Sensenbrenner syndrome, also known as cranioectodermal dysplasia (CED), is a rare autosomal-recessive disorder belonging to the ciliopathy group of diseases. It is characterized by a facial dysmorphism, abnormal bone development and ectodermal defects including dental anomalies. CED is a heterogeneous condition with significant phenotypic and molecular variability, whose spectrum may include cases of renal impairment, hepatic fibrosis, retinitis pigmentosa and/or brain anomalies. In many cases, patients develop chronic kidney disease (CKD) due to nephronophthisis between 2 and 6 years of age. The aim of this retrospective study is to better understand the characteristics of this syndrome and to find prognostic factors of CKD. We make the hypothesis that an early diagnosis of the syndrome would lead to a better global management of patients (quality of life, delayed onset of end-stage renal disease).

Inclusion Criteria:

• Boys and girls.
• Aged 3 to 18 years old.
• Subjects with a Sensenbrenner's syndrome diagnosis and followed from 2005
• Parents/ legal guardian must provide non opposition prior to participation in the study

Exclusion Criteria:

• Patients whose parents / legal guardian have object to using the data usually collected for care

Children with Sensenbrenner syndrome