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

The transition of the patient’s HC on a standard Korean girl’s head circumference chart (marked with red dots) [15]. Dramatic changes in HC around the cranial expansion surgery are described in the table. The patient’s HC declined after shunt surgery and remained small thereafter.
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f4-amjcaserep-15-246: The transition of the patient’s HC on a standard Korean girl’s head circumference chart (marked with red dots) [15]. Dramatic changes in HC around the cranial expansion surgery are described in the table. The patient’s HC declined after shunt surgery and remained small thereafter.

Mentions: Immediately after the operation, the patient’s HC increased from 43.0 cm to 45.0 cm. At postoperative day (POD) 2, her HC increased to 47.5 cm. She was still lethargic, with little spontaneous activity. A CT scan showed effacement of all cerebral sulci and ventricles. Mannitol was infused every 4 hours to reduce brain swelling. At POD 3, the patient’s HC decreased to 46.5 cm, and she became alert, with spontaneous activity. Her HC decreased gradually thereafter, and she was discharged at POD 8. One month later, she was healthy, without neurological sequelae, and her HC was 43.7 cm (5th–10th percentile), slightly greater than the preoperative level. Nine months after cranial expansion surgery (age 23 months), she could walk without assistance, climb up stairs, and play with puzzles, and her HC was 45.2 cm (3th–5th percentile) (Figure 4).


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)

The transition of the patient’s HC on a standard Korean girl’s head circumference chart (marked with red dots) [15]. Dramatic changes in HC around the cranial expansion surgery are described in the table. The patient’s HC declined after shunt surgery and remained small thereafter.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4061147&req=5

f4-amjcaserep-15-246: The transition of the patient’s HC on a standard Korean girl’s head circumference chart (marked with red dots) [15]. Dramatic changes in HC around the cranial expansion surgery are described in the table. The patient’s HC declined after shunt surgery and remained small thereafter.
Mentions: Immediately after the operation, the patient’s HC increased from 43.0 cm to 45.0 cm. At postoperative day (POD) 2, her HC increased to 47.5 cm. She was still lethargic, with little spontaneous activity. A CT scan showed effacement of all cerebral sulci and ventricles. Mannitol was infused every 4 hours to reduce brain swelling. At POD 3, the patient’s HC decreased to 46.5 cm, and she became alert, with spontaneous activity. Her HC decreased gradually thereafter, and she was discharged at POD 8. One month later, she was healthy, without neurological sequelae, and her HC was 43.7 cm (5th–10th percentile), slightly greater than the preoperative level. Nine months after cranial expansion surgery (age 23 months), she could walk without assistance, climb up stairs, and play with puzzles, and her HC was 45.2 cm (3th–5th percentile) (Figure 4).

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