Developmental Medicine:
An Orthopedic Surgeon’s Perspective

Charles R. Reina, MD.
*Orthopedic Consultant at Hunterdon Developmental Center, Clinton NJ,

*Assistant Clinical Professor Orthopedic Surgery Hahnemann Medical School/ Easton   Hospital Affiliate.
*Lecturer Allentown College of St. Francis de Sales.

The practice of medicine has developed from a series of experiences which date from
early civilization. Up until the Renaissance, when the empirical approach was developed by
many notable pioneers throughout the globe, the treatment of illness and injury was
relegated to the priestly class and those persons designated by personal choice, if not social
stature or training. The treatments delivered ranged from the organized body of then
acceptable and at times published if not oral tradition. Cults, religious applications, and
alchemy gave way to the science of observation and the method of testing hypotheses-the
birth of the "scientific method". As time evolved the epochs of knowledge and their varied
modes of reporting and describing treatments matured through desultory applications to
well-defined, consistent practices occurring over ever decreasing time periods. A calculus of
sorts; taking the “limit to zero” and contracting the time between scientific
accomplishments. Information readily available to the trained, contained in the austere
halls of study.

Today, anyone can access the formerly secret world of untested knowledge to learn
about the daily explosion of new information unimaginable to the giants like Hippocrates,
Democritus, Galen and Harvey. Where Galileo, Kepler, Brache and Huygens were amazed
by their observations of the aggregate worlds of the solar system; we can examine the
electron microscopic worlds of molecules and decipher the genetic code. With this ability
we have, as a profession, discovered better, faster and eventually less expensive and
intrusive treatments. Our patients are now our partners in information and education. Our
roles become coordinators instead of directors. Our information in the electronic dawn of
the twenty-first century may come to us through the layman, in lay journals and media as
well as by professional periodicals and internet sources.

Bearing these preliminaries in mind, we must not forget to “see, hear, smell, and
touch” our ill or injured charges.  Placing aside the scientific blossoming of the preceding
five years, our training as “medical scientists” whose ideal is to reach an absolute solution
but by necessity accept the near truth must always place the fundamentals of the history,
observation and examination of our patients above the reading of pixels and figures. For in
biology when we add 2 plus 2 we may get a set of answers as 3.8, 4.1, 3.6 and maybe 4.0. In
the physical sciences, nothing farther from 4.0 is successful let alone useful. In fact we may
send a probe to Jupiter and hit the moon instead or complete a bridge from opposite shoes
only to be feet apart and not as accurate as the fractions of an inch tolerance of the great
pyramid. Our intuitiveness and analysis of the particular patient’s needs, wants,
possibilities and probabilities along with that immeasurable quantity of divine intervention
or just luck mixed with skillful knowledge must be primary in the outline of a treatment. A
treatment which may depart from the cyber or printed word of experts and tested methods
to an application of skillful modification and relative harmlessness in the totality of a
holistic approach. “Primum non nocere,” has a lot to do with knowing what not to do as
well as providing a cookbook of methods based upon a tautology of experience better suited
to a “Fertile, forty year old, 70 kg white female with fever and right upper abdominal
pain.”

Having completed a "tour d’horizon" of medical history and  experience of which about
everyone in medicine can attest, I now wish to offer a few observations made during over
18 years experience as an orthopedic surgeon who consults for a state institution which cares for the developmentally disadvantaged by birth trauma or genetics, perinatal neurological
infection or metabolic insult and unable to be cared for at home. A spectrum of intelligence
and personalities, of paraplegics and di- or monoplegics, of ambulatory to bedridden young
and old with varying degrees of associated spasticity or flaccidity, metabolically imbalanced
to hormonally deficient, genetically deficient to neurologically unstable and combined with
hidden dangers of hepatic, gastrointestinal and renal disorders is only the beginning! A
patient population with subtle to obvious musculoskeletal deformities or problems insidiously developing in an adolescent to a septuagenarian with a panoply of family support and/ or governmental regulators all of which is nurtured medically by a staff of dozens from all walks of life, central and peripheral to the maintenance of dignity and well-being. Those of us even remotely involved as I, in a weekly orthopedic clinic marvel at the overwhelming paucity of complications and lurking disasters.

In the last 15 years, in the atmosphere of transforming trends in personal and scientific approach treating these people, I have understood how little we do know and how often we can make a problem worse by forgetting Occam’s Razor- to keep things simple; to avoid doing harm through commission as well as omission. Without the time-worn application of statistical methods to test a hypothesis for its credibility and thus explicit acceptance medico-legally, I cannot adequately prove or pontificate about those observations I make and present as treatment suggestions. Note well that I did not say treatment maxims or rules. There is no dogmatism in much of this dialectic. I actually depend upon the urgings or inspirations which hopefully spring from an appreciation of my more philosophical introductory paragraphs. And so, without further obfuscation I suggest the following:

A. Managing the painful limp: The variety of neurological and
temperamental combinations not withstanding, the orthopedist must always give heed to the
observations by the caring staff, attendants and therapists to determine the level and
gravity of change and its evolution. The implications of a stress fracture, septic hip or
neoplastic spinal element in a verbally challenged, at times belligerent patient cannot be
overstated. The history must include a team experience not just a primary doctor’s terse
reason for consultation. If it is practical, the observations of many than that of one
transporter unfamiliar with the patient or untrained to observe the daily routines is a big
help. Examination includes the critical and often one-shot opportunity to see that antalgia as
a behavioral quirk or an ataxic spell; even a variant seizure activity. The hypertonic,
uncooperative may suggest a septic joint requiring general anesthesia or sedation under
controlled sterile conditions - a potential danger to a few with cervical instability or cardiac
arrhythmia. All so obvious yet often motivated by liability concerns the orthopedist must
weigh the factors of fever, trauma or behavioral history and not often physical signs of
 localizing more benign problems as an ingrown toenail, sprained ankle, pressure sore or
callous from a poorly fitting orthosis or worn inner lining of a sneaker. X-rays are
traditional but a bone scan may be needed STAT at the local hospital familiar with these
patients- and oral or parenteral sedation a must when ruling out occult fracture from back
to toe.

B. The swollen joint: No problem here. An effusion of a knee, ankle or elbow can
result from a friction of crawling habits to banging for attention. The overlying redness or
heat and/or superficial nearby abrasion may suggest an infectious bursa or more serious
septic joint in a non-ambulatory individual. Again the appearance of aspirated fluid from a
well restrained patient to avoid iatrogenic or self inflicted inoculation of hepatitis virions
may necessitate an anti-inflammatory drug when coagulopathy , renal , hepatic or GI
associated conditions permit. Just plain warm compresses or wraps for the less sedate more
PICA oriented person may resolve the swelling as Lab, and X-rays are readied. .Looking at
recurrent effusions may prompt a synovectomy and, if bloody, a biopsy for villonodular
synovitis, occult trauma or a developing Charcot neurarthropathy. There’s that history,
again.

C. That displaced fracture: When diagnosing an intra-articular fracture the
neurovascular implications of non-operative care are universally applied as are the
considerations to malunion and its attendant painful arthritis. This is tenuously balanced
with the concerns of a belligerent patient whose uncontrolled rage may take an impending
compromise in the nonoperative case to one with floating hardware and a destroyed
anatomically reduced fracture. Unless imminent, closed treatment is probably safest in the
otherwise extra-articular yet adjacent fracture. Proper sedation for up to three or four
weeks may be imperative to maintain cast integrity; though in some neuromuscular patients
at the expense of deconditioning an ambulatory patient.  I didn't say it was easy!
The functional prognosis for a finger fracture or irreducible or unstable interphalangeal or
metacarpal-phalangeal joint must be weighed before jumping into wire fixed fragments or
dislocated joints imperative for the office patient pianist or artist or surgeon but potentially
infectious, osteomyelitic, or broken hardware in this clientele will make the best of
reductions not worth the subsequent disaster. Buddy taping and mittens ( to reduce self
abuse or removal)  changed prn but at least daily to reduce salivary or sudomotor
maceration may be a difficult modification to “complicated perfection.” It requires more
labor intensity but without more potential harm. Of course, the primary doctor more
familiar to the family or guardian MUST transmit the orthopedist’s explicit rationale for
such “skillful deviation.” This last statement applies to ALL treatment modifications and
may NEED TO BE INITIATED BY THE PRIMARY PHYSICIAN understanding the
variation through dedicated team work with the consultant.

D. Even casting can be dangerous: So you have decided that the patient is better
suited for nonoperative care of a displaced, unstable but neurovascular intact limb. Even
the “simple” fibula fracture clinically confirmed as not being an invidious unstable ankle  fracture can be casted if prevention is applied. Overpadding to reduce pressure in a recumbent limb paralyzed or just immobile from patient inertia can obviate that skin graft for a cast ulcer. The deformity MUST BE EQUATED with the functional prognosis. A prognosis which includes the perfunctory, well-intentioned expertise of a great looking intraoperative reduction and fixation with the post operative failed fixation of urinary incontinence soiling in a non-foley catheterized patient, or the unintentionally abusive personality who self mutilates or picks at the incision inoculating S. aureus. Sometimes a colleague’s second opinion may defuse a charged medicolegal briar patch. The careful communication with the caring guardians is again an absolute like 2 plus 2 IS 4 in this
scenario.

E. The unstable spine. How little guidance is manifest in the popular common orthopedic literature. In the flurry to publish in our research oriented residencies and the plethora of famous and obscure journals of which we are inundated, contain admonitions, dictates and expertise. Yet, it is the frontline treating physician who, though sometimes incompletely read (how can one read everything or expect the premise to be properly tested and tried-true) and overwhelmed, must decide what is best for the individual presented. Cervical spine instability in Morquio syndrome, rheumatoid arthritis or the ubiquitous Trisomy 21 patients is potentially overrated, overirradiated, overtreated. Some studies , the few which commit themselves, suggest yearly lateral active NOT PASSIVE cervical spine films if the C1 - C2 interval exceeds 4 - 6 mm and then only until late adolescence or third decade when the incidence of increase “subsides.” Then every five years unless upper motor neuron signs intervene. At risk activities like horseback riding or contact sport, more likely, may have to be precluded in the over 6mm spine. Don’t overlook the empirical midcervical spine degeneration and hypertrophic spine in the fourth decade trisomy-21 patient. A
reciprocal instability may develop later in life if the lower neck, which contributes 50%
motion is reduced and is compensated for by the insidious C1-C2 laxity.  Scoliosis, that’s another few pages. Suffice it to say that neuromuscular disorders notoriously deteriorate despite contoured chairs and TLSO bracers. Yet, the deformity not operatively corrected will stiffen and deform sufficiently to preclude any combined major anterior and posterior spinal release and bone grafting with internal fixation. Consider the osteoporotic whose bone is too soft for any fixation. Or the osteomalacic from long term dilantin use, here “skillful deviation”(from practice standards) is a must to prevent perioperative pulmonary or even neurologically compromising complications.

Well I have winded you enough to hopefully induce another dialogue on the “skillful modification/ deviation” necessarily individualized approach towards only a few of the myriad of extraordinary and routine symptoms confronted in this population. This developmentally challenged patient AND challenging treatment rationale  development must be continually reexamined for the pitfalls of common treatment modalities successful in other populations. The increase in interactive complications of treatment as the co-factors of other disorders, age, idiosyncrasies converge to make well-intentioned yet untailored plans reek havoc. It should be “de rigeur” to act as physicians at the bedside, hand to chin, eyebrows furrowed and stance askew while we contemplate not the recent journal article or the potential for one to be published but the manner of approach for that “Forty year old developmentally challenged patient with chromosomal deletion syndrome, joint laxity, minimally ambulatory, diabetic with spastic hemiparesis, on depakote with an intraarticular
ankle fracture and swollen hot knee.”

Now that is the reason we entered this profession!