Patient Presentation
A 9-month-old male came to clinic with a 2 day history of intermittent bleeding from one of his hemangiomas on his left arm. The bleeding was mainly of serous fluid with a small amount of blood. It was easily controlled with pressure and a bandage changed 2-3 times per day contained the fluid. The patient did not appear in pain and did not have redness around the lesion or fever. The patient was otherwise well. His mother was concerned because she had been told that if bleeding kept happening then he might need some treatment. The past medical history showed a healthy male infant who had his first hemangioma on his left lateral back appear around 6 weeks of age. Three others than appeared on his left arm, left flank and right anterior chest. All had increased in size but had been stable per his mother for a couple of months.

The pertinent physical exam showed a smiling infant with growth parameters in the 10-50%. His cardiac and abdominal examinations were negative. His skin examination showed one 0.5 cm cafe-au-lait spot on his right posterior calf. The left lateral back had a 5 mm x 2 mm raised red lesion that was circular. The left flank had a 10 mm x 3 mm raised red lesion that was circular. The right anterior chest had a 12 mm x 3 mm raised red lesion that was mainly oval but slightly more irregular. All had distinct borders and consistent coloration. The left arm was 22 mm x 5 mm raised red lesion that was circular. About 10 o’clock to the center of the lesion was a “crack” with a minimal amount of serous fluid. A thin wet scab was present. No pain or tenderness was elicited and there was no red streaking from the lesion. There was full range of motion in the arm. Documentation from previous visits showed the lesions to be about the same size previously. The diagnosis of a probably traumatized hemangioma was made. The mother was told to continue to monitor it and try to minimize repeated trauma if possible. She was also told how to control bleeding if necessary. The physician reiterated the natural history of the lesions and didn’t believe further treatment was necessary at this time unless the bleeding got worse or would not resolve.

Discussion
Infantile hemangiomas (IH) are the most common soft tissue tumors in infants. They are usually considered birthmarks but are dynamic lesions. They usually begin in the first few weeks of life and rapidly grow in the first 3-5 months of life. By 5 months, most lesions will have achieved 80% of their final size. Almost all IH have cessation of growth after 9 month of age. Images of IH can be seen in the To Learn More section below.

Learning Point
Often no treatment is necessary for IH other than expectant monitoring. Additional treatment may be necessary depending on the patient’s age, lesion type, location, size and complication being considered.

The results of a prospective cohort of 1058 children in 7 pediatric dermatology clinics found that overall 24% of patients had complications and 38% needed treatment. Hemangiomas are more likely to have complications and receive treatment if they are:

• Type: segmental (55.5%) or intermediate (24.9%); localized (9.6%) or multifocal (9.1%) were less likely
• Location: perineum (47.9%), face (43.0%) and head and neck (31.1%). Those on the extremity (20.6%) and the trunk (11.5%) are less likely
• Size: Large are more likely than small

Hemangioma complications include:

• Ulceration – the most common complication. A white discoloration on the lesion may be involution or ulceration. Ulceration is painful and usually heals with scaring.
• Bleeding – significant hemorrahage is rare
• Cardiac failure
• Infection – cellulitis, abscess
• Obstruction of vital organs with associated morbidity- eye, airway
• Element of syndrome or other process
  • Hypothyroidism
  • Diffuse neonatal hemangiomatosis
  • Kasabach-Merritt phenomenon
  • PHACE syndrome
  • PELVIS or SACRAL syndrome
  • Occult spinal dysraphism
• Scaring/disfigurement
• Psychosocial problems for patient and family

Treatment includes antibiotics, dressings, pulsed-dye laser or other type of laser surgery, surgical excision, propanolol, corticosteroids, and recombinant growth factors.
Consultations with dermatology, otolaryngology, ophthalmology, and plastic surgery may be necessary.

Questions for Further Discussion
1. What birthmarks are potential signs of an underlying medical problem?
2. What treatment options can be considered for recurrent bleeding in infantile hemangioma?

Related Cases

Disease: Infantile Hemangioma | Benign Tumors | Birthmarks

Symptom/Presentation:Bleeding and Bruising | Mass or Swelling

Specialty: Dermatology

Age: Infant

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: Benign Tumor and Birthmark.

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

To view images related to this topic check Google Images.

Haggstrom AN, Drolet BA, Baselga E, Chamlin SL, Garzon MC, Horii KA, Lucky AW, Mancini AJ, Metry DW, Newell B, Nopper AJ, Frieden IJ. Prospective study of infantile hemangiomas: clinical characteristics predicting complications and treatment. Pediatrics. 2006 Sep;118(3):882-7.

Chang LC, Haggstrom AN, Drolet BA, Baselga E, Chamlin SL, Garzon MC, Horii KA, Lucky AW, Mancini AJ, Metry DW, Nopper AJ, Frieden IJ; Hemangioma Investigator Group. Growth characteristics of infantile hemangiomas: implications for management. Pediatrics. 2008 Aug;122(2):360-7.

Antaya RJ, Dirk M, Elston DM. Infantile Hemangioma. Medscape.
Available from the Internet at http://emedicine.medscape.com/article/1083849-treatment (rev. 7/27/2011, cited 10/24/11).

ACGME Competencies Highlighted by Case

Thank you for being loyal Pediatric Education users. We’re taking a short break and will be back January 9th with our next case. Our best wishes to you in the New Year. Donna D’Alessandro and Michael D’Alessandro, Curators.

Patient Presentation
A 14-year-old caucasian male came to clinic because he had noticed his tongue becoming darker over the previous week. He was very concerned about the coloring as he said it would not go away. He was studying for school examinations and had been self-medicating an upset stomach with Pepto-Bismol®. About 1 month ago he was diagnosed with bronchitis at an urgent care center and had taken antibiotics. The past medical history was negative except for being overweight. The review of systems was negative. The pertinent physical exam showed a well-appearing male whose BMI was 28.9 with normal vital signs. HEENT showed poor oral hygiene with obvious caries. He had dark brown coloring of the top of the tongue anteriorly, not involving the sides, that was uniform. Scraping appeared to decrease the discoloration but it didn’t completely go away. There was no elevation of the tongue nor oral masses visible or palpable. There were some shoddy anterior cervical nodes. His neck had a normal thyroid examination and no masses. Skin examination showed acne on his face, and a few brown macules and freckles scattered on his trunk, arms and face that he reported to be unchanged. He had no discoloration of the palms or soles. The diagnosis of poor oral hygiene along with taking bismuth was made. He was counseled about the bismuth use and school stress. He was also counseled about oral hygiene including scraping of his tongue. Followup at 1 month showed that the discoloration had resolved and his stress after examinations.

Discussion
Although dental caries, strep throat and oral candidiasis are some of the most common oral pathology. The tongue itself can be a source of potential pathology. Geographic tongue, oral candidiasis, and lingual ulcerations are common problems. Most discolorations of the tongue are because of food, drink or medications that are ingested and are self-limited. However discoloration can be a sign of more significant problems.
Hairy tongue, often colored black, is a relatively uncommon problem in the US but has a higher incidence reported in Turkey and Iran. It is more common in adults than children but has been reported in a child 2 months of age. It is also called lingua villosa nigra and is a benign condition caused by keratin accumulation usually in the setting of poor oral hygiene and/or xerostomia. The accumulation is on the filliform papillae and will be seen on the dorsal surface anterior to the circumvallate papillae and not on the lateral sides of the tongue or tip. The discoloration can be different colors depending on the oral flora. Hairy tongue usually responds to oral hygiene including scraping of the tongue, but sometimes retinoids, keratolytic agents and other treatments are used.

Learning Point
The differential diagnosis of a black tongue includes:

• Normal variation
• Poor oral hygiene
• Acanthosis nigracans
• Adrenal insufficiency
• Congenital lingual melanotic macules
• Congenital melanocytic naevi

Patient Presentation
A medical student had seen a 6-month-old, former 32 week premature female in the clinic for a health supervision visit. The past medical history showed the infant had some mild respiratory distress at birth that was treated with nasal canula oxygen for 48 hours. She had done very well and was discharged at 32 days of life after she had learned to feed well orally. The pertinent physical exam revealed a smiling infant with growth parameters below the 5th percentile for chronological age but at the 5-10% when corrected for prematurity with a curve showing good catchup growth. The rest of her examination was normal including her development which found her to roll over from front to back, bring her hands to midline and starting to transfer objects, and she was cooing with various vowel sounds.
The diagnosis of a healthy former premature infant was made, and she was given routine health maintenance information. She had an appointment with the neonatal follow-up program in about a month to monitor her weight and development.

The medical student had several questions about premature infants, but in particular had noted the infant’s initial Apgar score was 6 and a 5-minute Apgar score of 8. He thought these were low and also asked how good the Apgar scores were for predicting how well the infant would do. The attending physician gave him a brief history of the Apgar score and emphasized that it was a measure of the status of the infant at that time only and wasn’t good for predicting outcomes. The attending said, “It really gives everyone an idea of how well the infant is transitioning from fetus to neonate at that point in time. The change in the score is even more important if the infant wasn’t doing very well to begin with and then we can see how the infant is responding to our resuscitation efforts. You always want to see the scores go up if they are initially low and you want them to remain high if they were high to begin with. Low scores aren’t good but they aren’t predictive by themselves. I suppose there are sensitivities and specificities for using the scores with certain groups of infants but I don’t think there is standard specificity and sensitivity overall as neonates are such a diverse group of patients.”

Discussion
Dr. Virginia Apgar was the first woman at Columbia University College of Physicians and Surgeons to hold a full professorship. She was also the Chairman of the Department of Anesthesia and was interested in obstetrical anesthesia and newborn resuscitation. Although it is unclear how she developed the “Apgar score,” a peer of hers says she began to be upset at the lack of resuscitation and treatment efforts for “…apneic, small for age or malformed newborns…[She]began to resuscitate these infants and to develop a scoring system that would ensure observation and documentation of the true condition of each newborn during the first minute of life.” The first minute was used because clinical depression is often maximal at this time. Pictures and a fuller biography of Dr. Apgar can be found on the Changing Face of Medicine website from the National Library of Medicine.

The scoring system gives 0-2 points for 5 different signs. The scoring system using the mneumonic “APGAR” is

Sign						0			1					2
Appearance - Color			Blue or pale	Acrocyanosis			Completely pink
Pulse - Heart Rate			Absent		< 100/minute			> 100/minute
Grimace - Reflex irritability	 No response	Grimace				Cry or active withdrawal
Activity - Muscle tone		Limp	 		Some Flexion			Active motion
Respiration				Absent		Weak cry or hypovention	Good cry

Basic interpretation of the scores is

0-4 = Severely depressed infants
5-7 = Mildly depressed infants
8-10 = Vigorous infants

Learning Point
The Apgar scoring system is very good because it is easy to learn, to apply, can be standardized and requires no special equipment. It focuses attention on the infant’s condition immediately after birth and can be a method to do ongoing assessment of the efficacy of the resuscitation efforts. There are problems with the scoring system though. Color, reflexes and muscle tone are subjective signs. Low birth weight and prematurity often have low scores. Congenital anomalies, hypoxia, hypovolemia, trauma and maternal drugs can also affect the score, as well as resuscitation efforts.

Initially the scoring system was used at 1 minute of life but was expanded to be used at 5 minutes, when it was shown to be correlated with neonatal mortality. A 5 minute score and particularly the change “…in the score between 1 and 5 minutes, is a useful index of the response to resuscitation.”

The scoring system was unfortunately abused. While low Apgar scores at longer time frames (ie. 5, 10, 15, and 20 minutes) after delivery indicate continued problems with the infant, they cannot by themselves indicate outcomes. More recent studies have found that low 5 minute Apgar scores (0-3 range) still correlate with neonatal mortality, but they do not correlate with neonatal morbidity with poor correlation with neurological outcomes in the future.

The American Academy of Pediatrics and the American College of Obstetrics and Gynecology in 1996 developed guidelines for determining hypoxic-ischemic encephalopathy.
All of the following must be present for the definition of asphyxia that is severe enough to result in neurological injury.

• “Profound metabolic or mixed acidemia (pH < 7) in an umbilical artery blood sample, if obtained
• Persistence of an Apgar score of 0-3 for longer than 5 minutes
• Neonatal neurologic sequelae (eg, seizures, coma, hypotonia)
• Multiple organ involvement (eg, kidney, lungs, liver, heart, intestines)”

The Apgar score continues to be an excellent scoring system for initial and ongoing assessment of the newborn in the very early perinatal period when properly used. The American Academy of Pediatrics recommends using an expanded scoring form which includes the 5 Apgar signs but with correlated documentation of the resuscitation efforts including amount of oxygen used, oxygen delivery method used (ie positive-pressure ventilation or nasal continuous positive airway pressure, intubation) chest compressions and epinephrine. A copy of the scoring form can be found in the To Learn More section below.

Questions for Further Discussion
1. What other pediatric subspecialties were started or influenced by non-pediatricians?
2. At what gestational age and/or weight is functional viability for preterm infants at your institution?

Related Cases

Disease: Apgar Score | Newborn Screening

Symptom/Presentation: Health Maintenance and Disease Prevention

Specialty: General Pediatrics | Neonatology | Medical History

Age: Premature Newborn

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: Newborn Screening

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

To view images related to this topic check Google Images.

Apgar V. A proposal for a new method of evaluation of the newborn infant. Anesth Analg 1953;32:260-7.

Committee on Fetus and Newborn, American Academy of Pediatrics and Committee on Obstetric Practice, American College of Obstetricians and Gynecologists. Use and Abuse of the Apgar Score. Pediatrics. 1996;98;141-142.

Changing the Face of Medicine Exhibition. National Library of Medicine. Virginia Apgar. Available from the Internet at http://www.nlm.nih.gov/changingthefaceofmedicine/physicians/biography_12.html (exhibition closed 11/19/2005, cited 10/13/11).

Finster M, Wood M. The Apgar score has survived the test of time. Anesthesiology. 2005 Apr;102(4):855-7.

American Academy of Pediatrics, Committee on Fetus and Newborn; American College of Obstetricians and Gynecologists, Committee on Obstetric Practice. The Apgar Score. Pediatrics. 2006;117:1444-1447.

Zanelli SA, Rosenkranz T. Hypoxic-Ischemic Encephalopathy. Medscape. Available from the Internet at http://emedicine.medscape.com/article/973501-clinical (rev. 8/17/2011, cited 10/13/11).

ACGME Competencies Highlighted by Case