Early treatment of infants with cranial enlargement and massive ventricular hydrocephalus

  Name of the child: Wang Moumou, female, age at admission: 6 months and 20 days, from Dalian Zhuanghe City, Liaoning Province;
  Complaint: The child was born on September 17, 2009, but two months after birth, in November 2009, she developed drowsiness and frequent vomiting from breast milk, which the family did not pay attention to because they heard it was normal. On March 18, 2010, 5 months after the child’s birth, the child’s head began to enlarge, and the enlargement of the forehead was a distinctive feature, when he was reminded of the vaccination. One month later, on the afternoon of April 1, 2010, when the child was 6 months old, the phenomenon of drowsiness and crying after waking up occurred again, and the mild “sunset sign” of “straightening” of the eyes, i.e., the dark eyeballs of both eyes gazing downward, was found again. Although the symptoms seemed to improve after four days, i.e., on April 5, he still cried every night, so he underwent a brain CT examination at a local hospital and was found to have “severe hydrocephalus. He was then quickly referred to Beijing, and was referred by a specialist at Beijing Tiantan Hospital to see Director Li Xiaoyong (note: he was at the Second Affiliated Hospital of Tsinghua University), and was hospitalized on April 7, 2010, and began to receive cerebrospinal fluid treatment for hydrocephalus.
  Examination on admission
  The examination showed (Figure 1): the child’s head circumference was 50 cm, which was significantly larger than that of children of the same age; the head shape was frontal and occipital expansion; the child could not sit or stand; when picked up by the parents, the child’s legs were straight and could not be stretched; when the parents supported the child to stand, the child’s feet were stretched forward and the thighs and hip and waist were stretched backward, showing the trunk and lower limbs folded at an angle; the eyes had difficulty in upward vision The infant had difficulty with upward vision or “sunset syndrome”.  
  Figure 1: The enlarged head circumference was 50 cm, significantly larger than that of children of the same age; both eyes showed the sunset sign.
  After admission to the hospital, a brain MRI was performed, which demonstrated a massive hydrocephalus in the ventricles (Figure 2 and Figure 3).  
  Figure 2: MRI sagittal image showing severe hydrocephalus in the ventricles and around the brainstem
  Figure 3: MRI axial image showing severe hydrocephalus in the ventricles and around the brainstem
  Hospitalization history and results
  On the third day after admission, on April 10, 2010, a decompressive drainage of intracerebrospinal fluid was performed, and a rapid reduction in head size and significant improvement in cerebral nerve function was observed after treatment.  
  Figure 4: Head circumference reduction of 1 cm after the third day of cerebrospinal fluid treatment
  This treatment was continued until July 19, 2010, which was another week of three months of treatment, when a repeat brain CT showed that the ventricles had basically shrunk to normal (Figure 5). On July 20, 2010, the child underwent a cerebrospinal fluid shunt (Figure 6).  
  Figure 5: CT on July 19, 2010 showed normalized ventricles with overlapping coronal sutures (cause of head circumference reduction)  
  Figure 6: The child underwent cerebrospinal fluid shunt on July 20, 2010
  The child had a good recovery after the cerebrospinal fluid shunt and the incisional sutures were removed on July 26, 2010, just before discharge from the hospital. After treatment, the child had a head circumference of 47 cm, which was 3 cm smaller than before treatment, and was able to stand upright with assistance, roll over and sit up to normal child status (Figure 7). A review of the brain MRI was also performed, which showed that the ventricles had largely returned to normal levels and that the “fluid” around the brainstem had disappeared (Figures 8 and 9). The child was discharged from the hospital on July 28, 2010.  
  Figure 7: Head circumference was 47 cm on July 26, 2010 (3 cm smaller than at the time of hospitalization), and he could stand smoothly with assistance.  
  Figure 8: July 26, 2010  
  Figure 9: July 26, 2010
  Post-discharge follow-up results
  One year after discharge, the child returned to Beijing for a follow-up examination, and on July 29, 2011, the results of the physical examination were: head circumference was 48.5 cm; thinking and behavior were completely normal (Figure 10). On the same day, the brain MRI was repeated and the results showed good brain and cerebellar development and complete healing of hydrocephalus (Figure 11).
  
  Figure 10: Normal head circumference of 48.5 cm; completely normal thinking and behavior
  
  Figure 11: One year after discharge from the hospital, the MRI on July 29, 2011 showed that the hydrocephalus was completely cured.