Dorsal medullary cavernous hemangioma presenting as obstinate hiccups and its surgical treatment: illustrative case

Abstract

BACKGROUND

Hiccups are characterized by involuntary, intermittent, repetitive, myoclonic, and spasmodic contractions of the diaphragm. Hiccups are termed “intractable” when they last for over 1 month.

OBSERVATIONS

A rare case of intractable hiccups due to an uncommon location of cavernous hemangioma in the dorsal medulla is illustrated. With respect to the management, surgical excision was performed, and postsurgical complete recovery was witnessed, which has been reported only in six cases worldwide to date.

LESSONS

A mechanism of the hiccups reflex arc is discussed in detail with special reference to the need for equal emphasis on evaluating central nervous system causes and peripheral etiologies for pertinent hiccups.

Hiccups are characterized by involuntary, intermittent, repetitive, myoclonic, and spasmodic contractions of the diaphragm and inspiratory intercostal muscles, leading to an abrupt and early closure of the glottis, terminating inspiration, and generating the characteristic “hic” sound.^1,2

A single episode of hiccups can last from a few seconds to a few minutes and is usually self-limiting. Hiccups are termed “persistent” or “protracted” when they last for more than 48 hours. They are termed “intractable” when they last for over 1 month. An episode lasting for more than 1 week resistant to sequential therapy using three different drugs warrants the use of the label “obstinate.”^3,4

In this article, we report such a rare case of obstinate hiccups due to cavernoma in the dorsal medulla in which the patient instantly and completely recovered after excision. Only six such surgically treated cases have been reported worldwide to date.

Illustrative Case

History and Examination

A young male in his 20s with no previous comorbidities presented to the neuroscience department with a complaint of continuous hiccups, sudden in onset, nonprogressive with no aggravating or relieving factors, and persistent even during sleep. The patient was asymptomatic 13 years earlier when he had an episode of fever with headache associated with one episode of vomiting, after which he developed sudden-onset hiccups. Initially, he tried to manage the condition with home or folk remedies. Unsuccessful, he consulted a physician, who prescribed multiple and repetitively revised medications, which included chlorpromazine, metoclopramide, itopride, and baclofen, over a span of months, but his hiccups failed to improve. He was then referred to a psychiatrist to rule out any psychological etiology and later to a gastroenterologist, upon which he underwent repetitive and varied trials of medications and underwent upper gastrointestinal endoscopy thrice with no significant results. A computed tomography scan of the brain and electroencephalography were performed, the results of which were normal.

After all the failed treatment attempts, he was then referred to us with the diagnosis of intractable hiccups, and he was clinically and radiologically evaluated in detail. Clinically, the patient had persistent myoclonus at a frequency for four hiccups every 10 seconds with synchronous palatal myoclonus (Video 1 and 2). He had no complaint of any weakness/sensory complaints in any of his four limbs. Even though no other neurological deficit was apparently present, contrast-enhanced magnetic resonance imaging (MRI) of the brain with the cervical spine was performed. The brain MRI report (Figures 1–4) was suggestive of a small, round, globular, hypointense lesion blooming (cavernoma), measuring about 10 × 11 mm, which, on diffusion tensor imaging, was seen in the dorsal part of the medulla compressing and splaying spinal nuclei of the trigeminal/hypoglossal nerves and the fasciculus cuneatus and gracilis.

FIG. 1.

Axial T2-weighted MRI showing a cavernoma in the dorsal medulla.

FIG. 4.

MRI tractography showing dorsal medulla compression.

FIG. 2.

Axial T1-weighted MRI with contrast showing a cavernoma in the dorsal medulla and contrast nonenhancement.

FIG. 3.

Sagittal T2-weighted MRI showing a cavernoma in the dorsal medulla.

VIDEO 1. Clip showing obstinate regular palatal myoclonus with rate of four every 10 seconds. Click here to view.

VIDEO 2. Clip showing obstinate synchronous diaphragmatic myoclonus (hiccups) along with the palatal. Click here to view.

The Department of Neurosurgery was consulted, and the case was discussed. Surgical excision of the cavernoma was the decided plan of action. The patient was planned for surgery with proper counseling regarding all the risks and benefits associated with the surgery.

Surgical and Perioperative Course

With the presumptive diagnosis of cavernous hemangioma in the medulla oblongata, surgery was planned. The patient was prone. A midline incision was made between the inion and C2 spinous process. Suboccipital craniotomy was performed, and the dura was opened in a Y-shaped fashion. The lesion was covered by normal parenchymal tissue and made a bulging contour of the medulla oblongata. A 1-cm longitudinal pial incision was made caudally at the posterior median sulcus. After dissection a few millimeters deep, the tumor mass was identified as an irregular dark brownish colored mulberry shaped mass. The plane of the dissection between the cavernoma and the parenchyma was well distinguished. The lesion was removed en bloc. The patient’s hiccups resolved immediately after surgery. The patient’s postoperative course was uneventful. He was extubated on day 1, then shifted to the ward on day 2. His myoclonus had completely resolved with no postoperative neurological deficit.

Discussion

Observations

Cavernomas are vascular malformations lined by thin walls devoid of smooth muscle. Generally, they are devoid of any brain tissue entrapped between the vascular channels. In the brainstem, they are considered vascular tumors producing long-tract signs or cranial nerve deficits due to real mass effect in such a densely eloquent area.⁵ The clinical manifestations of brainstem cavernomas are dependent on their precise anatomical location. In our patient’s case, the slow growth of the cavernoma in the dorsal aspect of the medulla oblongata interfered in the centrally mediated pathways, leading to hiccups. Porter et al.⁶ reviewed 100 cases of brainstem cavernous malformations, of which only 3 had hiccups as the clinical presentation. The six reported cases of medullary cavernomas presenting as intractable hiccups, which completely resolved on surgical excision, are listed in Table 1.^5–13

TABLE 1.

Cases of surgical excision of medullary cavernoma to treat intractable hiccups

Authors & Year	Region	Age (yrs)/Sex	Duration	Size of Cavernoma (cm)	Location of Cavernoma	Complications	Hiccup Resolution Postoperatively
Eisenächer & Spiske, 20119	Germany	26/M	Undefined	2.2	Undefined	Slight dysesthesia	Immediate
Mattana, 201010	Brazil	40/M	3 mos	<1	Lateral	None	Immediate
Musumeci et al., 20005	Italy	46/M	2 mos	<1.5	Lateral	None	Immediate
Pechlivanis et al., 201011	Germany	33/M	Several mos	<1	Lateral	None	Immediate
Thaci et al., 201312	USA	36/F	3 yrs	1.4	Lateral	Mild dysphonia, dysphagia	Immediate
Lee et al., 201413	Korea	28/M	15 days	2	Lateral	Transient sensorimotor weakness	Immediate
Present case	India	20s/M	13 yrs	1.1	Dorsal	None	Immediate

Hiccups were previously considered a gastrointestinal reflex whose center was located in the spinal cord at the cervical-thoracic level.⁸ But several studies and research have hypothesized a much more elaborate mechanism of hiccup generation.

Peripherally, the afferent pathway of the hiccup reflex arc comprises the sensory branches of the phrenic and vagus nerves as well as dorsal sympathetic fibers; its main efferent limb causes spasm of the diaphragm mediated by motor fibers of the phrenic nerves peripherally.⁸ The central nervous system (CNS) centers include the upper spinal cord (C3–5), the myoclonic triangle of Guillain-Mollaret at the pontomedullary level,⁷ the dorsolateral part of the medulla near the respiratory center, the reticular formation, and the hypothalamus.

Lessons

This case is an uncommon presentation of a brainstem lesion and a very infrequently considered etiology for intractable hiccups. With the results of all the broad neurological examinations being within normal limits, the rare possibility of vascular malformations in the brainstem is easily missed. Hiccups can be triggered by both CNS and peripheral lesions in a case of intractable hiccups, so, along with the basic investigations, MRI must be performed. Once properly diagnosed, years of morbidities and disturbed quality of life can be avoided; otherwise, the patient might undergo a series of ardent invasive and noninvasive investigations and treatments, despite the condition being completely resolvable almost immediately with an active surgical intervention.

Acknowledgment

The authors thank IMS & SUM Hospital, SOA University, Bhubaneswar for its expert guidance and support.

Disclosures

The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

Author Contributions

Conception and design: Dash, Acharya. Acquisition of data: Puppala. Analysis and interpretation of data: Dash, Choudhury. Drafting the article: Puppala. Critically revising the article: Puppala, Acharya. Reviewed submitted version of manuscript: Puppala. Approved the final version of the manuscript on behalf of all authors: Acharya. Study supervision: Choudhury.

Supplemental Information

Videos

Video 1. https://vimeo.com/777376996

Video 2. https://vimeo.com/777379179