What Causes Kyphosis?

Patient Presentation
A 10-year-old male came to clinic as his aunt had noticed that he seemed to have a more prominent spine around his shoulders when he was swimming and mentioned it to his mother. He and his mother denied any mobility problems and neurological issues. His mother had noticed it before, but he “looks just like his father’s family,” she said. She denied any significant progression of it. The family history was positive for a grandfather with prominent kyphosis but without any functional problems. The review of systems was negative for any systemic problems.

The pertinent physical exam showed normal vital signs including blood pressure. He was obese with a muscular build. His physical examination was negative including no significant skin changes. He was pre-pubertal. His spine was straight in all positions including with flexion. He had full range of motion without any pain, numbness or tingling. There was a more noticeable rounded kyphosis in the thoracic area that did not appear to change much with position. He had well-developed trapezius muscles that appeared to accentuate the kyphosis on inspection. There was no specific mass or increased tissue in the area noted. Neurological examination was normal.

The diagnosis of kyphosis was made. The patient was referred to orthopaedics who felt this was a developmental kyphosis but was not significant enough at this time to warrant additional treatment. Monitoring at intervals was planned.

Discussion
There are 3 planes to view and describe spinal deformities from:

  • Coronal or frontal plane – the view is from front or back of the patient
  • Sagittal plane – is the view from the side of the patient
  • Transverse or horizontal plane – is the view from the top or bottom of the patient

Normally the spine is straight when viewed from the frontal or coronal plane. Normally the spine also has a small kyphosis in the thoracic region (20-45%) and lordosis in the sacral region when viewed laterally in the sagittal plane.

Scoliosis or abnormal deviation of the spinal laterally is the most common spinal deformity. It is best viewed from the frontal or coronal plane.
A review can be found here

Kyphosis is the second most common spinal deformity. It is an abnormal deviation of the spine posteriorly and is best viewed from the sagittal plane. Kyphosis is located in the thoracic or thoracolumbar spinal area and defined as having more than a 40 degree Cobb angle deviation as determined on radiographs. In a study of normally development children (ages 5-16 years) serial measurements were made and found that kyphosis increased for both males and females as they aged. Kyphosis usually does not cause significant functional issues, but is associated with a modest risk of back pain and may have an increased risk for negative body image.

There are two major kyphosis groupings: round and angular. Round is a smooth, large radius curve to the back that usually involves a large number of vertebrae. It is usually more stable, flexible and reducible in childhood, but can become rigid. Angular kyphosis has a sharp angle to the curve as fewer vertebrae are involved. It can be stable but is more likely to be unstable than round kyphosis.

Evaluation includes inspection and palpation of the spine in all angles and Adam’s forward bend test should also be performed. Flexibility, changing curvature, pain and provocation of neurological symptoms should be noted. Patients are often evaluated with radiographs as it can be more difficult to ascertain the extent of kyphosis.
Treatment is usually monitoring and physical therapy and bracing, plus potentially surgical correction.

Learning Point
Causes of kyphosis include:

  • Postural – most common, this improves with patient in proper posture, usually round
  • Developmental kyphosis <ul
  • Scheuermann’s disease is the most common form affecting 4-8% of patients especially boys. Usually round. Usually presents in late childhood with wedged vertebral bodies and cause is unknown but possibly genetic.
  • Congenital malformations usually due to vertebral segmentation and formation problem, often angular
  • Vertebral dysplasia – round or angular
    • Bone fragility problems such as osteogenesis imperfecta, osteoporosis, metabolic diseases, degenerative
    • Soft tissue diseases – Ehler-Danlos syndrome
    • Bone dystrophy
  • Iatrogenic including radiation
  • Infection – tuberculosis is the classic example
  • Paralysis or muscular problems
  • Trauma – often angular
  • TumorQuestions for Further Discussion
    1. How do you perform the Adams Forward Bend Test? A review can be found here
    2. What causes lordosis?
    3. How common is idiopathic scoliosis?

    Related Cases

    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 and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for this topic: Spine Injuries and Disorders

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

    To view images related to this topic check Google Images.

    To view videos related to this topic check YouTube Videos.

    Campos MA, Weinstein SL. Pediatric scoliosis and kyphosis. Neurosurg Clin N Am. 2007;18(3):515-529. doi:10.1016/j.nec.2007.04.007

    Oskouian RJ, Sansur CA, Shaffrey CI. Congenital abnormalities of the thoracic and lumbar spine. Neurosurg Clin N Am. 2007;18(3):479-498. doi:10.1016/j.nec.2007.04.004

    Miladi L. Round and angular kyphosis in paediatric patients. Orthop Traumatol Surg Res. 2013;99(1 Suppl):S140-149. doi:10.1016/j.otsr.2012.12.004

    Gardner A, Berryman F, Pynsent P. The Development of Kyphosis and Lordosis in the Growing Spine. Spine (Phila Pa 1976). 2018;43(19):E1109-E1115. doi:10.1097/BRS.0000000000002654

    Sheehan DD, Grayhack J. Pediatric Scoliosis and Kyphosis: An Overview of Diagnosis, Management, and Surgical Treatment. Pediatr Ann. 2017;46(12):e472-e480. doi:10.3928/19382359-20171113-01

    Author
    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa

  • What Are Causes of Childhood Interstital Lung Disease?

    Patient Presentation
    An 8-year-old male came to clinic for a rash on his arms. He had been playing outside the day before in a field and this morning had red, papular lesions on his forearms and lower legs that were pruritic. His mother had used some hydrocortisone but was concerned. “You know he has dermatomyositis but this rash is different than his other ones,” his mother noted.

    The past medical history showed he was diagnosed at age 6, with muscle weakness and rash and he was monitored by the rheumatology service. There had also been a concern for possible interstitial lung disease and he was being monitored by the pulmonary service. The review of systems was negative for any other exposures, except he had been wearing sunscreen and insect repellent that he had previously used without a problem. He had no fevers or other signs of infections.

    The pertinent physical exam revealed a happy child who was itching. Vital signs were normal but he had a slightly elevated respiratory rate at 32/minute but was not in any respiratory distress. His forearms and legs in exposed areas had a non-specific macular-papular rash that had discrete and confluent areas. HEENT was negative and so was his lung examination.

    The diagnosis of a non-specific rash was made. The pediatrician recommended to monitor the patient and use a low-potency hydrocortisone cream and an antihistamine to help with the pruritis. As the family was very compliant with specialty treatment and followup, the pediatrician just briefly reviewed his overall health plan.

    Discussion
    Interstitial lung disease in children (chILD) is less understood than adult interstitial lung disease (ILD) and is rare. Prevalence is ~ 0.13 to 16.2 per 100,000 children under age 17 years. chILD is an general term for respiratory disorders that are heterogeneous, chronic and impair lung function. While some define diffuse parenchymal lung disease (DPLD) separately, the term ILD usually encompases DPLD. chILD has variable definitions and is “usually diagnosed if three of the following features are present:

      1) respiratory symptoms (cough, rapid and/or difficult breathing, and exercise intolerance),
      2) respiratory signs (tachypnea, adventitious sounds, retractions, digital clubbing, and failure to thrive or respiratory failure),
      3) hyposemia, and
      4) diffuse abnormalities on chest radiograph or computer tomography (CT) scan.”

    Different diagnostic tests can be used including pulmonary function testing, bronchoalveolar lavage, genetic testing and lung biopsy. For radiologic imaging the same is true including plain radiography, ultrasound, and magnetic resonance imaging but CT is considered the standard.

    The natural history varies depending on the etiology and some some etiologies hasten mortality. The main concerns are diseases leading to pulmonary fibrosis. The causes are listed below but it appears that progression is likely due to repetitive injuries in vulnerable lung cells. Genetic and epigenetic factors are known to play a part, but also injuries from infection and inflammation can also contribute.

    As the etiologies are broad, the treatments may or may not be specific. Generally providing oxygen therapy, nutritional support, prevention of infectious diseases (primarily influenza and COVID), treatment of infectious illnesses, and avoiding particulates and irritants such as tobacco smoke and air pollution are first-line treatments. Steroid medications can decrease inflammation as can macrolide antibiotics. Lung transplantation is also an options for some patients.

    Learning Point
    Excluded are other more common diseases such as bronchopulmonary dysfunction, cystic fibrosis, recurrent aspiration and infection, before considering these causes of chILD below.

    • Infancy-specific ILD
      • Neuroendocrine cell hyperplasia of infancy
      • Pulmonary interstital glycogenosis
      • Diffuse developmental disorders
      • Acinar dysplasia
      • Congenital alveolar dysplasia
      • Filamin A disorder
    • Environmental exposure
      • Hypersensitivity pneumonitis
        • Bronchiolitis obliterans, post-infectious
        • Bird fancier’s disease
        • Chemical lung disease – paints, fumes
        • Humidifier lung disease – due to aerosolization of various infectious diseases
      • Medication including chemotherapy
      • Radiation
  • Idiopathic
  • Primary lung dysfunction
    • Altered lung development
      • Plmonary dyplasia or hypoplasia
    • Diffuse alveolar hemorrhage
    • Eosinophilic lung disease
    • Lymphatic disorders
    • Surfactant disorders
    • Viral infections
  • Systemic problems
    • Autoinflammatory disease
    • Connective tissue disease
      • Ankylosing spondylitis
      • Dermatomyositis
      • Juvenile idiopathic arthritis
      • Mixed connective tissue disease
      • Systemic lupus erythematosus
      • Sjorgren syndrome
    • Granuloma
      • Sarcoidosis
    • Metabolic
      • Gaucher’s disease
      • Hermansky-Pudlak disease
      • Niemann-Pick disease
  • Neurocutaneous disorders
  • Organ transplantation
  • Vasculitis
    • ANCA-associated vasculitis
    • Anti-glomerular basement membrane diseases
    • Diffuse alveolar hemorrhage
    • Cryoglobulinimia
    • Henoch-Schonlein purpura

    Questions for Further Discussion
    1. How do cystic fibrosis and bronchopulmonary dysplasia present?
    2. How does dermatomyositis present?
    3. What causes respiratory distress? A review can be found here

    Related Cases

    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 and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for these topics: Interstitial Lung Disease and Myositis.

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

    To view images related to this topic check Google Images.

    To view videos related to this topic check YouTube Videos.

    Cunningham S, Jaffe A, Young LR. Children’s interstitial and diffuse lung disease. Lancet Child Adolesc Health. 2019;3(8):568-577. doi:10.1016/S2352-4642(19)30117-8

    Deterding RR, DeBoer EM, Cidon MJ, et al. Approaching Clinical Trials in Childhood Interstitial Lung Disease and Pediatric Pulmonary Fibrosis. Am J Respir Crit Care Med. 2019;200(10):1219-1227. doi:10.1164/rccm.201903-0544CI

    Griese M. Chronic interstitial lung disease in children. European Respiratory Review. 2018;27(147). doi:10.1183/16000617.0100-2017

    Nathan N, Berdah L, Delestrain C, Sileo C, Clement A. Interstitial lung diseases in children. La Presse Medicale. 2020;49(2):103909. doi:10.1016/j.lpm.2019.06.007

    Semple TR, Ashworth MT, Owens CM. Interstitial Lung Disease in Children Made Easier…Well, Almost. Radiographics. 2017;37(6):1679-1703. doi:10.1148/rg.2017170006

    Author
    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa

  • What is the Buffalo Treadmill Test?

    Patient Presentation
    A 12-year-old male came to clinic after falling off a sled the day prior. He wasn’t sure how he had landed but says there was no loss of consciousness or specific injuries and he continued sledding but soon became tired. He developed a headache on the way home from the sledding hill which continued to be present. The headache was dull to throbbing and described as annoying to hurting. Tylenol and rest improved his symptoms. He and his mother reported that he seemed “slow” today without a lot of energy. Sounds and lights seemed to annoy him. He denied any nausea/emesis, vertigo, difficulty walking or speaking. He stayed home from school and was resting more but wasn’t specifically sleepy. Trying to do homework or using computers made things worse. The past medical history revealed no specific head injuries and was otherwise non-contributory.

    The pertinent physical exam showed a well appearing male with normal vital signs. His physical examination was normal except for some scratches on his forehead.

    The diagnosis of concussion was made. His SCAT (sports concussion assessment tool) had 9 symptoms that were in the mild to moderate range. Cognitive and physical rest was recommended with some reasonable activities of daily living. He was to increase activity once the symptoms went away and followup the next week.

    The patient’s clinical course the following week showed he was worse with increased severity of symptoms and endorsing some dizziness intermittently. He had tried to do some light activity but his symptoms were exacerbated. The following week he started to feel somewhat better again and the family decided to try letting him go to school but his headache and fatigue returned after about 3 hours. The pediatrician who saw him at the point of 3 weeks after the initial injury decided to send him to her partner who was also trained in sports medicine. She said that that boy had remarkably improved over the ensuing week. “By the time I saw him, most of his symptoms were gone and he had increased his activity. Funny thing was that he told me he kept walking his dog because that was when he felt better. He’s going to go back to school this week and I’ll see him again next week,” she told the pediatrician. The pediatrician and her partner briefly started to discuss his activity, but the partner was called away to see a patient. As she walked away she said, “We can talk more later but do you know about the Buffalo Treadmill Test?”

    Discussion
    Head injury and concussion diagnosis and treatment continues to evolve. The current recommendations are evolving from more strict rest and wait to more limited activity and exercise. Interventions have shown data supporting reasonable exercise as not more harmful and potentially beneficial.

    Learning Point
    The Buffalo Concussion Treadmill Test (BCTT) evaluated 13-18 year old athletes (N=103) using a randomized controlled trial of early subthreshold aerobic exercise for sports-related concussion. The control group performed a stretching program for the same amount of time and were given the same general care instructions. The intervention group performed aerobic exercise on a treadmill or stationary bike wearing a heart rate monitor. Baseline heart rate when symptoms were exacerbated was determined at the initial evaluation. Patients were to perform daily exercise that was 80% of this symptom threshold. Exercise was to be for 20 minutes or to stop if symptoms worsened by 2 points on a visual analog scale. Patients in the intervention group recovered (i.e. were asymptomatic) at a mean of 13 days versus 17 days for controls. Other studies with the same participants showed that males reached symptom exacerbation threshold at a slightly lower heart rate than females but “…the BCTT provides comparable information and both sexes reach symptom exacerbation at similar (changes in heart rate).” They have also found similar outcomes for use of a treadmill versus a stationary bicycle. A stationary bicycle may be helpful for patients with limited mobility.

    While heart rate monitoring and supervised aerobic exercise is not practical for the majority of children with concussions, the current recommendation trend seems to be moving toward more focus on having children continue some activity but to slowly increase the intensity and duration of those activities but keeping them below the amount of activity which exacerbates symptoms.

    Questions for Further Discussion
    1. Describe the Glasgow Coma Scale?
    2. What items are on the SCAT or sports concussion assessment tool?

    Related Cases

    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 and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for these topics: Concussion and Traumatic Brain Injury.

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

    To view images related to this topic check Google Images.

    To view videos related to this topic check YouTube Videos.

    Maerlender A, Rieman W, Lichtenstein J, Condiracci C. Programmed Physical Exertion in Recovery From Sports-Related Concussion: A Randomized Pilot Study. Dev Neuropsychol. 2015;40(5):273-278. doi:10.1080/87565641.2015.1067706

    Grool AM, Aglipay M, Momoli F, et al. Association Between Early Participation in Physical Activity Following Acute Concussion and Persistent Postconcussive Symptoms in Children and Adolescents. JAMA. 2016;316(23):2504-2514. doi:10.1001/jama.2016.17396

    Halstead ME, Walter KD, Moffatt K, Council on Sports Medicine and Fitness. Sport-Related Concussion in Children and Adolescents. Pediatrics. 2018;142(6):e20183074. doi:10.1542/peds.2018-3074.

    Leddy JJ, Haider MN, Ellis MJ, et al. Early Subthreshold Aerobic Exercise for Sport-Related Concussion: A Randomized Clinical Trial. JAMA Pediatr. 2019;173(4):319-325. doi:10.1001/jamapediatrics.2018.4397

    Haider MN, Johnson SL, Mannix R, et al. The Buffalo Concussion Bike Test for Concussion Assessment in Adolescents. Sports Health. 2019;11(6):492-497. doi:10.1177/1941738119870189

    Chizuk HM, Willer BS, Horn EC, Haider MN, Leddy JJ. Sex differences in the Buffalo Concussion Treadmill Test in adolescents with acute sport-related concussion. Journal of Science and Medicine in Sport. 2021;24(9):876-880. doi:10.1016/j.jsams.2021.04.005

    Author
    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa

    How Are Port-Wine Stains Treated?

    Patient Presentation
    A 2-week-old female came to clinic for her health supervision visit. She had been noted to have some congenital dermal melanocytosis lesions over her lower back, and also had a different lesion on her right upper extremity. Neither had been bothering the newborn and she had been breastfeeding well.

    The pertinent physical exam showed an interactive female who was only 3% decreased from her birth weight. Her growth parameters were in the 10-50%. Her physical examination was normal except for some slate-grey, blush macules on her lower spine and hips. She also had a flat lesion approximately 3 cm wide and linear down the back of her right arm that skipped the elbow. It was relatively discrete but somewhat streaky in appearance and was a dark red color. She had no other obvious dermal lesions.

    The diagnosis of a healthy female with congenital dermal melanocytosis and a presumed port-wine stain was made. The infant had already been referred to dermatology for lesion confirmation and potential treatment and her parents were happy with that plan.

    Discussion
    Port-wine stains (PWS) or birthmarks are capillary and post-capillary venule malformations which are usually congenital but can be acquired. In newborns they occur in 0.3-0.5% of births. They are pink to dark-red to purple in color, usually flat and solid across the tissue. They are persistent lesions and can develop to have hypertrophy and nodular characteristics. They can occur in any location but tend to involve the head and neck. Isolated PWS are associated with GNAQ, GNA11 and PiK3CA hot spot genetic mutations. PWS are also associated with some syndromes such as Sturge-Weber, Klippel-Trenaunay, other phakomatosis and some overgrowth syndromes. Patients with lesions that are higher risk or have other stigmata for a potential syndrome will need intradisciplinary evaluation and treatment.

    Over time, many lesions in the limb and trunk distribution are stable or lighten and facial lesions may become darker, and can develop to have hypertrophy and nodular characteristics. Long-term patients can experience cosmetic and functional impairments and also intralesional pyogenic granulomas, and squamous or basal cell carcinomas.

    Learning Point
    Selective photothermolysis is the theory that underlies the treatment of vascular lesions using lasers. Light absorbing targets (chromophores) are heated and damaged with surrounding tissues having minimal injuries. The targets are oxyhemoglobin, deoxyhemoglobin and methemoglobin. Laser treatments carefully choose and weigh the wavelength to try to match the target, the pulse duration to the target vessels, and the fluence or energy emitted which is converted to heat to damage the vessels. Other factors that influence treatment planning and outcomes include cooling strategies, skin thickness (infants are 40-50% thinner than adults), melanin, hair follicles, lesion thickness, lesion location and extent, treatment intervals, and patient age.

    Some PWS are monitored over time. Pulsed dye laser (585-595 nm wavelength) is the gold standard for PWS active treatment since the 1980s. It can be safe and effective. Treatment discomfort is often described as “snapping a rubber band against the skin” which may or may not be tolerated by infants and children. Post-treatment often there is swelling which is treated with ice. Other problems can be irritation or even mild blistering or crusting which is usually managed with bland moisturizers. Sunscreen is always recommended. Other problems after treatment include potential scarring, hyperplasia or depressions, and pigmentary changes. Pulsed dye laser penetrates about 1 mm, but PWS may be 3-5 mm which may be why some can be resistant to pulsed dye laser treatment.

    Questions for Further Discussion
    1. What other common vascular stains or abnormalities can you name? A review can be found here
    2. What other types of physical examination signs of Sturge-Weber or Klippel-Trenaunay syndromes?

    Related Cases

    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 and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for these topics: Birthmarks and Plastic and Cosmetic Surgery.

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

    To view images related to this topic check Google Images.

    To view videos related to this topic check YouTube Videos.

    Zallmann M, Leventer RJ, Mackay MT, Ditchfield M, Bekhor PS, Su JC. Screening for Sturge-Weber syndrome: A state-of-the-art review. Pediatric Dermatology. 2018;35(1):30-42. doi:10.1111/pde.13304

    Tran JM, Kelly KM, Drolet BA, Krakowski AC, Arkin LM. Light-based treatment of pediatric port-wine birthmarks. Pediatric Dermatology. 2021;38(2):351-358. doi:10.1111/pde.14503

    Buch J, Karagaiah P, Raviprakash P, et al. Noninvasive diagnostic techniques of port wine stain. Journal of Cosmetic Dermatology. 2021;20(7):2006-2014. doi:10.1111/jocd.14087

    Author
    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa