administration. However, access to the newly approved drug in developing countries is challenging, often hindered by cost or supply issues, necessitating the use of alternative treatments.