Jon Matthew Farber, MD

Results of a retrospective study examining the prevalence and severity of complications in patients with Hirschsprung disease (HD) for whom surgery was anticipated but had not yet been performed, found that 17% of such patients developed complications. Infants awaiting surgery who had total colonic aganglionosis (TCA) were at signi cantly greater risk of complications than those with short-segment disease.

Investigators from the Netherlands analyzed data for 132 patients with HD who received surgery at their center during a 1-year period. Patients with HD usually undergo surgery when they are aged 3 to 6 months. Of the 22 patients who developed complications, 20 had at least 1 major complication, with the remaining 2 having complications that were minor. Some infants developed multiple complications: 5 patients developed 2 complications, 2 patients developed 4, 1 patient developed 6, and 1 patient developed 8.

The most common complication was bowel perforation: 6 cases were caused by transanal irrigation (TAI), 1 by Hirschsprung-associated enterocolitis, and 2 associated with meconium ileus. There were 2 cases of central venous line sepsis and 4 of sepsis caused by bowel perforation. The third most prevalent complications were ileus and persistent obstructions. Most often the complications arose in the hospital (64%), with the remainder appearing at home. Mean age at presentation of complications was 4 weeks (range, 0-25 weeks) with 10 patients having short-segment disease (45%), 3 with long-segment disease (14%), and 9 with TCA (41%).

Investigators also found that a TAI-induced perforation in TCA can be highly

dangerous. One child in the study group with such a perforation died and another was at risk of doing so. Given these findings, investigators recommended limiting the wait time for surgery in children with HD and suspected TCA.

If your surgical colleague suggests waiting until a child with TCA is older and larger, which would make surgery technically easier, suggest they read this article.

Beltman L, Labib H, Oosterlaan J, van Heurn E, Derikx J. Risk factors for complications in patients with Hirschsprung disease while awaiting surgery: beware of bowel perforation. 

Published online March 2, 2022. doi:10.1016/j.jpedsurg.2022.02.022

Articles

Results of a study in patients with Hirschsprung disease for whom surgery was anticipated but had not yet been performed found that 17% developed complications.

Delaying surgery for Hirschsprung disease may be risky

Teenagers with attention-deficit/hyperactivity disorder (ADHD) or parent-reported “trouble staying focused” are poorer drivers and make more driving errors than their peers during the teenagers’ learning permit period.­ These findings are based on an analysis of survey data from a prior randomized study in which 512 parent-teenager pairs were randomized to a web-based parent-teenager driving plan intervention or a usual practice control condition for 24 weeks during the learner’s permit period and were surveyed several times, including at the end of the study period.

Of the 134 adolescents who also underwent on-road driving assessments at 24 weeks and had complete survey data, 113 (84.3%) were typically developing (TD) adolescents, 12 (9.0%) had ADHD, and 9 (6.7%) had trouble staying focused. Sociodemographic variables were similar for all 3 groups. Overall, TD teenagers did better behind the wheel than did adolescents with ADHD or those who had difficulty staying focused.

Specifically, teenagers who had difficulty staying focused were more likely than those with ADHD or TD to have their on-road driving assessment (administered by a certified driver rehabilitation specialist) terminated because the specialist had to intervene to prevent a collision, the driver violated a traffic law, or because of another serious driver action or inaction. In addition, teenagers who had difficulty staying focused received marginally lower overall driving scores compared with TD teenagers, whereas those with ADHD made significantly more total errors than TD teenagers. As for specific tasks, teenagers with ADHD made more errors during high-demand tasks and right-turn tasks compared with TD teenagers, whereas teenagers with trouble staying focused made more errors during “straight at intersection tasks.”

Teenagers with trouble staying focused and those with ADHD also committed the most so-called critical errors, most often dangerous maneuvers, followed by disobeying traffic signs, lane violations, speeding, or striking an object or curb (Bishop HJ, et al. 

The authors also comment that no specific programs are available to help drivers with ADHD improve. Teenaged drivers, with or without ADHD, remain a concern. Just remember that a license says the state feels a child can drive, but the parents still need to decide if a child should drive. You’ll find that 

Teenagers with attention-deficit/hyperactivity disorder (ADHD) or parent-reported “trouble staying focused” are poorer drivers and make more driving errors than their peers during the teenagers’ learning permit period according to recent survey data.

Learning to drive poses extra risks for teens with attention problems

1. If a child has had severe respiratory syncytial virus (RSV), or a febrile seizure, the parents will be very worried the next time their child is ill. Acknowledge this fear, let them know what to watch for, and assure them that after a few benign illnesses, and seeing that their child does well, they will be less anxious in the future.

2. For night terrors, scheduled awakenings can be of benefit. After determining when the terrors are likely to occur, the parent can awaken the child shortly before the expected time, for several nights in a row. This apparently interrupts the cycle and can relieve the problem.

3. Showing parents reliable images online of what you are diagnosing (eg, urate crystals in the diaper) or not diagnosing (the actual rash of erythema chronicum migrans when the family is worried about Lyme disease) is very helpful.

4. Infants and young children are much smarter than we are in some ways. Thus, an adult who has hurt his neck might try to convince his doctor that he is not really hurt, but not so for youngsters. If a child is moving his or her neck freely after an injury, he or she has not suffered a serious spinal injury, and will not need x-rays.

5. The Oralflo cup (Oralflo Technologies; Raritan, New Jersey), or others, can be useful for helping children to swallow pills.

6. TGuard, formerly ThumbGuard, devices (MED et al, Incorporated; Matthews, North Carolina) can be useful for thumb-sucking. Bitter tasting products that are applied to the thumb seem less likely to work, unless the child is motivated to stop. That is, they appear to operate more as a reminder to take the thumb out, rather than as an actual deterrent.

7. I post tidbits about children where they can be easily seen whenever I open the chart. Examples of items that might go here include how to pronounce names, the child’s accomplishments/interests, if the family brought me homegrown tomatoes, and if a parent has a serious medical condition. Most electronic health records have an easy way to do this.

8. Update your equipment when needed. Stethoscopes develop cracks that can interfere with listening. The MacroView otoscope (Welch Allyn; Skaneateles Falls, New York) is vastly superior to the ones of the previous generation.

1. Frank NC, Spirito A, Stark L, Owens-Stively J. The use of scheduled awakenings to eliminate childhood sleepwalking. 

Jon Matthew Farber, MD, shares more observations on office practice and some useful websites for patient information.

Plain talk about office practices

Body mass index (BMI) percentile in tall children is sometimes overestimated.

 That is, a child who is aged 12 years with a BMI of 19.7 is at the 75th percentile, but a 10-year-old the same height and weight will be above the 85th percentile. Similarly, BMI percentile in short people is sometimes underestimated.

Glycerin, baking soda, and hydrogen peroxide

 have all been reported to be effective at treating cerumen buildup,

 but I have not been that impressed by the results in my patients.

 like doing warm compresses for the eye by making a cup of hot water

, dipping the washcloth tip in, and then, when the washcloth cools, redipping a different corner. This provides ongoing heat and a sufficient length of time.

Stroking down firmly along the spine can induce voiding in infants,

 if you are waiting around for a bagged specimen. Tapping rapidly for 30 seconds over the bladder has also been reported to help.

Phenazopyridine (Azo Standard), now available over the counter,

 can be used for dysuria in bladder infections in children until the antibiotic kicks in. For children in obvious pain, I usually suggest half a pill, crushed, every 8 hours as needed. Remind parents that it turns urine and tears orange.

 in addition to having them stop bubble baths, remind the parents to shampoo the child as the last thing before getting out of the tub, so she is not sitting in soapy material throughout the bath.

Some girls with dampness in their underwear are not incontinent,

 but leaking urine that has pooled in the vagina. Voiding while facing backwards on the toilet, forcing the legs apart, can help.

I have been staying away from rice cereal

 for infants because of reports of arsenic contamination. That having been said, I am not aware of any studies showing rice cereal causes harm, so parents need not feel guilty if they have been serving it.

Growing pains are almost certainly muscular.

Nine-month-olds have trouble letting go of objects.

 Giving a child this age 2 blocks to hold will usually allow you to use your stethoscope/otoscope without the infant grabbing it.

. For example, a patient with a mild cough for 2 weeks may come in with the parents wanting an antibiotic. The books do say one can start an antibiotic now for a possible sinus infection, but I am not convinced and I usually prefer to wait. If you tell parents you want to give it another week to see if the cough resolves on its own, and that you will phone in an antibiotic if the child is not better - without having to see him or her again- they will usually be satisfied with that.

12. In the above scenario, when I do start with the antiobiotic, 

I tell parents it often does not work, and to stop it if the child is not better after 3 to 4 days

. There is no point in a full 10-day course in this setting.

when she wanted her child to give up pacifiers, had the child bring them in so I could donate them to other children

. This solved the pacifier problem while also encouraging altruism, and I was happy to go along with mild deception.

1. Soy FK, Ozbay C, Kulduk E, Dundar R, YazÃ±cÃ± H, Sakarya EU. A new approach for cerumenolytic treatment in children: in vivo and in vitro study. 

2. Baxter MP, Dulberg C. "Growing pains" in childhood--a proposal for treatment. 

This month, I share several observations from various parental concerns.

13 tips for tackling parental concerns

	1. I see a fair number of children with mild anemia who do not respond to iron and have normal indices, red cell distribution width (RDW), iron/ferritin levels, reticulocyte counts, electrophoresis, and smears. I consider them in general to have “statistical anemia.” That is, these children are normal-at the lower end of the curve-just as there are people less than the second percentile in height who have no discernible medical cause. They do not need further evaluation or yearly testing.

	2. It is well known that an acute viral illness, such as parvovirus, can cause transient red cell aplasia in a hemoglobinopathy such as sickle cell. Viruses can also suppress red cell production, to a lesser degree, in children without underlying blood disorders. Thus, if you find an otherwise healthy child with an acute viral illness who has a mild anemia during a routine checkup, consider retesting the child when he or she is well rather than, for example, conducting a trial of iron supplementation.

	3. Similarly, when testing for causes of anemia, remember that ferritin is an acute phase variant, and can be spuriously elevated if a child is ill at the time.

 can occur throughout the body. In my experience, it is found often enough in a fourth location that perhaps it should be called “hand-foot-mouth-buttocks.”

	5. A positive leukocyte esterase urine test in an adolescent male suggests testing for Chlamydia and gonorrhea, which are much more likely than a urine infection.

	6. Gluteal hair shaving or laser hair removal

 can be used to prevent recurrent pilonidal infection. I have not seen any studies showing the value of over-the-counter depilatories here, but it makes some sense to me.

	7. Pseudomonas can cause folliculitis from a hot tub

 (topical treatment will suffice); and wound infection from a puncture through a sneaker

	1. Segre D, Pozzo M, Perinotti R, Roche B; Italian Society of Colorectal Surgery. The treatment of pilonidal disease: guidelines of the Italian Society of Colorectal Surgery (SICCR). 

	2. Centers for Disease Control and Prevention (CDC). Pseudomonas dermatitis/folliculitis associated with pools and hot tubs-Colorado and Maine, 1999-2000. 

	3. Schaefer P, Baugh RF. Acute otitis externa: an update. 

	4. Jacobs RF, McCarthy RE, Elser JM. Pseudomonas osteochondritis complicating puncture wounds of the foot in children: a 10-year evaluation. 

Consider these helpful hints when diagnosing anemia or infections in your patients.

Smarter testing for anemia and infections

	However, he or she may still decide to leap. That is why counseling is also helpful.

	Many parents read that autism rates rose when the measles-mumps-rubella (MMR) vaccine was introduced, so they are afraid of the vaccine despite numerous studies disproving a link. The autism rate has continued to rise despite a falloff in immunization rates. I propose an alternative pseudo-theory to explain this: Along with autism rates, cell phone usage has continued to rise. Furthermore, cell phones are known to emit radiation, which can obviously affect the brain. The conclusion is obvious. Perhaps “non-vaxxers” should be giving up their cell phones rather than vaccines. 

	Hence, vaccines should be spaced out. An equally valid theory (it has no empirical support, either) is that autism is due to the number of separate times vaccines are given, so spacing vaccines out increases the risk of autism. This last premise appears to be true in my practice, where a child is 

 spaced out. The reason for this is not due to the vaccine spacing, of course, but to the positive family history for autism, which increases the risk for autism while pushing parents to postpone immunizations. Of course, logical arguments such as the above will not be effective for true believers, but might sway others.

	Children with autism with large vocabularies but poor comprehension will often give associative answers (eg, what sleeps-bed). Questions and norms for this test can be found in Table 20 in the study “Arnold Gesell’s developmental assessment revalidation substantiates child-oriented curriculum” (Guddemi M, et al; 2014).

www.autismspeaks.org/family-services/tool-kits/100-day-kit

	1. Guddemi M, Sambrook A, Wells S, et al. Arnold Gesell’s developmental assessment revalidation substantiates child-oriented curriculum. 

This month I offer some observations on developmental disorders-ADHD and autism.