Pourfour du Petit Syndrome
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Disease entity
Disease
Pourfour du Petit (PDP) syndrome (i.e., reverse Horner syndrome) is a rare sympathetic nervous system disorder that consists of ipsilateral mydriasis, hyperhidrosis, and eyelid retraction. PDP syndrome (PDPS) has the opposite clinical presentation of the Horner syndrome (HS) which is an oculosympathetic paresis. PDPS involves overstimulation of the oculosympathetic pathway.[1]
Etiology
PDPS often is iatrogenic or traumatic. Like HS, the lesion affects the oculosympathetic chain[2] and PDPS has been reported in carotid artery dissection[3]; after regional anesthesia (e.g., intraoral, epidural anesthesia, brachial plexus blocks, interscalene blocks);[4] [5] [6] [7] and following surgeries affecting the carotid artery (e.g., vascular catheter insertion/discontinuation) that may compromise the cervical sympathetic chain.[7] Rarely, tumors can compress the oculosympathetic chain or local vasculature and produced PDPS (e.g., thyroid carcinoma with internal carotid artery compression[8], esophageal carcinoma with pleural extension).[9] Central ischemic lesions can also result in PDPS (e.g., top of the basilar stroke with bilateral thalamic, midbrain infarct).[10] There is also a report of PDPS as a presenting sign in subdural hematoma into the spinal subdural space.[11]
Pathophysiology
PDPS is believed to be due to oculosympathetic chain irritation causing the clinical signs of hyperactivity of the sympathetic nervous system (e.g., upper limb hyperhidrosis, lid retraction, anisocoria, and ipsilateral mydriasis).
Relevant Anatomy
Oculosympathetic Pathway - A three neuron pathway
First order neuron
First order neuron sympathetic fibers originating in the pupillary pathway begin in the hypothalamus, descend caudally and dorsally through the mesencephalon and medulla oblongata, meeting the C8-T2 segments of the spinal cord and then synapsing with the second order neuron..
Second order neuron
The second order neuron exits the spinal cord with the first ventral thoracic root to form a portion of the paravertebral sympathetic chain and travels upward to the cervical ganglion at the same level as the mandibular ganglion.
Third order neuron
The third order postganglionic fibers travel parallel to the internal carotid artery and enter the cavernous sinus, join for a short course with cranial nerve VI and then cranial nerve V to innervate the eye via the long ciliary nerves.
Diagnosis
PDPS is a largely a clinical diagnosis.
Signs/symptoms
PDPS generally follows the classic triad of mydriasis, hyperhidrosis, and eyelid retraction. A history of local/regional anesthesia or head/neck trauma or surgery in the three neuron sympathatic pathway should raise suspicion for PDPS.
Evaluation
Neuroimaging of the oculosympathetic axis (as in the HS) is recommended for PDPS to exclude a structural lesion (e.g., carotid dissection, neoplasm).In the acute setting, Computed tomography (CT) and CT angiogram (CTA) of the head neck is recommended but magnetic resonance imaging (MRI) of head and neck with MRA might still be necessary to exclude structural lesions along the sympathetic chain.
Management
Medical Management
Many patients do not require any treatment beyond therapy for the underlying etiology of the PDPS. The symptoms and signs of PDPS have been treated successfully with clonidine (an alpha-2 adrenergic agonist).[11]
Surgery
Sympathectomy has been used to reduce sympathetic activity in persistent cases of PDPS and has been found to be effective in up to 90% of cases.[12] [13]
Prognosis
The simultaneous presentation of these symptoms can last for a month at a minimum or be indefinite. In some cases, PDPS may spontaneously resolve.[14]
Summary
Clinicians should be aware of a rare presentation of oculosympathetic overaction (PDPS). The PDPS is the clinical opposite of the HS but has the same underlying mechanism (oculosympathetic chain damage) and the evaluation for PDPS should probably be the same as for HS including neuroimaging.
References
- ↑ Marín-Lambíes C, España-Gegori E, Gallego-Pinazo R, Climent-Vallano L, Muelas N, Díaz-Llopis M. Pourfour du petit syndrome associated with a cervical vertebral anomaly. Journal of Neuro-Ophthalmology. 2012;32(4):348-349. doi:10.1097/wno.0b013e3182625212
- ↑ Avellanal M, Fernández-Quero L, Barrios JM, Sánchez P, Navia J. Pourfour du Petit Syndrome: A case following a traffic accident with severe cranioencephalic trauma. Intensive Care Medicine. 1996;22(10):1090-1092. doi:10.1007/bf01699233
- ↑ Sánchez-de la Torre JR, Drake-Pérez M, Casado A, et al. Persistent isolated mydriasis as an early sign of internal carotid artery dissection: Pourfour du petit syndrome. Clinical Neurology and Neurosurgery. 2019;182:70-72. doi:10.1016/j.clineuro.2019.04.030
- ↑ Ségura P, Speeg-Schatz C, Wagner JM, Kern O. Le syndrome de Claude Bernard-Horner et son contraire, le syndrome de pourfour du petit, en anesthésie-réanimation [Claude Bernard-Horner syndrome and its opposite, Pourfour du Petit syndrome, in anesthesia and intensive care]. Ann Fr Anesth Reanim. 1998;17(7):709-724. doi:10.1016/s0750-7658(98)80108-1
- ↑ Large M, Salles C, Descoins PF, et al. [Pourfour Du Petit syndrome following brachial plexus block]. Annales Francaises D'anesthesie et de Reanimation. 1984 ;3(3):232-234. DOI: 10.1016/s0750-7658(84)80061-1. PMID: 6742547.
- ↑ Santhosh MC, Pai RB, Rao RP. Pourfour Du Petit syndrome after interscalene block. Saudi J Anaesth. 2013;7(2):203-204. doi:10.4103/1658-354X.114055
- ↑ 7.0 7.1 Aron J, Chatterjee D. Transient pourfour du petit syndrome. Anesthesia & Analgesia. 2007;104(6):1616. doi:10.1213/01.ane.0000260639.96416.2a
- ↑ Martinez-Ramirez S, Roig C, Martí-Fàbregas J: Pourfour du Petit Syndrome in a Patient with Thyroid Carcinoma. Case Rep Neurol 2010;2:96-100. doi: 10.1159/000319587
- ↑ Aouba A, Der Agopian P, Genty-Le Goff I, Mutschler C, Janin N, Patri B. Le syndrome de Pourfour du Petit : une cause rare d'exophtalmie unilatérale avec mydriase et élargissement de la fente palpébrale [Pourfour du Petit syndrome: a rare aetiology of unilateral exophthalmos with mydriasis and lid retraction]. Rev Med Interne. 2003;24(4):261-265. doi:10.1016/s0248-8663(03)00029-8
- ↑ Nurmukhamedov B, Rakhesh A, Shah V, Culebras V. Pourfour du Petit Syndrome in an Acute Top of the Basilar Artery Stroke. Neurology. 2017; 90(15): 344. https://n.neurology.org/content/90/15_Supplement/P4.344. April 9, 2018.
- ↑ 11.0 11.1 Balik V, Kolembus P, Svajdler M, Sulla I, Vaverka M, Hrabalek L. A case report of rapid spontaneous redistribution of acute supratentorial subdural hematoma to the entire spinal subdural space presenting as a Pourfour du petit syndrome and review of the literature. Clinical Neurology and Neurosurgery. 2013;115(7):849-852. doi:10.1016/j.clineuro.2012.09.007
- ↑ Milanez de Campos JR, Kauffman P, de Campos Werebe E, et al. Quality of life, before and after thoracic sympathectomy: Report on 378 operated patients. The Annals of Thoracic Surgery. 2003;76(3):886-891. doi:10.1016/s0003-4975(03)00895-6
- ↑ Gossot D, Galetta D, Pascal A, et al. Long-term results of endoscopic thoracic sympathectomy for Upper Limb Hyperhidrosis. The Annals of Thoracic Surgery. 2003;75(4):1075-1079. doi:10.1016/s0003-4975(02)04657-x
- ↑ Evans, R.W., Garibay, A. and Foroozan, R. (2017), Pourfour du Petit Syndrome Associated With Right Eye Pressure. Headache: The Journal of Head and Face Pain, 57: 937-942. https://doi.org/10.1111/head.13059.
