A RARE CASE OF COMPLICATED SURGICAL TREATMENT OF INBORN OCCLUSIVE HYDROCEPHALUS IN A CHILD Yakushin O.A., Novokshonov A.V.
Kuzbass Clinical Center of Miners' Health Protection, Leninsk-Kuznetsky, Russia,
Tsyvyan Novosibirsk Research Institute of Traumatology and Orthopedics, Novosibirsk, Russia
Hydrocephalus is an abnormal condition of inborn or acquired origin which is characterized by excessive accumulation of liquor in ventricular systems and intrathecal spaces, with their extension and compression of brain structures [1, 2]. In the structure of incidence of diseases in children, the incidence of hydrocephalus is 1-4 cases per one thousand of newborn children, and it achieves 1 % in children older 3 years [3, 4, 5].
Currently, hydrocephalus, which is classified in sections Q03 (inborn) and G91 (acquired) according to ICD-10, remains one of the leading abnormalities and attracts attention by doctors of various specialties.
The main method for treatment of hydrocephalus is surgical correction of natural ways of liquor flow (endoscopic surgical techniques) or creation of bypasses for draining of ventricular system of the brain (ventriculo-peritoneal shunting) [6].
Objective – to present a case of rare complication (valvular pump insufficiency) after ventriculo-peritoneal shunting in a child with inborn occlusive hydrocephalus.
MATERIALS AND METHODS
The study was conducted in compliance with World Medical Association Declaration of Helsinki – Ethical Principles for Medical Research Involving Human Subjects, 2013), and the Rules for Clinical Practice in the Russian Federation (the Order by Health Ministry of RF, 19 June, 2003, No. 266), with approval from the local ethical committee of Kuzbass Regional Center of Miners' Health Protection.
The article presents a clinical follow-up of early complication of surgical management of a child (age of 11 months) with progressing course of inborn internal occlusive hydrocephalus of degree 3 and dysfunction of previously installed liquor-shunting system. The cause of dysfunction of the ventriculo-peritoneal shunt was a fracture of the connector of the valve pump. The child received revision replacement of the ventricular drain and valve pump. At the background of the performed treatment, improvement in general condition and regression of general cerebral symptoms were noted.
A child (age of 11 months) was admitted to the neurosurgery unit No. 2 of Kuzbass Regional Clinical Center of Miners' Health Protection. The diagnosis: "Inborn internal occlusive hydrocephalus of degree 3, with progressive course. Condition after ventriculo-peritoneal shunting with implantation of valve pump of low pressure (on 1.02.2016). Shunting system dysfunction. Hypertension-hydrocephalus syndrome".
The child was urgently admitted. At the moment of admission, the child's mother reported on some complaints in her child: presence of a tumor-like formation in the left parietal region in the plane of previously installed valve pump; fatigue and drowsiness.
The history of disease progression on the basis of information from the mother and medical documentation. The child has been observed by neurologists from the age of 3 months, when additional examination techniques showed inborn internal occlusive hydrocephalus. Conservative management was carried out. On December, 2015, control examination was conducted. Progression of hydrocephalus was noted at the background of conducted treatment.
After consultation by the chief of the neurosurgery unit No. 2, Novokshonov A.V., MD, PhD, the child was admitted to the surgery unit. The surgery was performed as planned: ventriculo-peritoneal shunting with implantation of the low pressure valve pump to the left (Fig. 1). During surgical treatment, the shunting system of one of the leading foreign producers was installed. The postsurgical period was without complications. The wound healed with primary tension. The positive time course in neurological status at the background of treatment. The child was discharged for outpatient neurological follow-up according to place of residence.
Figure 1
The child, age of 11 months. Brain MSCT after surgical treatment (ventriculo-peritoneal shunting)
On March, 2016, the child was on inhospital treatment for hyperthermia at the background of acute respiratory viral infection in the pediatric unit. The pediatric neurologist gave a consultation. An examination showed a mass in the plane of previously installed valve pump of the ventriculo-peritoneal shunting system. The child's mother denied a fact of an injury. The boy was examined by the neurosurgeon. After correction of acute respiratory viral infection, considering the presence of signs of dysfunction of the ventriculo-peritoneal shunt, the child was urgently admitted the neurosurgery unit No. 2.
The objective examination at admission: general condition of middle severity which is determined by neurological symptoms. The child has normal body composition and satisfactory nutrition. Cry is loud, voice is normal. The skin is pale, with mild acrocyanosis, with paleness of nasolabial triangle. Mucosa is clear and wet. Moderate bright hyperemia in the oropharynx. Tonsils are quaggy, without deposits. Tragus palpation is positive on both sides. Body temperature − 36.5°C.
Nasal breathing is laboured slightly, muculent secretion. The chest is symmetrical, with equable participation in breathing. Pulmonary noise by percussion. RR − 40-42 per minute. Harsh breathing in lungs, without rale. Cardiac tones are rhythmical and clear, without abnormal noise. HR − 146 per minute. The abdomen is symmetrical, soft when palpated, painful in all parts.
Locally: the head is of moderate hydrocephalic shape, big fontanel 3 × 3 cm. There is a postsurgical U-shaped well-fixed connective tissue scar in the left parietal region. There is a elastic mass (3 × 4 × 4 cm) in the plane of the skin aponeurotic flap in the site of the previously implanted valve pump (Fig. 2). There is a fluctuation in the plane of the mass.
Figure 2
The child, age of 11 months. A tumor in the left parietal region in the plane of the installed valve pump of liquor-shunting system
Neurological examination. The child is in consciousness. The response to examination is slightly decreased, with events of fussiness. The face is symmetrically innervated. Swallowing is active. Palpebral fissures D = S, the pupils are equal, no nystagmus during examination. Muscular tone is increased in proximal parts of the hands and moderately high in the lower extremities in their proximal parts. Range of movement in hip joints is sufficient. Tendon reflexes are high and equal. Grasping reflex is positive. Normal support to lower extremities. step by step − on bended knees, on toes − non-constant. No meningeal signs.
Considering the mother's complaints, the disease history, data of objective examination and additional techniques of examination, presence of signs of dysfunction of ventricular segment of the ventriculo-peritoneal shunt and valve pump, the child received the urgent surgery on 11.03.2016: ventriculo-peritoneal shunting, revision and remounting of the low pressure valve pump. Narcosis − endotracheal. Surgery duration − 40 minutes. Surgery course according to the protocol: the U-shaped incision in the left parietal region (about 5 cm) along the postsurgical scar without its dissection. The skin aponeurotic periosteal flap was formed. During flap formation, free effuse of clear liquor was observed. The valve pump was separated from scar tissues. Revision showed a fracture of the plastic connector in the site of fixation of the distal end of the end of the drain (Fig. 3). The posterior horn of the left lateral ventricle was punctured with the ventricular silicone catheter with the mandrin. Clear liquor was on the depth of 2 cm, with high pressure effuse. The system of ventriculo-peritoneal shunting was mounted with installation of the low pressure valve pump. The valve pump was installed in the site of the bone defect and was fixed to periosteum. The functioning of the pump was satisfactory. The wound was sutured in layer-by-layer manner in the plane of surgical approach.
Figure 3
A fracture of the plastic connector of the valve pump in the site of fixation of the distal end of the liquor-shunting system drain
Stay in ICU was 1 day in the early postsurgical period. Drug therapy was carried out. Healing with primary tension. Positive time trends in neurological status at the background of treatment. The mother did not present any complaints. The child was active. Good sleeping. Digestion is normal. The child is conscious. Look is focused and cognitive. The child babbles and smiles. He can support his head well. The face is symmetrically innervated. Swallowing is active. Palpebral fissures are equal. Pupils D = S. Muscular tone is high in the hands in proximal parts, and moderately high in legs. Tendon reflexes are high and equal. No meningeal symptoms. The child has been discharged for neurological outpatient treatment on the 14th day of hospital stay.
RESULTS AND DISCUSSION
The conducted analysis of literature data shows that incidence of identification of hydrocephalus in children of early age has a trend to increase that is currently determined by decreasing infant mortality and increasing quality of care for premature infants with very low body mass [6].
The main etiological risk factors of hydrocephalus in children of early age are isolated anomalies and malformations of central nervous system; birth injury, perinatal, hypoxic-ischemic and hemorrhagic disorders of cerebral perfusion; inflammatory diseases of central nervous system in both ante- and postnatal period; untimely addressing to specialized medical neurosurgery centers or inadequate correction of liquor dynamics disorders [4].
Neurosurgeons have various techniques for surgical correction of hydrocephalus. Over time, the main surgical technique of hydrocephalus in children of early age is shunting surgery [7, 8]. Currently, the main technique of shunting interventions is ventriculo-peritoneal shunting (97.7 % of all implantations) [9].
Despite the fact that shunting surgery is quite technically simple, incidence of complications is high − 50-80 % of cases in the first 3-5 years after surgery [5, 10]. The literature describes some rare cases of postsurgical complications after shunting such as slit-like ventricle as result of hyperdraining of ventricular system [8] or migration of abdominal end of shunting system into scrotum [11].
However, the most common complication of liquor shunting operations is dysfunction of shunting systems (58-78 % of cases within the first year after surgery). One of causes of shunt dysfunction is its mechanical disconnection, resulting in increasing hypertension syndrome and, as result, to worsening general condition of a patient [4].
We have not found any publications, in which the cause of dysfunction of shunting system is mechanical disconnection of the shunt due to a fracture of the connector of the valve pump.
CONCLUSION
In the presented clinical case of treatment of the child with occlusive hydrocephalus, we met with a quite rare case of dysfunction of ventriculo-peritoneal system determined by a fracture of the connector of the valve pump, resulting in recurrent surgery and revision replacement of the ventricular drain and valve pump.
Information on financing and conflict of interests
The study was conducted without sponsorship. The authors declare the absence of any clear or potential conflicts of interests relating to publication of this article.