© Department of Continuing Medical Education, Christian Medical College, Vellore
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Learning Cases November 2004

STUDY 1: SURGERY - CASE OF THE MONTH

A Patient with an Acute Abdomen

Courtesy: Department of Surgery, CMC, Vellore.

HISTORY AND CLINICAL DATA

1. What is the first investigation you would like to do?

   A plain X-ray of the abdomen was taken and is shown below.
   
   Click on the picture to enlarge.
   
   

2. What is your diagnosis?

   Sigmoid volvulus. At laparotomy, there was a 11/2 clockwise twist of the sigmoid colon with no gangrene

3. How would you proceed?

   In this case table lavage was done followed by sigmoidectomy and primary anastomosis of bowel. One week post op the patient continued to have high gastric aspirates and the abdomen was distended.
   
   Click on the picture to enlarge.
   
   

4. Now what is your diagnosis?

   DIAGNOSIS: Post operative adhesive obstruction.

5. How will you manage this patient?

   Conservative management ensuring adequate fluid and electrolyte replacement. Two weeks post op the patient continued to have intestinal obstruction, hence was taken up for re-laparotomy. There was small leak noted at the anastomotic site with small bowel adherent to this point resulting in small bowel obstruction.

6. How will you proceed?

   Release of adhesions, with a proximal diversion ileostomy/colostomy, or a Hartmann`s procedure.

7. When would you close the colostomy?

   Six to eight weeks later. The patient improved after this and was discharged 10 days later.

8. What investigation will you do before closing the colostomy?

   Distal colonogram to ensure that there is no leak at the anastomotic site.

Acknowledgement: Submitted by Dr. Ajay Chaitanya Raju & Dr. Deepak Thomas Abraham, Department of Surgery, CMC, Vellore.

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STUDY 2: RADIODIAGNOSIS – X-RAY OF THE MONTH

A Patient with weight loss & pain in the joints

Courtesy: Department of Radiodiagnosis, CMC, Vellore.

HISTORY AND CLINICAL DATA

INVESTIGATIONS

• Serum calcium 13.6 mg%
• Alkaline phosphatase 1326 U/L.

RADIOLOGY

1. What are the radiological findings in Image 1?

   Image 1: Lateral radiograph of the skull outlines the characteristic mottling of the vault. Alternating areas of lucency and sclerosis produce the `salt and pepper` appearance.
   Causes of lytic lesions of the skull with/ without sclerosis:
        *   Multiple myeloma
         *Metastases
   *Paget`s disease
   *Langerhans cell histiocytosis
   *Hyperparathyroidism
   *Osteoporosis
   

Image 2: AP X-ray of the Hands. Click to enlarge.





2. What are the radiological findings in Image 2?

   Image 2: AP radiograph of the hand shows severe sub-periosteal resorption, particularly on the radial aspect of the middle phalanges and scapoids. And there is tuftal resorption with osteolysis of the phalangeal waist.
   Causes of acro-osteolysis:
   *phalangeal agenesis
   *neurofibromatosis
   *cleidocranial dysplasia
   *pyknodysostosis
   *leprosy
   *frost bite
   *hyperparathyroidism
   *diabetes
   

Image 3: Plain X-ray Abdomen showing Kidney Ureter & Bladder (KUB). Click to enlarge.





3. Discuss the findings in Image 3.

   Image 3: KUB showing pyramidal calcification with sparing of the cortex s/o medullary nehrocalcinosis.
   Causes of medullary nephrocalcinosis include:
   *Hyperparathyroidism
   *Renal tubular acidosis
   *Medullary sponge kidney
   *Renal papillary necrosis
   *Primary hyperoxaluria
   *Causes of hypercalcemia/hypercalciuria
   
   

4. What is your diagnosis?

   Diagnosis: Primary Hyperparathyroidism

5. Discuss the historical background of Hyperparathyroidism.

   In the 1930s Albright described the clinical entity of classic primary hyperparathyroidism on the basis of 17 cases from his clinical practice. Historically, the disorder was marked by characteristic skeletal changes, nephrolithiasis, and neuromuscular dysfunction\r\n\r\nToday, primary hyperparathyroidism is a different entity. Since the advent of chemical screening with an autoanalyzer in 1960s, most cases are discovered in asymptomatic patients with hypercalcemia. Patients may also present with nonspecific complaints of back pain, or they may have osteopenia, as depicted on radiographic studies. Primary hyperparathyroidism is the most common cause of hypercalcemia in the outpatient population, second only to malignancy in the inpatient population. The natural progression of disease in asymptomatic patients is unclear.

6. Discuss the pathophysiology of Hyperparathyroidism.

   Pathophysiology: Normal parathyroid glands function to maintain appropriate serum calcium concentrations and to regulate bone metabolism by means of the production of parathyroid hormone (PTH). In the nonpathologic state, PTH secretion increases in response to low serum calcium concentrations and enhances the synthesis of 1,25-dihydroxyvitamin D. PTH and 1,25-dihydroxyvitamin D act together to increase calcium reabsorption in the gut and kidney and to promote osteoclastic resorption and the demineralization of bone.\r\n\r\nPrimary hyperparathyroidism is caused by an overproduction of PTH, in excess of the amount required by the body. In contrast, secondary hyperparathyroidism involves an increase in PTH levels to meet some bodily requirement. In 75-80% of cases of primary hyperparathyroidism, one or more adenomas account for the overproduction, whereas approximately 20% of cases are secondary to diffuse hyperplasia of all glands. Carcinoma accounts for less than 2% of all cases.\r\n\r\nThe effects of hyperparathyroidism on bone are numerous. Excess PTH results in an increase in bone breakdown by means of osteoclastic resorption with subsequent fibrous replacement and reactive osteoblastic activity. The bone may have microfractures, with subsequent hemorrhage and growth of fibrous tissue and an influx of macrophages. The resulting mass is called a brown tumor because of the brown color of the vascular elements and blood in the mass. The process of bone resorption and fibrous replacement results in the characteristic radiologic features of generalized bone demineralization, resorption, cysts, brown tumors, erosion of the dental lamina dura, and pathologic fractures.\r\n\r\nOther effects of hypercalcemia include nephrolithiasis or nephrocalcinosis, neurologic changes, peptic ulcer disease, and pancreatitis. Incidence of primary hyperparathyroidism in women is 2-3 times the incidence in men, peaks in those aged 40-70 years

7. What are the ways of imaging the parathroids?

   IMAGING

   To image the parathyroid glands prior to a repeat operation for recurrent or persistent disease, technetium-99m sestamibi scanning or MRI are the preferred tests because of their high sensitivities in depicting ectopic or mediastinal glands.

   MUSCULO SKELETAL IMAGING

   The diagnosis of primary hyperparathyroidism is made by means of the laboratory confirmation of an elevated PTH level in the setting of hypercalcemia.
   
   Radiologically, radiographs may yield the most specific findings consistent with the disorder, and radiography is the preferred examination when the clinical findings suggest the disorder.
   
   Radiographs of the hands may yield the diagnostic finding of subperiosteal resorption, which is virtually pathognomonic for the disease. If radiographs of the hands reveal no abnormalities, other sites are unlikely to demonstrate abnormal findings. Dual-energy x-ray absorpiometry (DXA) and quantitative computed tomography (QCT) may provide evidence of osteoporosis consistent with the diagnosis; however, the finding is nonspecific for primary hyperparathyroidism.
   
   Today, the most common radiologic finding in primary hyperparathyroidism is osteopenia, which may be generalized or asymmetric. Fine trabeculations are lost initially, with resultant coarse and thickened trabeculae. The disease may progress with further destruction that results in a ground-glass appearance in the trabeculae. About 30-50% of the bone density must be lost to show changes on radiographs.
   
   Bone resorption may be classified as subperiosteal, intracortical, trabecular, endosteal, subchondral, subligamentous, or subtendinous. Subperiosteal bone resorption is an early and virtually pathognomonic sign of hyperparathyroidism, and it is marked by marginal erosions with adjacent resorption of bone and sclerosis.
   
   An unusual lacelike appearance may be seen beneath the periosteum with an occasional spiculated external cortex, the most common site in hyperparathyroidism is the middle phalanges of the index and middle fingers, primarily on the radial aspect Other sites of subperiosteal resorption include the phalangeal tufts (acro-osteolysis, the lamina dura around the teeth, the medial aspect of the tibia, the humerus the femur, and the distal clavicle . When the resorption extends to the margins of joints, particularly in the hands, wrists, and feet, findings may appear articular.
   
   In the skull, areas of decreased radiopacity are intermingled with sclerotic radiopaque areas, resulting in a classic appearance called the salt-and-pepper skull.
   
   Brown tumors are well-circumscribed lytic lesions of bone that represent the osteoclastic resorption of a confluent area of bone with subsequent fibrous replacement lesions may be single or multiple, with expansion of overlying bone. They may be present in any site, although they usually occur in cortical bone. Common sites include the mandible, clavicle, ribs, pelvis, and femur.
   
   Calcium pyrophosphate dihydrate crystal deposition disease (CPPD) is more common in association with primary hyperparathyroidism than with secondary hyperparathyroidism.
   
   Other radiographic findings in primary hyperparathyroidism include varying degrees of sclerosis, although generalized sclerosis is more common in secondary hyperparathyroidism. Soft tissue and vascular calcification is more common in secondary disease, as is superior and inferior band sclerosis of the spine, which is called rugger-jersey spine. ULTURUUU ULTRASO preferred tests because of their high sensitivities in depicting ectopic or mediastinal glands.

8. What are the other modalities of imaging available?

   NUCLEAR MEDICINE:
   Technetium-99m imaging has a sensitivity of 70-95% in depicting parathyroid tumors, and it allows 3-dimensional imaging with anterior-to-posterior localization of the tumor. Studies reveal equal sensitivities of technetium-99m sestamibi imaging and MRI in the localization of abnormal glands prior to repeat surgery, with sensitivities of 82-85%. By combining the 2 modalities, the sensitivity increases to 94%.\r\n\r\nULTRASOUND:\r\nUltrasonography is one of the primary modalities used to localize parathyroid tumors. The size of the adenoma is usually correlated with the degree of pparathyroid elevation. Adenomas appear as well-defined hypoechoic lesions with potential cystic or necrotic areas. Ultrasonography offers the advantage of depicting potential concomitant thyroid disease, which is present in approximately 40% of patients wih parathyroid disease.\r\n\r\nIn renal imaging, ultrasonography can demonstrate bilateral hyperechoic medullary pyramids consistent with medullary nephrocalcinosis.\r\n\r\nUltrasonography may be preferred for initial preoperative localization, if desired by the surgeon, because of its low risk, low cost, and high sensitivity in depicting glands that are not ectopic or in the mediastinum.

Acknowledgement: Submitted by Dr. S. Murali Krishna & Dr. K. Gopi Krishna, Department of Radiodiagnosis, CMC, Vellore.


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STUDY 3: ORTHOPAEDICS - CASE OF THE MONTH

Obstretic Brachial Plexes Palsy

Courtesy: Department of Orthopaedics, CMC, Vellore.

CASE HISTORY

1. What is the possible diagnosis?

   Erbs palsy

2. How can this condition prevented?

   This can be prevented by avoiding a prolonged labour, difficult forceps delivery, excessive traction and appropriate management of fetal malpositions fetal macrosomia and cephelo-pelvic disproportions.

3. What are the nerves and muscles affected?

   All muscles supplied by C5 C6 from the trunk level is affected like shoulder abductors and external rotators, elbow flexors and supinators, wrist extensors.

4. What is the management in first 3 months?

   Shoulder and elbow passive mobilization exercises through full range for avoiding joint contractures, preventing scarring around the plexus decreasing its intrinsic mobility and follow up in every 3 weeks for nerve recovery and preparing the child for a brachial plexus exploration if needed.

5. What is the indication for referring for brachial plexus exploration and at what age?

   Between 3 and 6 months in case of (a) No biceps recovery after 3 months, (b) Birth weight >3.5 kg

6. What is the prognosis in this patient?

   Biceps activity at 3 months is a good sign of recovery. Child may need a late reconstruction of shoulder external rotation and abduction after the age of 2½ years. If only C5 is damaged: 90% recovery and if C5 C6 & C7 damaged: 60% recovery; Overall recovery: 70%

Acknowledgement: Submitted by Dr. Vrisha Madhuri, Professor & Head, Department of Orthopaedics, CMC, Vellore.

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