Achalasia is a neurological disorder of the esophagus and the muscles associated with it. Achalasia is a Greek term which means lack of relaxation. It involves the sphincter muscles of the esophageal region which help in the movement of food in the alimentary by esophageal peristalsis. The upper esophageal sphincter muscle measures about 3- 4 cm and is composed of striated muscles. The lower or the distal esophageal sphincter muscle is smooth. The esophagus is further comprised of circular and longitudinal muscles which help in the peristaltic movement. The muscles of the mesenteric plexus have a significant role in the occurrence of conditions such as achalasia. This is because of the association of the mesenteric plexus with intramural nerve innervations. Studies reveal that the damage of the mesenteric plexus is caused by the immune system.
Clinical manifestations of Achalasia
Classical symptoms associated with achalasia include dysphagia, regurgitation and respiratory complications. The lower esophageal sphincter muscles are impaired in this condition causing distended lower esophagus. In this region, the food is stuck as the muscles of the lower esophageal sphincter do not relax causing disturbance in the peristaltic movements of the esophagus. The patient experiences pain or spasms as a result of the cramped food in the esophagus. Often there is a tendency to vomit followed by heartburn and weakness.
Incidences of chest pain and breathing difficulties due to nocturnal choking are not uncommon. Histological examination of the esophagus reveals the decrease in myenteric neurons which are predominantly responsible for the relaxation of the lower esophageal sphincter muscles. Achalasia is categorized as primary, secondary and pseudoachlasia depending upon the etiology of the disease. The primary cause of achalasia can be hereditary or underlying autoimmune disease. The secondary cause is associated with preexisting infections such as chagas disease. It is usually associated with malignancy.
Diagnosis of Achalasia
Achalasia onset is generally asymptomatic and the severity increases after five years. Patients who are probable suspects of achalasia are diagnosed using radiologic, manometric and endoscopic methods. The manometric analysis determines the esophageal pressure of the lower origin. This enables the peristalsis and relaxation associated with the esophagus. It also indicates the functionality of smooth muscle contraction pertaining to the lower esophageal region. Radiological analysis indicates the abnormalities in structural arrangement of the esophagus. The bird beak appearance of the esophagus is the classical sign for the occurrence of achalasia. A normal chest X-ray does not identify the presence of achalasia, but it gives an anatomical description of the respective changes pertaining to esophagus such as widening of the mediastinum due to esophageal dilation and presence of gastric air bubble which occurs because of lack of relaxation in the lower esophagus.
Treatment of Achalasia
The restoration of the esophagus is difficult. However, many treatment options are available for achalasia. Use of nitrates and calcium blocking drugs help in the prevention of calcification of the esophagus. In some cases, balloon dilation of the lower esophagus is done. Although this method has a short time recovery there is always a risk of perforation during the procedure. Surgical methods such as thoracotomy and myotomy are considered. In addition to these treatment options, endoscopic administration of botulinum toxin has also become a possible option.
Blount's disease or 'tibia vara,' is a growth disorder in the shin bone that affects the bones of the lower leg causing the lower leg to angle inward. This resembles a bow leg.
Named after the American orthopedic surgeon, Putnam Blount (1900 - 1992), Blount's disease is characterized by progressive lower limb deformity. Though Blount can affect people at any time during the growing process, it is more common in kids younger than four and in teens. A lot of pressure is put on the growth plate on the top of the tibia. This portion is called the physis - made out of cartilage, weaker than bone. The function of the physis is to allow the bone to lengthen and grow.
Due to excess pressure, the bone does not grow normally and instead the lateral outer side of the tibia keeps growing whereas the medial or inner side of the bone does not. Because of uneven bone growth, the tibia tends to bend outward instead of growing straight. Blount is not the same as naturally bowed legs that babies and toddlers have which usually straighten out when they start walking.
Blount is described as two distinct forms, early or infantile and late or adolescent Blount disease.
Infantile Blount disease is diagnosed between age one and three years. The disease presents when a child begins to ambulate. This disease is often bilateral and is less commonly associated with obesity.
Quite unlike the infantile Blount, late onset of Blount disease occurs in older children and is commonly associated with obesity and is often unilateral.
A combination of mechanical and biological factors influences Blount's disease to varying degrees. The mechanical forces contributing to the disease are weight of the child, age at walking, and varus deformity. The compressive forces across the medial femoral physis lead to growth retardation. Adolescent Blount does not appear to be progressive, or as common as the infantile form.
The cause of Blount disease remains controversial but it is mostly due to a combination of hereditary and developmental factors. There is increased incidence of the disease in overweight children who walk at an early age. Certain theories that mechanical overload of the proximal tibia contribute to Blount disease has been found. This mechanical overload is attributed to obesity and varus deformity. But this alone cannot be a cause as the disease is also noticed in children with normal weight.
Increasingly it is more common in people of African heritage, where kids start walking at an early age and whose family member might have had it. There is a genetic component to the disease as well, though a direct pattern of inheritance has not been clearly revealed. Hence, Blount is multifactorial and may differ in the early or late onset forms of the disease.
It is imperative to understand that Blount disease starts in early childhood or late teen years, the curve can get worse if not treated. Hence early diagnosis is important. The most obvious sign of Blount is bowing of the leg below the knee. While in young kids it is usually not painful, it teens it can be. It can feel like a growing pain in the knee area. The pain may come and go and many teens resort to over-the-counter pain relievers. As the lower leg bears the weight of the body, other problems such as rotation of the tibia are noticed. This causes a condition called in-toeing, wherein the feet point inward instead of straight out. Blount disease, over several years, can lead to arthritis of the knee joint and trouble walking. One leg may become slightly shorter than the other.
If there is knee pain that seems to be getting worse and cannot be traced to an injury, then the doctor might possibly consider Blount. A complete physical examination will be done, and X-rays of legs taken. The doctor will look for any abnormal growth pattern at the top of the tibia - a tell tale sign of Blount. This will help the doctor measure how severe the bowing is.
Treatment for Blount depends on the age of the patient and how far the disease has progressed. Young kids are advised braces, which are long-legged and lock the knee and need to be worn whilst weight bearing. But bracing is usually unsuccessful in girls and those with obesity. Older kids and teens will need surgery. There are different types of surgeries to correct Blount disease. These involve cutting the tibia, realigning it and holding it in place with plate and screws. This procedure is called Osteotomy. Sometimes, the damaged growth plate is removed and a device called external fixator is used to hold bones in place from the outside. In case of a twisted toe, surgeons correct the cause of it. Surgery is done under general anesthesia, and the patient might wear a cast and use crutches for a while. Physical therapy will be needed after surgery.
Spinal anesthesia is administered through injection to anaesthetize or numb the lower part of the body before performing surgery on the patient. Spinal anesthesia is a type of regional anesthesia and will block the pain below the site of the injection. This kind of anesthesia is injected into the spine of the lower back through a long needle. The medicine is directly administered into the fluids surrounding the spine. This is normally given between L3 - L4. Unlike general anesthesia, here the patient is awake and conscious during the procedure.
Surgeries performed under spinal anesthesia
Spinal anesthesia suits simple surgical procedures involving short duration. It is a single shot and hence medicine is given only once and no catheter is placed for further medication. Once the medicine is administered, spinal anesthesia starts working instantly. As soon as the shot is given, patient will begin to experience a hot feeling in the lower part of the body and subsequently the area becomes numb. Spinal anesthesia is appropriate for the surgeries performed below the umbilicus like:
Spinal anesthesia procedure
Firstly, patient will be positioned appropriately. The patient will be made to sit on the edge of the operating table keeping the stool under the legs for support or the patient will be made to lie down on one side, knees tucked up to the chest. Once positioned, the lower back will be cleaned with antiseptic lotion. Small dose of local anesthesia will be given at the site where the spinal will be administered so that patient does not feel the piercing pain. The anesthetist administers the spinal injection and will remain in the operation theatre throughout to check the progress and the effect of anesthesia.
Spinal anesthesia complications
Spinal anesthesia is less risky when compared to general anesthesia. Patient can return to normalcy in no time and also there are less chances of developing chest and lung infections and other respiratory disorders. However, spinal anesthesia may give rise to few side effects like:
These conditions are quite manageable and treatable and will wane off within few hours after the procedure. Neurological injury is an extremely rare complication that may be caused by spinal anaesthesia.
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Bibliography / Reference
Collection of Pages - Last revised Date: September 23, 2019