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Multiple Cutaneous Hemangiomas and Coarctation of the Aorta With Right Aortic Arch

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EXPERIENCE

AND

REASON-Briefly

Recorded

‘In Medicine one must pay attention not to plausible theorizing but to experience and reason together. . . . I agree that theorizing is to be approved, provided that it is based on facts, and systematically makes its deductions from what is observed. . . .But conclusions drawn from unaided reason can hardly be serviceable; only those drawn from observed fact.” Hippocrates: Precepts. (Short communications of factual material are published here. Comments and criticisms appear as Letters to the Editor.)

Multiple

Cutaneous

Hemangiomas

and Coarctation

of the Aorta

With Right Aortic

Arch

Strawberry hemangiomas are the most common congenital vascular malformation seen in infants. Usually, they appear as a raised bright or pun-plish red lobulated tumor with well-defined bor-ders and capillaries protruding from its surface. They are not present at birth. They develop dun-ing the first weeks oflife, grow rapidly during the first 6 months, remain stationary for awhile, and then undergo complete or partial involution and resolution without treatment. Usually, these he-mangiomas are solitary or in small number’ and are not associated with congenital anomalies.

We report a case of strawberry hemangiomas

that is unusual because of the multiplicity and extension associated with laryngeal hemangioma and coanctation with night aortic arch.

CASE REPORT

Jeremy, a 3,850-g male newborn, born after an un-eventful 9-month gestation, was the third child in a family without any significant medical history. On the

eighth day after birth, a strawberry hemangioma ap-peared on his right arm. Between the eighth and the

45th day, several large strawberry hemangiomas were noticed on the right upper limb (Fig 1) and on the right side of the face (Fig 2), the eyelids, the tip of the nose,

the lips, and the right shoulder. There were also

he-mangiomas ofthe nostril and the right side ofthe

phar-ynx. Because of laryngeal dyspnea, a bilateral

heman-Reprint requests to (LV.) Service de Dermatologie, Hopital Trousseau, 37044 Tours, Cedex, France.

PEDIATRICS (ISSN 0031 4005). Copyright © 1988 by the American Academy of Pediatrics.

gioma of the larynx was found when the infant was 2 months old and was confirmed by laryngoscopy. Oral

betamethasone (2 mgfkg/d) was prescribed for the

layn-geal hemangioma and reduced his dyspnea within 48 hours. Betamethasone was progressively decreased during a 5-month period. Evolution of the cutaneous hemangiomas was typical of immature hemangiomas: after a period of stabilization, probably enhanced by

corticosteroids, they began to regress by the 8th month. When the child was 4 years of age, the cutaneous

he-mangiomas had completely disappeared, but the mu-cous hemangiomas of the lips and the tip of the tongue persisted.

A systolic heart murmur and weak peripheral pulses

had been noted since birth. At age 2 months, this mur-mur was of grade IV/VI intensity, maximal at the base of the neck, and radiated to the skull and the abdomen.

No peripheral pulses were palpable. A chest

roentgen-ogram revealed dextroposition of the aorta. There was

no sign of heart failure and cardiac, hepatic, and

cer-ebral ultrasound findings were normal. In spite of the apparent absence of cardiovascular abnormality, the persistence of this murmur at 26 months of age led to the performance of an IV arteriography which

dem-onstrated the right aortic arch in a cervical position

without bronchial compression. Successively, the left

common carotid artery, right common carotic artery, and right subclavian artery arose from the aortic arch;

the left subclavian artery originated from a posterior

diverticulum ofthe distal aortic arch. In addition, there

was a coarctation of the aorta at the level of the

hori-zontal part of the aortic arch (Fig 3). A pressure

gra-dient between the two ends of the hypoplastic segment was 40 mm Hg during cardiac catheterization. At 4 years of age, a grade 1/VI slight systolic murmur

re-mained, and all peripheral pulses were palpable, except

in the right upper limb as a result of the right subcla-vian artery being used for aortoplasty.

DISCUSSION

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Fig 1. Strawberry hemangioma of upper arm.

I,j 3. Arteriography: right aortic arch with

coarcta-tion of aorta.

Fig 2. Strawberry hemangioma of right hemiface.

708 PEDIATRICS Vol. 81 No. 5 May 1988

rarely associated with a congenital

malforma-tion,2 but when they are multiple, regardless of their size, it is imperative to look for associated vascular abnormalities, especially disseminated neonatal hemangiomatosis.4

In our case, the strawberry hemangiomas had

the typical evolution of immature hemangiomas but were unusual with regard to their multiplicity and extension with localization to the right part of the body (face and upper limb), and in their association with hemangiomas involving the mouth, pharyngeal and laryngeal mucosa, coanc-tation of the aortic arch, and a right aortic arch. Laryngeal hemangioma is rare.5 In 50% of cases, it is associated with cutaneous heman-giomas of the face as in our case. It is life-threat-ening and often requires tracheal intubation to avoid acute respiratory distress. Management has

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included drug therapy with systemic corticoste-roids, surgical excision, and carbon dioxide laser excision. Good response to systemic

corticoste-noids without corticodependence or recurrence, as

in our case, is unusual.5 Disseminated neonatal hemangiomatosis may be entirely cutaneous and benign6 on it may be accompanied by visceral he-mangiomas, particularly hepatic but also splenic,

meningeal, encephalic, or pulmonary. These may be responsible for heart failure or hemorrhages

that threaten life.7 Multiple strawberry

heman-giomas have been reported in association with vascular abnormalities such as arteniovenous

fis-tula,8 aneurysm of subclavian or innominate an-tenies,9 and, as in our case, coanctation of the

aorta.9”#{176}

Coarctation of the aorta is rarely associated with cutaneous hemangiomas. In Israel, Schneeweiss et a19 found only four children with cutaneous hemangiomas among 68 children with a coanctation of the aorta confirmed by angiog-raphy, but in these four children the heman-giomas were multiple. Coarctation of the aorta with a right aortic arch, as in our case, is uncom-mon; only eight cases have been reported.’#{176}’6 Right aortic arch is an abnormality of the em-bryologic development of the aorta. Stewart et al,’5 proposed a classification of aortic arch

ab-normalities. His group III consists ofpatients with

right aortic arch due to absence of involution of the right dorsal aortic root; type IIIB is due to involution of the fourth left aortic arch with per-sistence of right and left dorsal aortic root. In our

case, the aortic arch abnormality is type IIIB but with persistence of the third aortic arch rather than the fourth, thus explaining the cervical p0-sition of the aortic arch. It seems possible that a right aortic arch may reduce the risk of occurrence of coarctation of the aorta.’6 During fetal life the bypass through the ductus arteniosus favors low physiologic blood flow into the aortic arch,’7 but with a right aortic arch, the ductus arteniosus is stretched and narrowed, leading to a decrease in the volume ofblood bypassing through the ductus and therefore to an increase in flow through the aortic arch. Because coanctation is favored by a low flow of blood through the aortic arch,’ coarc-tation is rare with right aortic arch because of the relatively high flow of blood through it. Although right aortic arch is not usually associated with

other congenital abnormalities and is rarely as-sociated with coanctation of the aorta, the asso-ciation ofcoanctation ofthe aorta with right aortic arch of type IIIB and multiple strawberry he-mangiomas was reported by Honey et al,’#{176}who reported a 22-month-old boy with large heman-giomas on the right cheek and right side of the

neck and smaller ones on the lips and chin,

to-gether with a coanctation of the aorta located in the right aortic arch between the origin ofthe two subclavian arteries. In the case of Honey et al catheterization showed 92% oxygen saturation in the right subclavian vein; this finding was at-tnibuted to the large hemangiomas.

The exceptional feature of coarctation of the aorta with right aortic arch, the rarity of multiple strawberry hemangiomas, and the similarity of

our observation with that of Honey et al suggest more than a mere coincidence. A low tissue oxy-genation enhances angiogenesis.’8 We

hypoth-esize that coanctation may favor hemangioma be-cause of low blood output below it. Schneeweiss et al,9 who reported four infants with coanctation of the aorta and multiple hemangiomas, proposes

another explanation for this unusual association: the coarctation may result from a possible severe ischemia due to the increase of blood flow within the hemangiomas. The presence in the observa-tion of Honey et al of an arteniovenous shunt within hemangiomas is another argument for this explanation.

Our case demonstrates that, when multiple and

extensive, strawberry hemangiomas should alert the physician to possible vascular anomalies.

ACKNOWLEDGMENT

We thank M. Jaffar, MD, J. Murat, MD, and D.

Zac-char, MD, for their help in the preparation of manu-script.

REFERENCES

LoIc VAILLANT, MD

GERARD LORETTE, MD

ALAIN CHANTEPIE, MD

MICHEL MARCHAND, MD

DANIEL ALIs0N, MD

MARIE-CATHERINE VAILLANT, MD

JEAN LAUGIER, MD

Departments of Pediatrics and

Dermatology

Centre Hospitalo-Universitaine Tours, France

1. Eschwege E, Lelouch J, Schwartz D, et al: Etude #{233}pid#{233}-miologique des angiomes tub#{233}reuxet cutan#{233}s.Arch FrPe-diatr 1966;23:703-714

2. Hurwitz D: Clinical Pediatric Dermatology: A Textbook of Skin Disorders of Childhood and Adolescence. Philadel-phia, WB Saunders, 1981, pp 190-194

3. Margileth AM, Museles M: Cutaneous hemangiomas in children: Diagnosis and conservative management. JAMA 1975;194:523-526

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erup-710 PEDIATRICS Vol. 81 No. 5 May 1988

tive hemangiomata in infants. Pediatr Dermatol 1984;

1:312-317

5. Narcy P, Lacau Saint-Guily J, Manac’h Y, et al: Traite-ment des angiomes sous-glottiques du nourrisson: A

pro-05 de 32 observations. Ann Pediatr 1981;28:721-725 6. Stern JK, Wolf JE, Jaratt M: Benign neonatal

heman-giomatosis. J Am Acad Dermatol 1981;4:442-445 7. Holden KR, Alexander F: Diffuse neonatal

hemangiom-atosis. Pediatrics 1970;46:411-421

8. Cooper AG, Bolande RP: Multiple hemangiomas in an in-fant with cardiac hypertrophy post-mortem angiographic demonstration of the arteriovenous fistulae. Pediatrics

1965;35:27-35

9. Schneeweiss A, Blieden L, Shem Toy A, et a!: Coarctation of the aorta with congenital hemangioma of the face and neck and anevrysm or dilatation of a subclavian of innom-mate artery: A new syndrome? Chest 1982;37:937-945 10. Honey M, Lincoln JLR, Orbone MP, et al: Coarctation of

aorta with right aortic arch. Br Heart J 1975;37:937-945 11. Edelman R, Weintraub R, Paulin 5: Coarctation of the

aorta with right aortic arch ofthe mirror-image type. AJR 1983;140:1135-1 136

12. Felson B, Palayew MJ: The two types of right aortic arch.

Radiology 1963;81:745

13. Grossman M, Jacoby WJ: Right aortic arch and coarctation of the aorta. Dis Chest 1969;56:158

14. Price HL, Schieken RM: Right aortic arch with coarctation ofthe aorta. Chest 1974;65:110-112

15. Stewart JR, Kincaid OW, Edwards JE: An Atlas of Vas-cular Rings and Related Malformation ofthe Aortic Arch System. Springfield, IL, Charles C Thomas publisher, 1969 16. Zelikowsky A, Vidne B, Levy MJ: Mirror image

dextro-cardia with situs inversus and coarctation of the aorta.

Chest 1974;66:297-299

17. Rudolph AM, Meymann MA, Spitznas U: Hemodynamic considerations in the development of narrowing of the aorta. Am J Cardiol 1972;30:514-525

18. Remensnyder JP, Majno G: Oxygen gradients in healing wounds. Am J Pathol 1968;52:301-323

Simple

Interventions-Self-Treatment

The hospital environment is a threatening at-mosphene to a child and so is even the most in-nocent intervention. The sight of a group of

doe-tons, nurses, and students surrounding his on her bed terrifies a young child before he on she is even touched. If the child has experienced an unpleas-ant on painful intervention in the past, he on she will not permit it to happen again unless forced to do so. This usually means that four on six hands will be needed to restrain the child. The inter-vention then turns into a battle between doctor and patient.

Two main psychologic components characterize

this situation: anxiety state and avoidance be-havior. Several methods have been described to

deal with patients’ behavior that interferes with

the effective treatment of their health-related problems. All have in common patient exposure, whether imaginative or neal, to an experience that elicits controlled anxiety. This exposure helps the patient to cope with the frightening con-dition. The various methods include the following: systematic desensitization,’ modeling,2 and ex-posune in vivo.3 Others attempt to enhance the cooperation between child and attending staff by

Received for publication April 13, 1987; accepted Aug 27, 1987. Reprint requests to (R.S.) Department of Plastic Surgery, Re-kach Hospital, Tel-Aviv Medical Center, P0 Box 51, Tel-Aviv, Israel.

PEDIATRICS (ISSN 0031 4005). Copyright © 1988 by the American Academy of Pediatrics.

showing films,4 providing sensory preparatory in-formation,5 or presenting joint parent-child preparation.6 All ofthese techniques are time con-suming, need team work, and, in most hospitals and clinics, are not feasible.

The four patients described illustrate the prob-lem and our approach to solving it without in-vestment of much time or energy.

CASE REPORTS

Patient 1

A 5-year-old boy had surgery twice previously for correction of hypospadias. On several occasions before and after surgery, he had had urethral catheters in-serted for x-ray examinations and other procedures.

Prior to a third operation for closure of a

urethrocu-taneous fistula, we wished to insert a catheter to as-certain the patency of the urethra distal to the fistula. The child heard the doctor ask for a catheter and “went

wild.” Neither the parents nor anybody else could per-suade the boy to allow us to insert the catheter. It was

then suggested that he insert the catheter himself, and he did so without difficulty. Thereafter, the attitude of both the child and his parents became much more

trust-ing toward the staff.

Patient 2

This 6-year-old boy had a history similar to that of

patient 1. When we could not persuade him to insert the catheter himself, he was sent home with a catheter and instructions concerning how to use it. He later

re-turned to the clinic and inserted the catheter in our

presence.

Patient 3

A 4’/2-year-old boy who had undergone several pro-cedures for the repair of hypospadias required dilation

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(5)

1988;81;707

Pediatrics

LAUGIER

MARCHAND, DANIEL ALISON, MARIE-CATHERINE VAILLANT and JEAN

LOÏC VAILLANT, GÉRARD LORETTE, ALAIN CHANTEPIE, MICHEL

Arch

Multiple Cutaneous Hemangiomas and Coarctation of the Aorta With Right Aortic

Services

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1988;81;707

Pediatrics

LAUGIER

MARCHAND, DANIEL ALISON, MARIE-CATHERINE VAILLANT and JEAN

LOÏC VAILLANT, GÉRARD LORETTE, ALAIN CHANTEPIE, MICHEL

Arch

Multiple Cutaneous Hemangiomas and Coarctation of the Aorta With Right Aortic

http://pediatrics.aappublications.org/content/81/5/707

the World Wide Web at:

The online version of this article, along with updated information and services, is located on

American Academy of Pediatrics. All rights reserved. Print ISSN: 1073-0397.

American Academy of Pediatrics, 345 Park Avenue, Itasca, Illinois, 60143. Copyright © 1988 by the

been published continuously since 1948. Pediatrics is owned, published, and trademarked by the

Pediatrics is the official journal of the American Academy of Pediatrics. A monthly publication, it has

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Figure

Fig 1 .Strawberryhemangiomaofupperarm.

References

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