Patient Presentation
A 25-month-old male came to the emergency room with increased somnolence and breaththrough seizures.
The patient had known congenital hydrocephalus secondary to neonatal meningitis, and usually had well-controlled generalized tonic-clonic seizures on Keppra®.
He had increasing somnolence for 24 hours and 2 seizures in the last 6 hours. He was able to eat and drink with no emesis, and his mother said that he would awaken but was more irritated. He did not complain of pain and had no infectious disease symptoms.
The past medical history showed no previous shunt revisions, but evaluations for possible shunt malfunction had happened 3 times in the past.

The pertinent physical exam showed a small but normal appearing male who was sleeping in his mothers lap. Heart rate was 130 beats per minute with a normal blood pressure and temperature. Growth parameters were 10-25%. HEENT showed 3 mm reactive pupils and a ventriculoperitoneal shunt on the right side. With palpation, no discontinuity of the shunt was noted in the head or neck. Abdomen showed a small scar in the mid right abdomen that was well healed. Abdomen was soft without obvious masses or distention. Neurological examination revealed cranial nerves 3-12 were intact, and deep tendon reflexes were slightly increased in upper and lower extremities with 2 beats of clonus in the feet, consistent with previous examinations. He was easy to arouse but very cranky and wanted to return to sleep. The diagnosis of probable shunt malfunction was made. The radiologic evaluation of a head computed tomography exam showed a discontinuous proximal shunt at the angle of the reservoir and ventriculomegaly. He was taken to the operating room and had the shunt replaced. He did well and was discharged home. The patient’s clinical course one week later revealed him returning to the emergency room with fever and increased somnolence. Head computed tomography at that time showed an intact shunt and he was diagnosed with a viral upper respiratory tract infection. He was well at his last follow-up appointment at 6 months.

Figure 85 – AP radiograph of the skull demonstrates the tip of the VP shunt to be disconnected from its reservoir.

Figure 86 – CT scan of the brain performed without intravenous contrast demonstrates marked dilation of the lateral, third and fourth ventricles in axial (top), coronal (middle), and sagittal (bottom) planes.

Discussion
Hydrocephalus can occur for many reasons including congenital anomalies (e.g. spina bifida, aqueductal stenosis, Arnold-Chiari or Dandy-Walker malformations), trauma, intraventricular hemorrhage and meningitis. The common pathway is cerebrospinal fluid accumulation causing pressure effects on the central nervous system. While not perfect, ventriculoperitoneal shunts allow many children with hydrocephalus to live symptom free lives and many others to have much improved quality of life. In the pre-shunt era, only 20% of children survived to adulthood and 50% had brain damage.
One major improvement was the development of the DWT pressure valve by Roald Dahl (the famous author), Stanley Wade (engineer particularly of hydraulic pumps) and Kenneth Till (first full-time pediatric neurosurgeon in Great Britain) valve (DWT)

Any medical device can have complications. For shunts these are divided into 3 categories, infective, overdrainage and mechanical.

• Shunt infections occur in 5-10% of surgeries usually within the first 3 months after surgery and more commonly in younger patients. Staphylococcus is the most common organism, and common symptoms include abdominal pain, nuchal rigidity, and swelling, drainage or erythema of the shunt site.
• Overdrainage of the ventricles can cause subdural hematomas and excessive siphoning can actually cause mechanical shunt obstruction.
• Mechanical problems can be caused by disconnection of the parts of the shunt system, breakage of the tubing, and obstructions at either end of the tubing (especially glialependymal tissue in the brain).
  Children with acute mechanical shunt malfunctions can present in several ways including headache, emesis, personality changes, papilledema and cranial nerve palsies. New onset seizures or changes in the patients normal seizure pattern should also raise concerns. Chronic malfunction may present with loss of developmental milestones, abnormally increased head growth, papilledema or optic atrophy and again changes in seizure patterns.

Learning Point
Of the shunts placed about 80% will have a need for revision with 30% of these occurring in the first year. The average child over their childhood will have 2-3 operations for shunt revisions. This would come out to be 1-1.25 shunt revision surgeries per week for a large neurosurgical practice following 500 children. The number of evaluations for possible shunt malfunction because of seizures, headaches etc. to identify a child who needs a shunt revision is even higher.

Questions for Further Discussion
1. What is the role of a general pediatrician in shunt management and evaluation for possible shunt malfunctions?
2. What are the sports and activities limitations recommended for children with shunts?

Related Cases

Disease: Hydrocephalus

Symptom/Presentation: Mental Status Changes | Seizures

Specialty: Developmental Disabilities | Emergency Medicine | Medical History | Neurology / Neurosurgery | Radiology / Nuclear Medicine / Radiation Oncology

Age: Toddler

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Hydrocephalus

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

Prusseit J, Simon M, von der Brelie C, Heep A, Molitor E, Volz S, Simon A. Epidemiology, prevention and management of ventriculoperitoneal shunt infections in children.
Pediatr Neurosurg. 2009;45(5):325-36.

Obituary of Kenneth Till. Guardian Newspaper. August 26, 2008. Available from the Internet at: http://www.guardian.co.uk/society/2008/aug/26/health

Conant, Jennet. The Irregulars. Roald Dahl and the British Spy Ring in Wartime Washington. Simon & Schuster. New York, NY. 2008;343.

Kramer LC, Azarow K, Schlifka BA. Management of Spina Bifida, Hydrocephalus and Shunts. eMedicine. Available from the Internet at http://emedicine.medscape.com/article/937979-overview (rev. 11/19/2009, cited 5/20/2010).

ACGME Competencies Highlighted by Case

Patient Care
1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
2. Essential and accurate information about the patients’ is gathered.
3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
4. Patient management plans are developed and carried out.
7. All medical and invasive procedures considered essential for the area of practice are competently performed.
8. Health care services aimed at preventing health problems or maintaining health are provided.
9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

Medical Knowledge
10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

Practice Based Learning and Improvement
13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

Author

Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa Children’s Hospital

Patient Presentation
A 9-year-old Caucasian female came to clinic for her health supervision visit. Her mother is concerned because she noticed breast budding within the last month and is worried that her daughter “is developing too early.” She nor her daughter have noticed any axillary or pubic hair. They denied any unusual growth spurt or body odor. Her menses have not started, and she has not had any abnormal vaginal discharge. The past medical history and review of systems are negative. The family history shows that her mother and mother’s sister had menarche at age 11. Her father had his growth spurt around age 13-14 but the mother is not sure about the timing.

The pertinent physical exam shows a healthy female with normal vital signs. She is 28 kilograms (25-50%) and 140 cm (75%) and has grown 2.5 kilograms and 6 cm over the past year. Her skin examination shows two 0.5 cm cafe-au-lait spots. She is Tanner stage II for breast, and Tanner I for pubic hair. She has no axillary hair. The rest of her examination is normal. The diagnosis of normal pubertal changes was made. The patient and mother were counseled that this was normal, particularly as the mother and aunt appeared to have a similar pubertal pattern. However, the mother was counseled that if her daughter were to experience rapid changes going through puberty to recontact the office. Patient handouts including an information prescription for appropriate Internet resources for the daughter’s age were given.

Discussion
During normal pubertal development “[h]igh-amplitude pulses of [gonadotropin releasing hormone] cause pulsatile increases in the pituitary gonadotropin-luteinizing hormone (LH) and follicle-stimulating hormone (FSH). Increased LH levels stimulate production of sex steroids by testicular Leydig cells or ovarian granulosa cells. Pubertal levels of androgens or estrogens cause the physical changes of puberty….” These include growth spurt, breast budding, public and axillary hair, and penile and testicular enlargement. Production of ovarian follicular maturation and spermatogenesis also occurs.

Normal female puberty (mean age in years)

• Thelarche (breast budding caused by estrogens) is age 9 years
• Adrenarche (pubic hair caused by androgens) is age 10 years for Caucasian and age 9 years for African American girls.
• Menarche is age 12.8 years for Caucasian and 12.2 for African American girls.

Thelarche may be unilateral or bilateral. Most Caucasian girls begin puberty with thelarche (20%) and most African American girls begin with adrenarche. Average amount of time from thelarche to menarche is 2.5 years.

Definitions of what determines the age of diagnosis for precocious puberty have decreased recently:

• Basically any changes below age 8 should be concerning
• African American = adrenarche or thelarche < 6 years
• Caucasian = adrenarche or thelarche < 7 years
• Caucasian = adrenarche and thelarche < 8 years

Learning Point
Precocious puberty in girls is a problem that can lead to short stature, early sexuality, difficulty with menstrual hygiene and problems with being different from their peers. Children should be referred for precocious puberty evaluation when the diagnosis is made and/or if they have rapidly progressive puberty, predicted short stature compared to mid-parental height, bone age advanced 2 years or more, neurological symptoms or history of unusual symptoms or signs. unusual symptoms or signs.

Causes of precocious puberty include:

• Central (gonadotropins cause early maturation of the entire hypothalamic-pituitary-gonadal axis)
  • Idiopathic (95% of cases)
  • Tumors (primary or metastatic, benign or malignant)
  • Malformations
  • Damage – trauma, encephalitis
• Peripheral (gonadotropins are not the cause)
  • Ovarian cysts
  • Ovarian or adrenal tumors producing hormones
  • Hypothyroidism (severe)
  • McCune-Albright syndrome
  • Toxins – environment, ingestion, topical

Evaluation of precocious puberty usually includes:

• Random LH is considered the best screening test for central precocious puberty as the prepubertal (low) and pubertal (higher) levels are basically distinct from one another and do not overlap.
• Random FSH is not helpful to determine precocious puberty, but it can be useful if a gonadotropin-releasing hormone study is being performed.
• Estrogen levels are not as consistent an indicator of puberty for girls as testosterone is for boys.
• DHEA is the preferred androgen steroid to measure and is usually elevated in girls and boys with precocious puberty.
• Thyroid testing is usually not indicated unless other symptoms are present.
• Pelvic ultrasound is indicated if peripheral precocious puberty is being considered but not for central.
• Head imaging may be considered especially in very young children as the incidence of a tumor is higher.

Treatment for precocious puberty usually consists of lutenizing hormone releasing hormone and gonadotropin releasing hormone agonists.

Questions for Further Discussion
1. What is the normal pubertal progression for boys?
2. At what ages is puberty considered delayed for girls and boys?

Related Cases

Disease: Precocious Puberty | Endocrine Diseases | Growth Disorders

Symptom/Presentation: Growth Problems | Health Maintenance and Disease Prevention | Precocious Puberty

Specialty: Adolescent Medicine | Endocrinology | General Pediatrics

Age: School Ager

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Endocrine Diseases and Growth Disorders.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

Herman-Giddens ME, Slora EJ, Wasserman RC, et al. Secondary sexual characteristics and menses in young girls seen in office practice: a study from the Pediatric Research in Office Settings Network. Pediatrics.1997;99:505-512.

Adams Hillard PJ. Menstruation in young girls: a clinical perspective. Obstet Gynecol. 2002 Apr;99(4):655-62.

Kaplowitz PB, Precocious Puberty. eMedicine.
Available from the Internet at http://emedicine.medscape.com/article/924002-overview (rev. 3/29/2010, cited 5/19/10).

Ryan G. Problems with menarche: When the First Period Comes Early or Late. Carver College of Medicine Pediatric Symposium Presentation. 10/4/08.

ACGME Competencies Highlighted by Case

Patient Care
1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
2. Essential and accurate information about the patients’ is gathered.
3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
4. Patient management plans are developed and carried out.
5. Patients and their families are counseled and educated.
6. Information technology to support patient care decisions and patient education is used.
7. All medical and invasive procedures considered essential for the area of practice are competently performed.
8. Health care services aimed at preventing health problems or maintaining health are provided.

Medical Knowledge
10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

Practice Based Learning and Improvement
13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

Professionalism
20. Respect, compassion, and integrity; a responsiveness to the needs of patients and society that supercedes self-interest; accountability to patients, society, and the profession; and a commitment to excellence and on-going professional development are demonstrated.
21. A commitment to ethical principles pertaining to provision or withholding of clinical care, confidentiality of patient information, informed consent, and business practices are demonstrated.
22. Sensitivity and responsiveness to patients’ culture, age, gender, and disabilities are demonstrated.

Author

Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa Children’s Hospital

Patient Presentation
A 5-year-old male came to clinic with his mother for his health supervision visit. She stated that he was doing well but that the child care provider says that he doesn’t want to do coloring or writing activities very much. Both have noted that he seems to tire quickly in these activities and just kind of lays on the table or squirms in his chair. His mother also says that he seems to turn his hand around while holding a crayon or pencil and doesn’t seem entirely comfortable with it. The past medical history and review of systems are negative. The family history shows that his father needed some speech therapy as a child for dysarticulation.

The pertinent physical exam shows a smiling boy with normal vital signs and growth parameters. His physical examination is normal including his neurological examination. His writing on the chalkboard shows immaturely formed letters and shapes. He is able to form squares, crosses and triangles but they have a shaky appearance. When asked to draw on the board, he holds the chalk in an awkward position, somewhere between a fisted grip and a proper tripod position. When a formal Denver Developmental Screening Test was performed he was normal in all areas.

The diagnosis of a possible writing problem was made and the child was referred to an occupational therapist (OT). The OT found a mild hypotonia in the upper extremities and an abnormal grip, and began intervening with him. She began working with him to increase his strength using PlayDoh®, playing with a peg board and picking small objects with tweezers, tongs and other similar items. The OT and the school personnel were working collaboratively to improve his fine motor skills. At his last appointment he was making good progress on opening up the space between his thumb and fingers to increase the webspace, but he needed an adaptive pencil grip at that time. Therapy was ongoing.

Discussion
Fine motor movements affect almost everything we do on a daily basis such as moving and manipulating tools and objects, preparing and eating meals, personal hygiene, communicating through writing and typing, counting change, opening doors, etc.

Occupational therapists (OTs) are highly skilled professionals who assist children and families in some of the most important activities of life. OTs may work in hospitals, clinics, schools, rehabilitations centers and other locations, and a master’s degree is needed for an entry level position. Pediatric occupational therapists consider the wide range of developmental attainment for children. Depending on the problem, they may also need to consider rehabilitation needs if skills are lost through disease or accident in a child. OTs also work in areas of mental health including substance abuse, eating disorders and depression. OTs often work in interprofessional teams because most problems do not occur in isolation. For example, a child may be having difficulty writing because of general hypotonia or decreased physical stamina, and therefore the child may not be able to get into a proper truncal body posture or keep it there to be able to support the writing. An OT is qualified to treat these problems but may recommend co-treatment with a qualified physical therapist also. It is especially important for the OT to partner with the family and others to adapt/modify fine motor activities for the child while the strengthening and grasp therapy is getting started, i.e. finding ways for the child to be successful in spite of the fine motor problems.

Self care tasks (e.g. clothing management before/after toileting, difficulty with fasteners, cutting foods, etc) can be difficult to assess in a medical clinic setting. Noticing if the child has difficulty taking off or putting on clothing at the visit, or even asks for help for a developmentally appropriate task may indicate a more global fine motor problem. Dyskinetic or awkward movement patterns could be coordination problem or weak grasp, but could also be sensory in nature. An OT can be valuable in assessing these problems.

While there are many checklists that both parents and healthcare providers can use to screen children for developmental problems, it is important to note that it is not just the presence or absence of the skills which may be of concern (i.e. a quantitative measure), but concerns about how the skills are carried out should also be noted (i.e. a qualitative measure). For example, a child with mild hypotonia or decreased stamina slumping over the work or putting their body into different postures such as hooking their legs around the chair legs. In these cases, this may be the child’s way of trying to hold up their body to be able to produce the work. On the checklist, the child would pass, but it is the way in which the child does the task which is of concern. Medical personnel (e.g. doctors, nurses, etc.) tend to have more training in screening to determine IF the skill is performed, and less about HOW it is performed. For this reason, even skilled medical professionals can miss less obvious developmental problems. It is often useful to refer a child for an evaluation by an OT if there are concerns by family members, caretakers or other personnel about how a child performs a task.

Learning Point
Medical personnel should screen children for developmental delays at each health supervison visit.
Normal fine motor skills attainment in the first 5 years of life is by noted below along with areas of concern that should be considered for further evaluation and possible referral to an OT.

• End of 12 Months (1 Year)
  • Uses pincer grasp
  • Bangs two objects together
  • Puts objects into container
  • Takes objects out of container
  • Lets objects go voluntarily
  • Pokes with index finger
    • Developmental Concerns During 0-12 months
      • 3.5 months – persistence of grasp reflex
      • 4-5 months – unable to hold rattle
      • 7 months – unable to hold an object in each hand
      • 10-11 months – absence of pincer grasp
    • Developmental Concerns During 13-24 Months
      • 15 months – unable to put in or take out object, does not seem to know the function of common household objects (brush, telephone, bell, fork, spoon)
      • 20 months – unable to remove socks/gloves by self
• End of 24 Months (2 Years)
  • Scribbles on his or her own (generally with a fisted pencil grip)
  • Turns over container to pour out contents
  • Builds tower of four blocks or more
  • Might use one hand more often than the other
  • Fingers move independently of each other often
    • Developmental Concerns During 25-36 Months
      • 24 months – unable to stack 5 blocks or not scribbling
      • 30 months – not turning single page of book
      • 36 Months – unable to stack 8 blocks, draw straight line, cannot grasp a crayon between thumb and fingers, has difficulty scribbling, cannot stack four blocks

• End of 36 Months (3 Years)
  • Makes up-and-down, side-to-side, and circular lines with pencil or crayon (pronated pencil grip – fingers pointed toward the end of the writing instrument)
  • Snips paper with scissors
  • Turns book pages one at a time
  • Builds a tower of more than six blocks
  • Holds a pencil in writing position
  • Screws and unscrews jar lids, nuts, and bolts
  • Turns rotating handles
    • Developmental Concerns During 37-48 Months
      • 48 Months – unable to stack 6-8 blocks, cannot copy circle
      • During this time period – seems uncomfortable holding crayons
• End of 48 Months (4 Years)
  • Copies square shapes (generally with a tripod pencil grip)
  • Draws a person with two to four body parts
  • Uses scissors
  • Draws circles and squares
  • Will try to color within the lines but with limited success
  • Begins to copy some capital letters
  • Scissoring is more efficient with forward motion and following a simple S curve on the paper
    • Developmental Concerns During 49-60 Months
      • 54 months – unable to copy square
      • During this time period – has trouble taking off clothing, cannot brush teeth efficiently, cannot wash and dry hands
• End of 60 Months (5 Years)
  • Copies triangle and other shapes
  • Colors usually well within the lines
  • Draws person with body
  • Prints some letters
  • Dresses and undresses without help
  • Uses fork, spoon, and (sometimes) a table knife
  • Usually cares for own toilet needs
  • Hand dominance should be fairly well established and totally established by 72 months
  • Can cut out a square

Children who are performing writing-type tasks should have the ankles, knees and hips at 90 degree angles. The forearms should rest on the desk/table top. The height of the desk/table top is 2 inches above the elbows when the elbows are held at the child’s side. There are a variety of pencil grasp patterns (including developmental ones that are perfectly normal – see To Learn More Below), but the emphasis should be on a functional grasp. The tripod grasp with open web-space is a common one where the writing utensil is “… held with the tip of the thumb and index finger and rests against the side of the third finger. The thumb and index finger form a circle.” There are variations of this including the adaptive tripod and quadripod grasp. For children who have difficulties with consistently creating a functional grasp, adaptive pencil grips may be utilized.

Questions for Further Discussion
1. Where are OT services provided locally?
2. What problems do children with writing problems show in elementary school?

Related Cases

Disease: Dysgraphia | Learning Disorders | Muscle Disorders

Symptom/Presentation: Developmental Delay | Hypotonia | Learning Problems

Specialty: Developmental Disabilities | General Pediatrics | Occupational Therapy

Age: School Ager

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Learning Disorders and Muscle Disorders.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

OTility.com. Pencil Grasp Patterns and Pre-Writing Skills.
Available from the Internet at http://www.otility.com/articles/Pencil-Grasp-Patterns.aspx (cited 5/17/10).

Skillbuildersonline.com. Fine Motor Develoment 0-6 Years.
Available from the Internet at http://www.skillbuildersonline.com/SBA/Description.asp?ID=2 (cited 5/19/10).

Occupational Outlook Handbook. Occupational Therapists. Bureau of Labor Statistics. 11th edit. Available from the Internet at http://www.bls.gov/oco/ocos078.htm (rev. 12/17/2009, cited 5/17/10).

Bright Futures. Developmental Milestones Age 2, Ages 3-4 years, Age 4-5 years.
Available from the Internet at http://www.aap.org/healthtopics/stages.cfm (rev. 3/17/10, cited 5/17/10).

ACGME Competencies Highlighted by Case

Patient Care
1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
2. Essential and accurate information about the patients’ is gathered.
3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
4. Patient management plans are developed and carried out.
5. Patients and their families are counseled and educated.
7. All medical and invasive procedures considered essential for the area of practice are competently performed.
8. Health care services aimed at preventing health problems or maintaining health are provided.
9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

Medical Knowledge
10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

Interpersonal and Communication Skills
19. The health professional works effectively with others as a member or leader of a health care team or other professional group.

Author

Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa Children’s Hospital

Larissa Treat, MOT, OTR/L

Patient Presentation
A 15-year-old female came to clinic after she noted a mass in her neck while studying a few days ago. She reported that it was painless and causing no problems breathing or swallowing. She also reported normal energy and sleeping and eating patterns, no changes in weight, hair, palpitations, bleeding or bruising problems, change in voice, coughing, muscle twitchiness or weakness, or other masses. The past medical history was negative. The family history showed an elderly grandmother with hypothyroidism and diabetes. There was no cancer in the family. The review of systems was otherwise negative.

The pertinent physical exam showed a healthy appearing female with a height and weight in the 25-50% that were stable from a previous visit 10 months earlier. Vital signs were normal. HEENT showed a perceptibly but mildly enlarged thyroid on the left. Palpation revealed a diffusely enlarged left hemithyroid without discrete nodules. It moved appropriately with swallowing and no pain was elicited. There was no perceptible enlargement on the right side of the thyroid. A 0.5 cm cervical node at the angle of the mandible was noted and was freely mobile. No other adenopathy was palpable bilaterally.

The laboratory evaluation of a T4 and TSH were normal. The radiologic evaluation of an ultrasound of the thyroid showed 3 nodules on the left, with the largest ones 2 cm and 3 cm in size, plus one small nodule on the right. The patient and family consented to fine needle biopsy at the time of the ultrasound as they had been told by their physician that this was usually the next step in the process. The biopsy showed histology consistent with follicular adenoma, hyperplasia and much less likely follicular carcinoma. After consultation with the primary care provider, the patient was referred to a surgeon who after discussion with the family elected to have a total thyroidectomy performed. The surgery went well and the final pathology report made the diagnosis of follicular cell carcinoma in one nodule that was totally contained within the thyroid, with no local metastasis. The patient did not undergo radioablation per low-risk cancer staging, and has been followed closely for 4 years currently with no signs of recurrence.

Figure 84- Transverse  ultrasound image of the thyroid gland (above) shows the single nodule in the right lobe of the thyroid and the largest nodule in the left lobe of the thyroid. Longitudinal ultrasound image of the left lobe of the thyroid (below) shows the three nodules in the left lobe of the thyroid.

Discussion
Endemic goiter is not a common problem in the U.S. with the addition of iodine to food particularly salt. Thyroid nodules are relatively unusual for children (solitary nodules 0.22-1.35%) compared with adults (4%). Malignancy unfortunately is more common in children than adults; for solitary nodules 15-25% compared to 4% in adults. During adolescence the rate of malignancy decreases to adult levels in late adolescence/early adulthood.

Learning Point
Most single and multiple thyroid nodules are benign but not all and this makes treatment decisions more difficult. Nodules which have obvious malignant changes require surgical treatment and medical followup. It is the nodules which show indeterminant pathology that can be associated with increased malignancy risk which are particularly frustrating for patients and health care providers. Thyroid nodules’ evaluation and treatment has improved with the more consistent availability of ultrasound evaluation and fine needle aspiration biopsy (FNAB) with appropriate pathological consultation. These are particularly helpful for diagnosing benign cystic nodules which may require aspiration but are benign and monitored.

The American Thyroid Association has published an extensive guideline which outlines the recommended evaluation and treatment of thyroid nodules and differentiated thyroid cancer. See To Learn More below. All patients with an enlarged thyroid should have a thyroxine (T4) and thyroid stimulating hormone (TSH) measured (parathyroid hormone is not indicated usually as part of the initial evaluation). This helps to understand the patient’s thyroid state and in planning evaluation and treatment. Patients with hypothyroidism are recommended to have a diagnostic iodine or technetium scan to determine if the nodule is hot or cold. Cold nodules are more consistent with malignancy. Euthyroid or hyperthyroid patients are recommended to be referred for ultrasound and if a nodule is found, then FNAB is performed.

• If the biopsy is non-diagnostic then another FNAB is performed.
• If frankly malignant or suspicious for malignancy, surgery is recommended. Radiofrequency ablation is often used post-thyroidectomy to irradicate residual thyroid tissue left in-situ to spare the recurrent laryngeal nerve, parathyroid glands and other adjacent structures.
• Intermediate pathology (Hurtle cell or follicular neoplasm) is also recommended to have surgical consultation because of the increased risk of malignancy for these usually benign nodules (~20% for Hürtle cell and ~5-10% for follicular neoplasms).
  Follicular neoplasms such as the one above are particularly vexing for pathologists to rule out malignancy because the capsule of the nodule needs to be examined in great detail which is not truly an option without removal of the entire nodule.
  Genetic markers appear to be promising for future determination of indeterminant pathology.

Increased risks for malignancy include:

• Age 70 years
• Male gender
• Prior head and neck radiation
• Prior thyroid cancer
• Family history of thyroid disease (benign or malignant0
• Recurrent laryngeal nerve or other nerve involvement (i.e. dysphonia, dysphagia)
• Hard, firm or immobile thyroid nodule
• Cervical lymphadenopathy

Questions for Further Discussion
1. What is the differential diagnosis of an enlarged thyroid gland?
2. What is the role of an endocrinologist in the evaluation and management of thyroid nodules?
3. How is hypothyroidism managed post-operatively with patients who underwent thyroidectomy?
4. What are the potential complications of thyroidectomy?

Related Cases

Disease: Thyroid Cancer | Thyroid Diseases

Symptom/Presentation: Neck Mass

Specialty: Endocrinology | Oncology | Pathology | Radiology / Nuclear Medicine / Radiation Oncology | Surgery

Age: Teenager

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Thyroid Diseases

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

Niedziela M. Pathogenesis, diagnosis and management of thyroid nodules in children.
Endocr. Relat. Cancer. 2006;13(2):427-453.

Hebra A, Miller M, Thomas PB. Solitary Thyroid Nodule. eMedicine.
Available from the Internet at http://emedicine.medscape.com/article/924550-overview (rev. 11/26/2008, cited 5/14/2010).

Dankle SK. Thyroid Nodule. eMedicine.
Available from the Internet at http://emedicine.medscape.com/article/127491-overview (rev. 3/9/10, cited 5/14/10).

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ACGME Competencies Highlighted by Case

Patient Care
1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
2. Essential and accurate information about the patients’ is gathered.
3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
4. Patient management plans are developed and carried out.
5. Patients and their families are counseled and educated.
7. All medical and invasive procedures considered essential for the area of practice are competently performed.
8. Health care services aimed at preventing health problems or maintaining health are provided.
9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

Medical Knowledge
10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

Interpersonal and Communication Skills
17. A therapeutic and ethically sound relationship with patients is created and sustained.
19. The health professional works effectively with others as a member or leader of a health care team or other professional group.

Systems Based Practice
23. Differing types of medical practice and delivery systems including methods of controlling health care costs and allocating resources are known.
24. Cost-effective health care and resource allocation that does not compromise quality of care is practiced.
25. Quality patient care and assisting patients in dealing with system complexities is advocated.
26. Partnering with health care managers and health care providers to assess, coordinate, and improve health care and how these activities can affect system performance are known.

Author

Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa Children’s Hospital