syndrome_of_the_trephined_case_reports

An elderly gentleman with traumatic brain injury underwent DC. He later developed a Syndrome of the trephined as unexplained agitation which responded to cranioplasty by returning to a state of calm. His cognitive function further improved over a period of 6 months. This is an unusual observation reported in this case.

Timely recognition of the cognitive complications of craniectomy that may respond to early cranioplasty promises to decrease the length of hospital stay and enhance rehabilitation in such patients 1)


Demaerel R, Klein S, Van Calenbergh F. Syndrome of the Trephined presenting as Foix-Chavany-Marie syndrome [published online ahead of print, 2020 Jun 30]. Clin Neurol Neurosurg. 2020;196:106058. doi:10.1016/j.clineuro.2020.106058



A case report of the syndrome of the trephined revealed by vertical diplopia 2).

2015

A 52 year old male suffered severe head injury in a road traffic accident and underwent a craniectomy and contusectomy of the left Fronto-Temporo-Parietal (FTP) region for treatment of Acute Subdural hematoma (SDH) as well as hemorrhagic and non-hemorrhagic contusions of the brain with severe mass effect. On recovery from this acute event he was bed bound, on tracheostomy, his GCS was E4VTrM4 with residual right sided hemiparesis. Three months later, he developed Hydrocephalus for which a Right Ventriculo-Peritoneal (V-P) shunt was performed. Following this procedure, severe depression of the skin/scalp flap occurred and the neurological recovery was not as expected. He was diagnosed as a case of “Syndrome of the trephined”. An immediate Cranioplasty was performed, on the third month following the craniectomy procedure, in an attempt to resolve the rapidly deteriorating neurological status of the patient.

In the case presented, following the early Cranioplasty which was performed within three months of the initial craniectomy, the patient's neurological condition and cognitive functions showed a remarkable, immediate and dramatic improvement. The early Cranioplastic repair led to a remarkable clinical recovery of the patient, with improvement in the cognitive behavior and motor deficit with a rapid reversal of the sensorimotor paresis, reflecting an improvement in brain perfusion 3).

2012

Kwon et al., report a case of a patient with sinking skin flap syndrome who suffered from reperfusion injury after cranioplasty 4).

2009

A 77-year-old male patient with an acute subdural hematoma was treated using a hemicraniectomy and evacuation of the hematoma. On the 9th postoperative day there was deterioration in sensorium associated with a sunken scalp flap and worsening midline shift on CT. A significant improvement in sensorium and a filling up of the scalp flap occurred after maintaining the patient's head in a dependent position. The patient subsequently made an excellent recovery following replacement of the bone flap 5).


An extreme syndrome of the trephined after decompressive craniectomy is reported by Bijlenga et al. The most extensive clinical syndrome observed was established over 4 weeks and consisted of bradypsychia, dysartria, and limb rigidity with equine varus feet predominating on the right. The syndrome was aggravated when the patient was sitting with the sequential appearance over minutes of a typical parkinsonian levodopa-resistant tremor starting on the right side, extending to all four limbs, followed by diplopia resulting from a left abducens nerve palsy followed by a left-sided mydriasis. All signs recovered within 1-2 h after horizontalisation. It was correlated with an orthostatic progressive sinking of the skin flap, MRI and CT scan mesodiencephalic distortion without evidence of parenchymal lesion. Brain stem auditory evoked potential wave III latency increases were observed on the right side on verticalisation of the patient. EEG exploration excluded any epileptic activity. Symptoms were fully recovered within 2 days after cranioplasty was performed. The cranioplasty had to be removed twice due to infection. Bradypsychia, speech fluency, limb rigidity and tremor reappeared within a week after removal of the prosthesis. While waiting for sterilisation of the operative site, the symptoms were successfully prevented by a custom-made transparent suction-cup helmet before completion of cranioplasty 6).

2004

A 45-year-old lady underwent right fronto-parietal craniotomy and subtotal excision of a parasagittal meningioma. Bone flap was not replaced as it was infiltrated by the tumor. In the postoperative period she developed episodes of altered sensorium associated with worsening of left hemiparesis and a sunken scalp at the site of bone defect. Computed tomography (CT) of brain showed sunken scalp flap in the right fronto-parietal region with compression of the underlying brain. A diagnosis of syndrome of the trephined was considered and her symptoms improved with cranioplasty 7).


1)
Aziz HF, Baqai MWS, Jooma R. Unusual presentation in syndrome of trephined - A unique case observation. Surg Neurol Int. 2022 Dec 2;13:562. doi: 10.25259/SNI_988_2022. PMID: 36600760; PMCID: PMC9805635.
2)
Hohenbichler K, Duprez T, Ribeiro Vaz JG, Ferrao Santos S, El Tahry R. Syndrome of the trephined revealed by vertical diplopia. Acta Neurol Belg. 2016 Dec 24. doi: 10.1007/s13760-016-0713-3. [Epub ahead of print] PubMed PMID: 28013486.
3)
Jeyaraj P. Importance of Early Cranioplasty in Reversing the “Syndrome of the Trephine/Motor Trephine Syndrome/Sinking Skin Flap Syndrome”. J Maxillofac Oral Surg. 2015 Sep;14(3):666-73. doi: 10.1007/s12663-014-0673-1. Epub 2014 Aug 12. PubMed PMID: 26225060; PubMed Central PMCID: PMC4510081.
4)
Kwon SM, Cheong JH, Kim JM, Kim CH. Reperfusion injury after autologous cranioplasty in a patient with sinking skin flap syndrome. J Korean Neurosurg Soc. 2012 Feb;51(2):117-9. doi: 10.3340/jkns.2012.51.2.117. Epub 2012 Feb 29. PubMed PMID: 22500207; PubMed Central PMCID: PMC3322209.
5)
Joseph V, Reilly P. Syndrome of the trephined. J Neurosurg. 2009 Oct;111(4):650-2. doi: 10.3171/2009.3.JNS0984. PubMed PMID: 19361266.
6)
Bijlenga P, Zumofen D, Yilmaz H, Creisson E, de Tribolet N. Orthostatic mesodiencephalic dysfunction after decompressive craniectomy. J Neurol Neurosurg Psychiatry. 2007 Apr;78(4):430-3. PubMed PMID: 17119005; PubMed Central PMCID: PMC2077792.
7)
Kumar GS, Chacko AG, Rajshekhar V. Unusual presentation of the “syndrome of the trephined”. Neurol India. 2004 Dec;52(4):504-5. PubMed PMID: 15626847.
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