Asymmetric sweating and flushing in infants with esophageal atresia

doi:10.1016/j.jpedsurg.2009.03.028

Journal of Pediatric Surgery

Volume 44, Issue 6, June 2009, Pages e27-e29

https://doi.org/10.1016/j.jpedsurg.2009.03.028 Get rights and content

Abstract

Of 136 infants with repaired esophageal atresia, one presented an unilateral facial flushing and 2 presented a flushing and sweating of one half of the body. The topography of these disorders and/or the associated clinical manifestations suggest that the asymmetry may be related to an instability of unilateral autonomic centers more than to a surgical injury of upper thoracic sympathetic chain during esophageal repair.

Section snippets

Patients and methods

Between 1967 and 2007, 136 patients with esophageal atresia and/or tracheoesophageal fistula were treated at our institution. We reviewed the hospital records of patients consecutively admitted between January 2003 and December 2007 to update the database used in a previous study [2]. In our database, flushing was not coded. We then selected all patients who presented with hyperhidrosis and reviewed their original written notes to select the patients who presented with unilateral sweating. The

Results

Of 136 patients with esophageal atresia and/or tracheoesophageal fistula, 40 patients presented hyperhidrosis. Of these, cases 2 and 3 presented unilateral sweating, and case 1 presented an hemifacial flushing associated with a symmetrical hyperhidrosis. All the 3 infants had a right thoracotomy to repair an esophageal atresia with a lower tracheoesophageal fistula. Case 2 had a right-sided aortic arch which required also a left thoracotomy for excision of a severe esophageal stricture at the

Discussion

Hemifacial flushing without body involvement (Case 1) is most likely to be the first reported in an infant with repaired esophageal atresia. Redness of the skin of one half of the body was first described by Neligan and Strang [4] in premature babies or in full-term babies with clinical evidence of cerebral damage. They called this condition “Harlequin color change.” The unilateral distribution of color change suggested a dysfunction of the autonomic centers which control the tone of the skin

Acknowledgment

We thank Dr Francesco Morini for signalizing that the hemifacial flushing of case 1 had been represented in the literature as Harlequin syndrome.

References (12)

CozziF. et al.
Esophageal atresia, choanal atresia, and dysautonomia
J Pediatr Surg
(1991)
CozziF. et al.
Maturational dysautonomia and facial anomalies associated with esophageal atresia: support for neural crest involvement
J Pediatr Surg
(1993)
NeliganG.A. et al.
A “Harlequin” colour change in the newborn
Lancet
(1952)
CozziD.A. et al.
Pathogenesis of apparent life-threatening events in infants with esophageal atresia
Pediatr Pulmonol
(2006)
LanceJ.W. et al.
Harlequin syndrome: the sudden onset of unilateral flushing and sweating
J Neurol Neurosurg Psychiatry
(1988)
DarvallJ. et al.
Coexisting harlequin and Horner syndromes after paediatric neck dissection: a case report and a review of the literature
J Plastic Reconstr Aesthetic Surg
(2007)

There are more references available in the full text version of this article.

Cited by (3)

Harlequin syndrome in a melanoderm patient
2016, Revue de Stomatologie, de Chirurgie Maxillo-faciale et de Chirurgie Orale
Le syndrome d’Arlequin est un trouble neurologique de topographie faciale, lié à une dysfonction de l’innervation sympathique de la face. Il est rarement décrit dans la littérature. Les auteurs décrivent un cas observé chez un sujet mélanoderme.
Un patient de 38 ans, mélanoderme, sans antécédents de chirurgie ni de traumatisme cervical, est venu consulter pour une hyperhidrose faciale strictement unilatérale droite. Les explorations clinique et radiologique ont conclu à un syndrome d’Arlequin, de cause idiopathique probable. L’abstention thérapeutique a été proposée en raison du caractère non invalidant des symptômes.
Le syndrome d’Arlequin est un trouble vaso-sudoro-moteur rare. Sur peau noire, le flush et l’érythrose peuvent être masqués, rendant le diagnostic moins évident. Les étiologies sont variées mais dominées par la cause idiopathique. Son association avec d’autres syndromes dysautonomiques de la face est possible. Le principal diagnostic différentiel est le syndrome de Frey. Le traitement n’est pas encore clairement codifié.
Harlequin's syndrome is a neurological disorder due to a dysfunction of the sympathetic innervation of the face. It has been rarely reported in the literature. The authors report one case occurring in a melanoderm patient.
A 38-year-old melanoderm man, without any history of surgery or neck trauma, consulted for a strictly right unilateral facial hyperhidrosis. Clinical and radiological investigations concluded to an idiopathic Harlequin's syndrome. Therapeutic abstention was proposed because of non-invalidating symptoms.
Harlequin's syndrome is a rare sudoral and vaso-motor disorder. On dark skin, flush and erythrosis may be subtle and the diagnosis less obvious. Etiologies are varied but essentially idiopathic. Its association with others dysautonomic facial syndromes is possible. Main differential diagnosis is the Frey's syndrome. Treatment is not clearly codified.
Dysautonomic syndrome of the face with Harlequin sign and syndrome: Three new cases and a review of the literature
2013, Revue Neurologique
Le phénomène d’Harlequin associe une érythrose faciale avec flush et hyperhydrose strictement unilatérale, associé à une pâleur avec anhydrose controlatérale : il est isolé (syndrome d’Harlequin [Sy d’Harlequin]) ou peut apparaître comme signe (signe d’Harlequin [Si d’Harlequin]) s’intégrant alors dans d’autres syndromes dysautonomiques comme un syndrome d’Horner, un syndrome d’Holmes Adie ou encore un syndrome de Ross.
Les auteurs rapportent les observations de trois cas. Deux patients se présentaient avec un Si d’Harlequin, associé avec un syndrome d’Horner droit pour le premier et associé avec un syndrome de Ross à droite pour le deuxième patient : l’enquête étiologique fut normale permettant de retenir le caractère idiopathique des troubles. La troisième patiente présentait un syndrome de Harlequin gauche lequel survenait dans un contexte de néoplasie mammaire avec dissémination métastatique notamment osseuse, et en particulier une infiltration de la vertèbre T2 à droite.
Une revue de la littérature est proposée au travers de 108 cas décrits. Quarante-huit cas de Sy d’Harlequin sont rapportés, à côté de 60 signes d’Harlequin avec respectivement 38 syndromes d’Horner, six syndromes de Holmes et Adie, six syndromes de Ross, cinq cas avec à la fois un syndrome de Ross et de Holmes et Adie et cinq cas avec un syndrome dysautonomique non caractérisable. Les mécanismes physiopathologiques, avec la discussion d’une neuropathie autonome crânienne, sont évoqués à côté d’une discussion des étiologies possibles ainsi que de leur prise en charge thérapeutique.
Le phénomène d’Harlequin avec rougeur et hyperhydrose unilatérale est rare ; survenant isolément ou associé à d’autres syndromes dysautonomiques du visage, il est idiopathique deux fois sur trois, ailleurs il est le résultat d’un phénomène compressif, notamment néoplasique, ou encore est iatrogène. Hormis les étiologies compressives, il ne justifie pas de traitement spécifique ; une sympathectomie est discutée dans les seules formes sévères avec retentissement social important.
Harlequin phenomenon is characterized by a strictly unilateral erythrosis of the face with flushing and hyperhydrosis, and controlaterally a pale anhydrotic aspect. This syndrome can occur alone or associated to other dysautonomic phenomena such as Horner syndrome, Adie syndrome or Ross syndrome.
We report three cases: two patients presented a Harlequin sign, associated with Horner syndrome for one and Ross syndrome for the second. The etiologic investigation was normal, allowing recognizing the idiopathic nature of the disorder. For the third patient, Harlequin syndrome was observed in a neoplastic context due to breast cancer, metastatic dissemination, and bone metastases involving the right side of the T2 body.
We reviewed the literature: 108 cases have been described. This syndrome occurred alone in 48 patients and was associated with other dysautonomic syndromes such as Horner syndrome in 38 patients, Holmes Adie syndrome in six, and Ross syndrome in six; both Ross and Holmes Adie syndrome were associated five cases and associations were not reported in five patients. The pathophysiological mechanisms of this autonomic cranial neuropathy, the possible etiologies, and therapeutic management were discussed.
Harlequin phenomenon with flushing and unilateral hyperhydrosis is rare, occurring alone or in combination with other autonomic syndromes of the face. Idiopathic in two-thirds of cases, Harlequin phenomenon does not require specific treatment; sympathectomy may be discussed in the severe cases with a significant social impact.
Harlequin Syndrome
2018, Indian Journal of Pediatrics

^☆: No grant or other form of payment was given to anyone to produce the manuscript. There is no potential conflict of interest, real or perceived.

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Asymmetric sweating and flushing in infants with esophageal atresia☆

Abstract

Section snippets

Patients and methods

Results

Discussion

Acknowledgment

J Pediatr Surg

J Pediatr Surg

Lancet

Pathogenesis of apparent life-threatening events in infants with esophageal atresia

Pediatr Pulmonol

Harlequin syndrome: the sudden onset of unilateral flushing and sweating

J Neurol Neurosurg Psychiatry

Coexisting harlequin and Horner syndromes after paediatric neck dissection: a case report and a review of the literature

J Plastic Reconstr Aesthetic Surg