Limits...
Slit ventricle syndrome and early-onset secondary craniosynostosis in an infant.

Ryoo HG, Kim SK, Cheon JE, Lee JY, Wang KC, Phi JH - Am J Case Rep (2014)

Bottom Line: Challenging differential diagnosis.A 4-month-old girl with a Dandy-Walker malformation underwent a ventriculoperitoneal shunt procedure.Her symptoms were temporarily relieved, but she repeatedly visited the emergency room (ER) for the same symptoms and finally collapsed, with an abrupt increase in ICP, 3 months later.

View Article: PubMed Central - PubMed

Affiliation: Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul, Republic of Korea.

ABSTRACT

Patient: Female, 14 months

Final diagnosis: Slit ventricle syndrome Symptoms: Hydrocephalus • lethargy and seizure • vomiting

Medication: - Clinical Procedure: - Specialty: Pediatrics and Neonatology.

Objective: Challenging differential diagnosis.

Background: Shunt surgery is a common solution for hydrocephalus in infancy. Slit ventricle syndrome and secondary craniosynostosis are late-onset complications after shunt placement; these 2 conditions occasionally occur together.

Case report: We report a case of early-onset secondary craniosynostosis with slit ventricle syndrome after shunt surgery in an infant, which led to a catastrophic increase in intracranial pressure (ICP). A 4-month-old girl with a Dandy-Walker malformation underwent a ventriculoperitoneal shunt procedure. Her head circumference (HC) gradually decreased to approximately the 5(th) percentile for her age group after shunt surgery. Seven months later, she developed increased ICP symptoms and underwent a shunt revision with a diagnosis of shunt malfunction. Her symptoms were temporarily relieved, but she repeatedly visited the emergency room (ER) for the same symptoms and finally collapsed, with an abrupt increase in ICP, 3 months later. Further evaluation revealed the emergence of sagittal synostosis at 7 months after initial shunt surgery. After reviewing all clinical data, slit ventricle syndrome combined with secondary craniosynostosis was diagnosed. Emergent cranial expansion surgery with shunt revision was performed, and the increased ICP signs subsided in the following days.

Conclusions: Clinical suspicion and long-term HC monitoring are important in the diagnosis of slit ventricle syndrome and secondary craniosynostosis after shunt surgery, even in infants and young children.

No MeSH data available.


Related in: MedlinePlus

(A) 3D-CT brain image and (B) intraoperative photograph taken before cranial expansion surgery, with the same orientation. The closed sagittal suture is clearly visible on both images. (C) Sagittal suturectomy with barrel-stave osteotomy was performed. (D) Postoperative brain CT image 1 day after cranial expansion surgery. The ventricles and sulci are all effaced.
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f3-amjcaserep-15-246: (A) 3D-CT brain image and (B) intraoperative photograph taken before cranial expansion surgery, with the same orientation. The closed sagittal suture is clearly visible on both images. (C) Sagittal suturectomy with barrel-stave osteotomy was performed. (D) Postoperative brain CT image 1 day after cranial expansion surgery. The ventricles and sulci are all effaced.

Mentions: A proximal shunt obstruction was again suspected because of the slightly expanded left lateral ventricle and the dry shunt tap. However, the right lateral ventricle was completely obliterated where the majority of proximal catheter holes were located. The slit ventricle mechanism might have been working, disabling the shunt system. Reviewing the radiological images, we found that the patient’s sagittal suture was invisible on the CT scan. 3D-CT images confirmed the closure of a sagittal suture. We re-evaluated all previous CT scans, and the closure of the sagittal suture began at 7 months after initial shunt surgery (at the time of the previous shunt revision) (Figure 2). Slit ventricle syndrome combined with secondary craniosynostosis was diagnosed. It was not clear whether the shunt malfunction was temporary or permanent. Emergent cranial expansion surgery and shunt revision were performed to relieve the ICP and to restore shunt function. The closed sagittal suture was opened wide, and several barrel-stave osteotomies were made coronally. The shunt proximal catheter and valve were replaced simultaneously (Figure 3).


Slit ventricle syndrome and early-onset secondary craniosynostosis in an infant.

Ryoo HG, Kim SK, Cheon JE, Lee JY, Wang KC, Phi JH - Am J Case Rep (2014)

(A) 3D-CT brain image and (B) intraoperative photograph taken before cranial expansion surgery, with the same orientation. The closed sagittal suture is clearly visible on both images. (C) Sagittal suturectomy with barrel-stave osteotomy was performed. (D) Postoperative brain CT image 1 day after cranial expansion surgery. The ventricles and sulci are all effaced.
© Copyright Policy
Related In: Results  -  Collection

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getmorefigures.php?uid=PMC4061147&req=5

f3-amjcaserep-15-246: (A) 3D-CT brain image and (B) intraoperative photograph taken before cranial expansion surgery, with the same orientation. The closed sagittal suture is clearly visible on both images. (C) Sagittal suturectomy with barrel-stave osteotomy was performed. (D) Postoperative brain CT image 1 day after cranial expansion surgery. The ventricles and sulci are all effaced.
Mentions: A proximal shunt obstruction was again suspected because of the slightly expanded left lateral ventricle and the dry shunt tap. However, the right lateral ventricle was completely obliterated where the majority of proximal catheter holes were located. The slit ventricle mechanism might have been working, disabling the shunt system. Reviewing the radiological images, we found that the patient’s sagittal suture was invisible on the CT scan. 3D-CT images confirmed the closure of a sagittal suture. We re-evaluated all previous CT scans, and the closure of the sagittal suture began at 7 months after initial shunt surgery (at the time of the previous shunt revision) (Figure 2). Slit ventricle syndrome combined with secondary craniosynostosis was diagnosed. It was not clear whether the shunt malfunction was temporary or permanent. Emergent cranial expansion surgery and shunt revision were performed to relieve the ICP and to restore shunt function. The closed sagittal suture was opened wide, and several barrel-stave osteotomies were made coronally. The shunt proximal catheter and valve were replaced simultaneously (Figure 3).

Bottom Line: Challenging differential diagnosis.A 4-month-old girl with a Dandy-Walker malformation underwent a ventriculoperitoneal shunt procedure.Her symptoms were temporarily relieved, but she repeatedly visited the emergency room (ER) for the same symptoms and finally collapsed, with an abrupt increase in ICP, 3 months later.

View Article: PubMed Central - PubMed

Affiliation: Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul, Republic of Korea.

ABSTRACT

Patient: Female, 14 months

Final diagnosis: Slit ventricle syndrome Symptoms: Hydrocephalus • lethargy and seizure • vomiting

Medication: - Clinical Procedure: - Specialty: Pediatrics and Neonatology.

Objective: Challenging differential diagnosis.

Background: Shunt surgery is a common solution for hydrocephalus in infancy. Slit ventricle syndrome and secondary craniosynostosis are late-onset complications after shunt placement; these 2 conditions occasionally occur together.

Case report: We report a case of early-onset secondary craniosynostosis with slit ventricle syndrome after shunt surgery in an infant, which led to a catastrophic increase in intracranial pressure (ICP). A 4-month-old girl with a Dandy-Walker malformation underwent a ventriculoperitoneal shunt procedure. Her head circumference (HC) gradually decreased to approximately the 5(th) percentile for her age group after shunt surgery. Seven months later, she developed increased ICP symptoms and underwent a shunt revision with a diagnosis of shunt malfunction. Her symptoms were temporarily relieved, but she repeatedly visited the emergency room (ER) for the same symptoms and finally collapsed, with an abrupt increase in ICP, 3 months later. Further evaluation revealed the emergence of sagittal synostosis at 7 months after initial shunt surgery. After reviewing all clinical data, slit ventricle syndrome combined with secondary craniosynostosis was diagnosed. Emergent cranial expansion surgery with shunt revision was performed, and the increased ICP signs subsided in the following days.

Conclusions: Clinical suspicion and long-term HC monitoring are important in the diagnosis of slit ventricle syndrome and secondary craniosynostosis after shunt surgery, even in infants and young children.

No MeSH data available.


Related in: MedlinePlus