Female 9 years 7 months old with Haddad syndrome or Hirschprung's disease which is a disorder of colon causing chronic constipation,  resulting from an aganglionic part of bowel from the anus upwards. Also including congenital central hyoventilation, called Ondine syndrome. Presented with hydrocephalus secondary to  intracranial bleeding after birth, treated with ventriculo-atrial shunt.  Presents clinically with valve malfunctioning. She was referred for shunt assessment. Images obtained in the first 5 min after injecting a small bolus of 99m Tc in the pump reservoir (A, left) show severe ventricular CSF reflux with no flow into the distal device consistent with complete obstruction. (B, middle), CT shows hydrocephalus, yellow arrow points to the intracranial valve tube, whose end is located in the right cerebral ventricle. She underwent valvular revision and it was changed by a new one installed from the lateral ventricle to the peritoneal cavity, which reestablished normal flow in the derivative. (C, right), CT after valve replacement depicts ventricular size normalization

References:

Thom AF et al. Medicina Nuclear en la Hidrocefalia del Adulto, p: 491-497 in: Medicina Nuclear Aplicaciones Clínicas. Eds: I. Carrio - P. González. Editorial Masson, Barcelona España, 2003.

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