Fallot’s Tetralogy in a 8 y.o. girl

While on his community service, my friend Dr. Víctor Hugo Roa-Castro, just diagnosed a girl with Fallot’s Tetralogy (a congenital heart disease), wich consists in the combination of a ventricular septal defect (VSD) with Pulmonary Stenosis, with the Aorta “Overriding” (sitting “astride”) the VSD and with right ventricle hypertrophy (RVH).

It’s about 10% of all types of congenital heart disease. Both sexes affected with slight male predominance. Detected weeks or months after birth. Clinical features are cyanosis from birth or developing in the first year of life caused by the lung’s inability to oxygenate sufficient blood for the system, difficulty in feeding, failure to gain weight, retarded growth and physical development, dyspnea on exertion, clubbing of the fingers and toes, and polycythemia.

Here is the clinical case:

A “previously healthy” 8 years old came to Víctor’s consult because she has been feelling with fatigue in the last 7 months and her mother states that she tought that her daughter wasn’t sick despite her blueish tones in her skin since she was born.

On the clinical exam, Victor found this:

Cyanosis in the perioral area

Central cyanosis

Central cyanosis

Cyanosis & clubbing of the fingers

Cyanosis & clubbing of the fingers

Click on the player to hear the murmur

wich was a 3rd grade systolic in the 3rd left intercostal space.

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On the EKG: aQRS 150 grades, right ventricle hypertrophy, right atrial enlargement, transition V2-V3.

On the Chest X-ray: Situs solitus, cardio-thoracic index 0.6. Boot shaped heart with an upturned apex and a concave main pulmonary artery segment. Pulmonary flow appear slightly decreased.

On the Echocardiogram: Interventricular abnormal communication (restrictive type), Aorta overriding, right ventricular hypertrophy, infundibular pulmonary stenosis.

Many thanks to Dr. Víctor Hugo Roa-Castro for the case.

Volkmanns ischemic contracture

The brachial artery is the major artery that transverses the antecubital fossa; injury to the brachial artery will cause radiating pain, decreased skin temperature, decreased pulses, and pallor of the distal arm and may result in Volkmann contracture.

The Volmann’s ischemic contracture is a contracture of the flexor muscles and median and ulnar nerve palsies. a long-term sequelae of compartimental syndrome and posterior elbow dislocation.

It could be prevented by early microvascular repair of brachial artery injuries.

Leprosy (Hansen’s disease)

Leprosy has a rich history dating to biblical times.

“Leper” is an ancient term used to identify patients with leprosy who were severely stigmatized by the deformity that resulted from Mycobacterium leprae infection.

In the clinical context, the term Hansen’s disease should be used instead of leprosy. Gerhard Henrick Armauer Hansen, a Norwegian physician, discovered the microbe in 1874 just before Koch’s discovery of Mycobacterium tuberculosis.

In Mexico, in the last 16 years the prevalence of Hansen’s disease has been reduced about 95%, from 16,694 reported cases in 1990 to 820 reported cases in 2006. The World Health Organization (WHO) established the goal of eliminating leprosy as a public health problem by the year 2000, with “elimination” defined as a reduction in prevalence to <1 case per 10,000 population in all endemic countries. The six countries with the highest rates of endemic leprosy in 2001 are: Brazil, India, Madagascar, Mozambique, Myanmar, and Nepal Transmision
This disease is probably spread by the respiratory route, like tuberculosis.

Risk Factors
Close contact
Older persons
Impaired cell mediated immunity

Clinical manifestations
The cardinal manifestations of leprosy are infiltrative skin lesions, hypoesthesia, and peripheral neuropathy
The clinical manifestations of leprosy are closely related to the polarity of the cellular immune response to M. leprae in the patient.
The history of a patient with suspected leprosy should include whether the person has resided in an area with high prevalence and whether the person has been previously diagnosed or treated for leprosy. Certain patients may deny knowledge of a prior diagnosis or may report that skin lesions or neuropathy or both are acute, as they wish to avoid the stigma of a diagnosis of leprosy, even in emigrants to developed countries.

If lepromatous leprosy is left untreated, it can progress to “leonine” facies as seen in the following images.

This includes physical exam and skin biopsy.

Once monthly: Rifampin, Dapzone and Clofazimine for two years and then stop.
Daily: Sulfone and Clofazimine for two years and then stop.

Results of treatment

Limb to crotch (updated)

Israel Sarrío arrived at the Rehabilitation Center of Levante from Hospital Peset in Valencia (Spain) on January 31st of 2004 with an amputation of the left arm 2 inches above the elbow.

Amputated ArmTrunnionrx
Click on images for large size

At 14:00hrs, the microsurgery implant began. The first maneuver consisted in lavage and sterilization of the amputated arm and its connection to the brachial artery by a silicone tube in order to revascularize it.

Later we rebuilt the humerus and repaired the brachial artery, the basilic and cephalic veins, and the three nerves of the arm.

This proccess lasted 21 hours, and the reimplantation was successful. The patient went to the Intensive Care Unit during that night and the next day, he went to a normal room.

first implant

Two days later, we found a wound infection that was seriously compromising the viability of the reimplantation. We talked to the family and they told us that the day of the accident the arm fell in a drain. This contamination was the probable cause of the unfavorable progress. The situation was critical, and we said to the family that there were two different options: reamputate the arm or try to save it by taking it into a healthy zone where it would be possible to nourish it while we cleaned the infected area.

wound infection

The idea of taking the arm to another anatomical location came by reading a similar case published by Michael Wood (Mayo Clinic, Rochester, MN, USA) in which he attached an arm in the groin. The uniqueness of our case consisted of using the procedure to rescue the limb from an infection, called a Deferred Transitory Heterotopic Implant. This was the first such case of involving an elbow.

With the agreement of the family it was decided to transfer the arm to the groin where large blood vessels are readily accessible. In 4 hours we disassembled the reconstruction performed earlier and connected the blood vessels of the arm to those of groin by means of microsurgery.

implant in dangerleg implantleg implantleg implant

The psychological impact on the patient was very important. As soon as he woke up, we told him that the arm was infected and we have to save it in other location on his body. After a couple of hours he realized that the arm was on his leg. He stayed that way for nine days with daily cleanings until it was verified that the stump was clean.

On February 12nd of 2004, we reimplanted the arm in its original place, rebuilding bone, arteries, veins, nerves, muscles and skin in a procedure of 6 hours.

replantpost replant

Afterwards, the patient was doing physically and psychologically well, happy that he have not have to lose his arm.

By this time the patient could move his elbow. From the time of the surgery, the repaired nerve functionality is growing at a speed of 1 milimeter per day. In about 6 months we hope that he will be able to move his hand and fingers.

The function which we hoped to obtain from this procedure was better than those obtained with an amputation and the prosthesis. The patient will be able to grasp objects, to perform normal life functions, such as tie his shoes, all with small orthopedic aids.

final status

The complete surgical team of the Unit of Hand and Reconstructive Surgery was involved in this operation, with Dr. Pedro Cavadas leading the team, and Dr. Navarro, Dr. Soler, Dr. Duke and Dr. Landin as assistants.


Quiz: Hair-on-End Skull

I always enjoy the Image Challenge Contest at The New England Journal of Medicine, time ago there was a similar picture of this:

Click on image for large size
Best viewed in large size

U P D A T E: A nice comment and correct answer by Drmikeunoe

We can see generalized osteopenia, widening of the diploetic space, and thinning of the outer table.

This characteristic “hair on end” appearance of the outer table is compatible with the presence of massive hyperplasia of the bone marrow, as can be seen in the homozygous state of beta-thalassemia (i.e. thalassemia major).

Beta thalassemia syndromes are a group of hereditary disorders characterized by a genetic deficiency in the synthesis of beta-globin chains (adult hemoglobin usually is α2β2) resulting in excess α-chains and severe transfusion-dependent anemia, thus causing bone marrow hyperplasia.

Bilateral Coral Shaped Urolithiasis

Staghorn Calculi

coral shaped nephrolitiasis
Click on the image for large size

82 years old woman with chronic urolithiasis (40 years of evolution). She has suffered chronic urinary tract infections, dull lumbar pain, hematuria and end stage kidney disease.

Because of her severe comorbidities, she couldn’t get the operation, and she went to hemodyalisis where she got an infection that finally ended her life.

First surgery with no gravity

zero gravity surgery zero gravity surgery

zero gravity surgery zero gravity surgery

zero gravity surgery zero gravity surgery
Click on images for large size

A french surgical team leaded by Dominique Martin has performed the first surgery in a zero-gravity flight. The patient a 46 years old male, was a volunteer and he just needed local anesthesia in the forearm.

The procedure was successful, and the chief surgeon said: “the operation went right with no complications. Now we know that a human being could be operated while on space”.

The surgical team (three surgeons and two anesthesioligists), was fixed to the floor and worked with the instruments that was fixed with magnets.

Their approval: “More than 400 people had traveled to the space, the possibilities that anyone could suffer an accident are there. With these kind of procedures, things will be easier”

Which is your favorite surgery/procedure?

Everyone have a predilection of a rare surgical procedure.

My favorite surgical procedure is the left thoracoabdominal esophagogastrectomy (proximal esophagogastrectomy with esophagogastrostomy), it is a difficult and plaintiff procedure and not too common, because of its high morbidity and mortality rates.

I have been assisting on once recently and it was nice to be there. Here is a picture of the dissected and tubulized stomach. It requires hard work and perfectly know the anatomy of the region. It is pretty challenging for all the people involved, from patient, to surgeon, anesthesiologist, etc.

Tubulization of stomach

What is your favorite surgery and why?