Riedels thyroiditis, is a chronic form of thyroiditis. It is now believed that Riedels thyroiditis is one manifestation of a systemic disease that can affect many organ systems called IgG4-related disease. It is often a multi-organ disease affecting pancreas, liver, kidney, salivary and orbital tissues and retroperitoneum. The hallmarks of the disease are fibrosis and infiltration by IgG4 secreting plasma cells. Signs and symptoms
Pathophysiology
Riedels thyroiditis is characterized by a replacement of the normal thyroid parenchyma by a dense fibrosis that invades adjacent structures of the neck and extends beyond the thyroid capsule. This makes the thyroid gland stone-hard (woody) and fixed to adjacent structures. The inflammatory process infiltrates muscles and causes symptoms of tracheal compression. Surgical treatment is required to relieve tracheal or esophageal obstruction. Diagnosis
It typically present as a painless, hard anterior neck mass, which progresses over weeks to years to produce symptoms of compression, including dysphagia, dyspnea choking and hoarseness. Patients may present with symptoms of hypothyroidism and hypoparathyroidism as the gland is replaced by Fibrous tissues .physical examination reveals a hard,"woody" thyroid gland with fixation to surrounding tissue. The diagnosis needs to be confirmed by open thyroid biopsy because the firm and Fibrous nature of the gland renders FNAB inadequate

Treatment
Therapy usually consists of prednisolone, nonetheless some cases may require surgery. Tamoxifen has been proposed as part of a treatment plan.Treatment is directed to surgical relief of compressive symptoms. Tamoxifen may also be beneficial.The type of surgery which is indicated here is isthmectomy. Prevalence
Riedels thyroiditis is classified as rare.
Cornelia de Lange syndrome (CdLS) is a genetic disorder. People with this syndrome experience a range of physical, cognitive, and medical challenges ranging from mild to severe. The syndrome has a widely varied phenotype, meaning people with the syndrome have varied features and challenges. The typical features of CdLS include thick or long eyebrows, a small nose, small stature, developmental delay, long or smooth philtrum, thin upper lip and downturned mouth.The syndrome is named after Dutch pediatrician Cornelia Catharina de Lange, who described it in 1933. It is often termed Brachmann de Lange syndrome or Bushy syndrome and is also known as Amsterdam dwarfism. Its exact incidence is unknown, but it is estimated at 1 in 10,000 to 30,000. Signs and symptoms
The phenotype of CdLS is highly varied and is described as a spectrum; from Classic CdLS (with a greater number of key features) to mild variations with only a few features. Some people will have a small number of features but dont have CdLS.Key features:

Long and/or thick eyebrows
Short nose
Concave nasal ridge  and/or upturned nasal tip
Long and/or smooth philtrum
Thin upper lip vermilion and/or downturned corners of mouth
Missing fingers or toes
Congenital diaphragmatic herniaOther suggestive features:

Developmental delay and/or intellectual disability
Small prenatal and birth size / weight
Small stature
Microcephaly (prenatally and/or postnatally)
Small hands and/or feet
Short fifth finger
HirsutismThe following health conditions are more common in people with CdLS than in the general population.