bubble_chart Overview

Congenital hydrocephalus, also known as infantile hydrocephalus, typically involves the continuous production and absorption of cerebrospinal fluid (CSF) within the cranial cavity, maintaining a dynamic balance. If excessive production or impaired absorption and circulation occurs, the ventricular system or subarachnoid space may accumulate an excessive amount of CSF, leading to expansion and the formation of hydrocephalus. Clinically, it can be classified into two types: communicating hydrocephalus and obstructive hydrocephalus. The former involves a patent ventricular system, while the latter results from blockages within or at the exits of the ventricular system. The main clinical manifestations include abnormal head enlargement after birth, neurological dysfunction, and signs of increased intracranial pressure. The incidence of this condition shows no regional or gender differences, and surgical intervention is the primary treatment. Prognosis varies significantly, with most cases being favorable and intelligence quotient (IQ) potentially returning to normal. Poor outcomes are often associated with delayed treatment, missed surgical opportunities, or the presence of severe malformations in other organs.

bubble_chart Clinical Manifestations

1. The head progressively enlarges abnormally, often appearing weeks or months after birth, and may gradually manifest even after one and a half years. The head enlarges while the face appears relatively small, with distended scalp veins, enlarged fontanels, and widened cranial sutures. Percussion produces a "cracked pot sound."
2. Neurological impairment: Inability to move both eyes upward, presenting as the "setting sun sign," intellectual disability, and possible spastic paralysis of the limbs.
3. Symptoms of increased intracranial pressure: Repeated vomiting, head grabbing, crying, and even drowsiness.

bubble_chart Diagnosis

1. Abnormal enlargement of the infant's head, bulging and full anterior fontanelle, recurrent vomiting and crying, "cracked pot sound" on cranial percussion, and bilateral eyeballs showing the "setting sun sign."
2. Skull X-ray examination shows an enlarged cranial cavity, thinned skull bones, widened cranial sutures, and an expanded fontanelle.
3. Brain ultrasound reveals symmetrical enlargement of the bilateral ventricles.
4. Head CT or MRI can visualize the extent of ventricular enlargement and measure cortical thickness, identifying the site of obstruction and the disease cause of hydrocephalus.

bubble_chart Treatment Measures

1. The disease is primarily treated surgically and can be divided into three categories: (1) surgery to remove the cause of obstruction; (2) surgery to reduce cerebrospinal fluid secretion; (3) cerebrospinal fluid shunt surgery.
2. For patients with significant intracranial hypertension before surgery, dehydration therapy to lower intracranial pressure may be used.
3. Symptomatic treatment, infection prevention, and nerve nutrition therapy are also administered.

The occurrence of this disease is related to the deficiency or excess of certain vitamins during the fetal period, as well as genetic factors from both parents. Therefore, proper dietary adjustments and avoiding consanguineous marriages can reduce the incidence of the disease. As the saying goes, "A big head means great intelligence," which often leads some parents of children with abnormally enlarged heads to harbor wishful thinking, thereby delaying treatment. Recognizing this disease should serve as a cautionary lesson. Hence, when progressive abnormal head enlargement is observed in infants after birth, it is advisable to consult a specialized neurosurgeon at a major hospital to achieve early diagnosis and early surgery, ensuring normal intellectual development in the child.

bubble_chart Cure Criteria

1. Cure: The head stops enlarging or shrinks, intracranial pressure is normal, and intellectual development is normal.
2. Improvement: Symptoms of increased intracranial pressure are alleviated, and neurological symptoms are stable.
3. No cure: The original symptoms do not improve, and intelligence and growth development are poor.