What's Your Diagnosis? [logo]

A monthly case study featured in Infectious Diseases in Children, with treatment information and discussion to follow.

by James H. Brien, DO
Special to Infectious Diseases in Children

 

June 2004

The guest columnist and contributor for this two-part series, found in this issue and the July issue of Infectious Diseases in Children, is Louis Giangiulio, MD, who is a board-certified pediatrician in the U.S. Army, currently deployed in Afghanistan. While he is actually assigned to Keller Army Community Hospital at West Point, N.Y., he is attached to the 10th Mountain Division out of Fort Drum, N.Y., which is operating out of Kandahar as part of Operation Enduring Freedom. Dr. Giangiulio graduated from Jefferson Medical College in Philadelphia in 1999. His residency training was at the National Capital Consortium (Walter Reed Army Medical Center in Washington and the National Naval Medical Center in Bethesda, Md.).

In response to my previous requests for input, Dr. Giangiulio documented in pictures several cases of various pediatric infectious diseases. This issue will feature three brief cases and the next issue will present two more.

His official duties are mostly for soldier sick call and dealing with lots of acute injuries and illnesses. He also is often the initial provider for major trauma. All deployed Army physicians are advanced trauma life support (ATLS)–certified for this reason. So, while Dr. Giangiulio is not deployed for the purpose of practicing pediatrics, he has shown once again that if you open a medical treatment facility, sick and injured children will find you. This is true anywhere in the world, but it is especially true in war-torn areas.

At the time of this writing, we are still gathering information about the details of what pediatricians are doing in these deployment environments, but Dr. Giangiulio gives some excellent insight as to what most deployed military physicians, especially pediatricians, experience.

When you read these cases, you should mentally place yourself in a situation where there is a significant language barrier; therefore, very little history may be obtained. Also, you are not going to have much of the laboratory and imaging support you are used to in your facility. And, oh yes, there may be shooting going on nearby!

— James H. Brien, DO

CASE 1

A 10-year-old boy presented to the unit gate with fever and mentally obtunded. On examination, he was found to have a stiff neck and a rash as shown in Figure 1. No other information is available.

What's Your Diagnosis?

  1. Tetanus
  2. Meningococcemia
  3. Haemophilus influenzae type b sepsis and meningitis
  4. Pneumococcal sepsis and meningitis

Figure 1

CASE 2

A 14-day-old boy presents to a forward base treatment facility for feeding difficulty and is referred for further evaluation and care. Apparently, he had been getting gradually worse for about a week. Again, no other history is available regarding the pregnancy, etc. On examination, the baby is noted to be very spastic as shown in Figure 2.

What therapy should be started?

  1. Penicillin G
  2. Pyrimethamine and sulfadiazine
  3. Metronidazole
  4. Acyclovir

Figure 2

CASE 3

A 3-year-old girl was placed in a sack and dropped off at a forward base by her nomadic family, who could no longer care for her. Therefore, there was no history available. She was found to be malnourished and in respiratory distress. She was also noted to have an obvious deformity of her back as shown in Figure 3. She preferred the position shown in Figure 4 to make breathing easier.

What’s the most likely cause?

  1. Congenital scoliosis
  2. Spina bifida occulta
  3. Spinal tumor
  4. Pott disease
Figure 3 Figure 4

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Case 1

The first patient has a fairly straightforward case of meningococcemia with meningitis (B). While pneumococcus and Haemophilus influenzae type b (Hib) may present in an identical fashion, this is fairly classic for meningococcemia. He survived with IV antibiotics (penicillin G) and supportive care with some tissue loss as shown in Figures 5 and 6, taken two weeks after presentation. This case also demonstrates the capability of well-trained physicians, nurses and other supportive personnel in these austere conditions.

While meningococcemia can occur anywhere, it is more common in certain geographic areas such as sub-Saharan Africa and the Middle East. There are recommendations for meningococcal vaccine for travelers to these and other high-risk areas of the world, and all military personnel are routinely immunized. However, the vaccine does not contain antigen from group B meningococcus, which is responsible for many of the cases reported, especially in the Middle East. Therefore, one should remember that anyone in close contact with these patients should receive prophylaxis, regardless of their vaccine status. You are unlikely to ever know what group of meningococcus you are dealing with in a timely fashion, certainly not in these situations.

There are various options for prophylaxis depending on the age of the individual exposed. Oral rifampin can be used regardless of age. Intramuscular ceftriaxone (Rocephin, Roche) can also be used for all but neonates. Oral ciprofloxacin can be used in adults. I would recommend using the Red Book for guidance. Essentially, the recommended doses and choices for prophylaxis are as follows. For neonates, rifampin at 5 mg/kg every 12 hours for 2 days (four doses). For children over 1 month of age and adults, rifampin at 10 mg/kg (600 mg maximum) every 12 hours for 2 days. Ceftriaxone may be used for children older than 1 month and younger than 12 to 15 years of age at a single dose of 125 mg and for older children and adults, 250 mg. For adults, ciprofloxacin can be used as a single dose of 500 mg.

By the way, Dr. Giangiulio pointed out that he also took care of a 1-year-old girl with Hib meningitis. She also had a good outcome with IV ceftriaxone (a commonly available antibiotic that is used in all military hospitals and aid stations). This should also remind us all that Hib is not eradicated, especially in undeveloped countries. We rarely see it in this country because of widespread use of Hib immunizations and herd immunity. But it still occurs fairly commonly in these desperate parts of the world.

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Case 2

Case 2 is a baby with neonatal tetanus. The severe spasms are causing the opisthotonus seen in the figure, and the baby’s mouth was also clenched shut. Prior to transfer, the baby was appropriately treated by the local provider with penicillin and tetanus antitoxin, as they did not have tetanus immune globulin (TIG) available, which would normally be preferred. TIG and penicillin G (choice A), as well as supportive care with sedation, paralytics and assisted ventilation, is fairly routine for managing these patients in our neonatal intensive care units (NICU). And this is how he was treated in this Army hospital. However, he died due to airway complications while on the ventilator. Survival of these neonates is difficult enough in a NICU.

We have a high level of immunity in young adults, which makes this disease very uncommon in the United States. However, it is fairly common in developing countries due to the lack of routine immunizations of the children, who then grow up to be mothers devoid of tetanus antibody to passively transfer to their newborn. The port of entry is usually the umbilical cord, which is easily contaminated at the time of delivery with dirty instruments. The cause is Clostridium tetani, an anaerobic gram-positive bacillus that produces a neurotoxin that blocks inhibitory impulses to the motor neurons. The preferred treatment is as noted above. Metronidazole is the antibiotic of choice for older infants and children. The duration of antibiotic treatment should be 10 to 14 days.

Pyrimethamine and sulfadiazine is the treatment for congenital toxoplasmosis, and acyclovir would be for congenital or neonatal herpes infection. Neither of these infections would present this way.

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Case 3

The third patient most likely had Pott disease (tuberculous spondylitis that has resulted in a gibbus), but this was never proven due to inadequate imaging ability. Gibbus, from the Latin word for hump, is a deformity typical of that shown in Figure 3. Sir John Percivall Pott was a British surgeon (1714–1788) who was active in describing numerous conditions. While spinal tuberculosis had been known for centuries, Pott’s description in 1779 became a classic.

Congenital scoliosis is an uncommon malformation of the spine that results in an abnormal curve in the spine in the young child; it may result in a kyphosis but is not likely to form a gibbus. A spinal or mediastinal tumor is possible, just less likely, especially in a part of the world where tuberculosis is common. Spina bifida occulta is also not as likely to result in a gibbus.

The exact cause will never be known as the child died due to respiratory failure while with her adopted family.

Figure 3 Figure 5
Figure 6 Figure 7

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Dr. Brien’s closing thoughts

The success stories, such as the meningitis cases noted above, are not given much, if any, publicity. However, those children would have certainly died without the intervention of Dr. Giangiulio and other members of his medical unit.

As a result, their parents can celebrate future birthdays at their homes rather than their gravesites. This is how you win the hearts of the “occupied” population, and our military medical personnel are among the best ambassadors that we have.

Through their work, which is usually taken for granted, people are helped in an up-close and very personal way, one at a time. And these people are not stupid. They understand when you have provided a life- or limb-saving procedure or service for their loved one, and it will change them forever.

Dr. Giangiulio returned to the United States with his unit as this column was being completed, and is shown in Figure 7 with his children, Nicholas (14 months) and Francesca (3 years), during the homecoming.

Acknowledgement: Thanks go to Major Phil Chadwick, the brigade surgeon, for his technical assistance with the pictures and electronic transmission.

For more information:
  • James H. Brien, DO, Pediatric Infectious Disease, Scott and White’s Children’s Health Center and Associate Professor of Pediatrics, Texas A&M University, College of Medicine, Temple, Texas. E-mail: jhbrien@aol.com.

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