TORCH Syndrome

At a glance

An acronymic syndrome referring to a group of fetal infectious malformations. It stands for Toxoplasmis, Other agents, Rubella, Cytomegalovirus, and Herpes simplex. Common signs involve essentially intracranial anomalies.

Incidence

Unknown (eg, in cytomegalovirus infection approximately 1% of all newborns are infected, but only 10% present symptoms at birth).

Pathophysiology

TORCH Syndrome refers to infection of a developing fetus or newborn by any of a group of infectious agents. Other agents include: Coxsackie, Treponema pallidum (syphilis), HIV, parvovirus B19, and herpes zoster.

Diagnosis

Association of intrauterine growth retardation, a hepatosplenomegaly, microcephaly, eye anomalies (chorioretinitis), jaundice, intracranial calcifications, and high intracranial pressure. Blood screening is possible.

Clinical aspects

May include fever, difficulties feeding, small areas of bleeding under the skin (petechial rash), hearing impairment, abnormalities of the eyes, seizures, mental retardation. Each infectious agent may also result in additional abnormalities.

Precautions before anesthesia

Evaluate neurological function (clinical, CT, MRI, EEG) and liver function (clinical, echography). Laboratory investigation should include bilirubin, serum glutamic-oxaloacetic transaminase (SGOT), serum glutamic-pyruvic transaminase (SGPT), coagulation test, hematocrit, and platelet count.

Anesthetic considerations

Challenges of the handicapped child. Vascular access can be difficult in cases of edema. Patient may be prone to bleeding problems, ensure sufficient blood products available.

Pharmacological implications

Avoid succinylcholine and ketamine if possible due to concerns of intracranial pressure. Consider interaction between anesthetic drugs and antiepileptic medications. If bilirubin level is very high, it can displace other drugs from albumin, thereby increasing the free fraction and clinical effect of drugs such as muscle relaxants.

Other conditions to be considered

• Pseudo-TORCH Syndrome (Baraitser-Reardon Syndrome, Baraitser-Brett-Piesowicz-Syndrome; Pseudotoxoplasmosis Syndrome, Microcephaly with Calcification of Cerebral White Matter Syndrome): Characterized by microcephaly, quadriplegia, seizures, developmental delay, marked microphthalmia, congenital cataracts, cerebral and cerebellar hypoplasia, and intracranial calcification without evidence of intrauterine infection. Spasticity. Previous reports of features resembling intrauterine infection and associated with an autosomal recessive inheritance have been reported.

• ☞Congenital Varicella Syndrome: Rare congenital nongenetic disorder because of maternal transmission of varicella in the first and second trimesters of pregnancy manifesting with cutaneous, neurological, and limb involvement.

• ☞Aicardi-Gouttieres Syndrome (Cree Encephalitis; Encephalopathy, Familial Infantile, with Calcification of Basal Ganglia and Chronic Cerebrospinal Fluid Lymphocytosis, AGS-1): Characterized by progressive familial encephalopathy in infancy, calcification of the basal ganglia and chronic cerebrospinal fluid (CSF) lymphocytosis, evolving rapidly to a refractory epilepsy, vegetative state, and early death in infancy. It has been suggested that it is a distinct type of leukodystrophy transmitted as autosomal recessive.

References