PEDIATRICS Vol. 116 No. 6 December 2005, pp. 1603-1604 (doi:10.1542/peds.2005-2013)
Left-Hemispheric Hydranencephaly With Less Favorable Findings
Eric Lewin Altschuler, MD, PhDDepartment of Physical Medicine and Rehabilitation,
University of Medicine and Dentistry of New Jersey,
Newark, NJ 07101,
Department of Rehabilitation Medicine,
Mt Sinai School of Medicine,
New York, NY 10029
Brittany Matsumura, MD
Jaishree Capoor, MD
Department of Rehabilitation Medicine,
Mt Sinai School of Medicine,
New York, NY 10029,
Department of Rehabilitation Medicine,
Elmhurst Hospital Center,
Elmhurst, NY 11373
Karen Weinshelbaum, BA
Mt Sinai School of Medicine,
New York, NY 10029
Hema Ghuznavi, BSBA
Department of Rehabilitation Medicine,
Elmhurst Hospital Center,
Elmhurst, NY 11373
To the Editor.—
We read with great interest the recent article by Ulmer et al1 regarding a child with left-hemispheric hydranencephaly (only the eighth reported case of hemihydranencephaly1–8) who remarkably had a nearly normal life with only mild hemiparesis and no cognitive deficits despite lacking a left cerebral hemisphere. We want to note here the ninth reported case of hemihydranencephaly, also in a patient with left-hemispheric hydranencephaly; unfortunately, it seems that our patient will likely not have as good an outcome as that of the patient described by Ulmer et al.
We have been following a young male child who was born at term. He had mild hydrocephalus, and a computed tomography scan showed left-hemispheric hydranencephaly (Fig 1). Motor and neurologic examination at birth and 2 months were normal (consistent with the thinking that motor control at this age is subcortical) except for a strong rightward head-rotation preference that has persisted. At 3 months of age he was found to have gross motor delay, generalized hypertonia (upper extremities greater than lower, right greater than left), less right upper-extremity spontaneous movement than left, and increased right-hand fisting on attempted voluntary grasping by the left hand.
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The case described by Ulmer et al,1 our case, and the other published cases2–7 show the marked range in motor ability on the side contralateral to the missing hemisphere. As well, good cognitive development is possible without either a left1 or a right7 hemisphere. The known cases of hemihydranencephaly clearly illustrate how much there is still to be learned about human development, and close follow-up of patients with hemihydranencephaly may give important insights into the role of cortical and subcortical structures subserving motor, language, speech, cognitive and visual processes, and neural development and plasticity.
We also note that in 8 of the 9 reported cases of hemihydranencephaly the patient had an absent left hemisphere. (Our patient lacked a left hemisphere, as does the patient described by Ulmer et al. The patient described by Greco et al8 also lacks a left hemisphere. In their Table 1, Greco et al list the 6 other cases of hemihydranencephaly, with 3 patients lacking a left hemisphere, 2 patients lacking a right hemisphere, and for 1 patient no mention of which hemisphere is lacking. Inspection of the original articles shows that there is 1 case of a patient lacking a right hemisphere.7 However, another case listed as such in Table 1 of the Greco et al article in fact lacks a left hemisphere,4 and the case listed without mention of which hemisphere is absent also lacks a left hemisphere.3) If left or right is a chance occurrence, assuming a simple Bernoulli trial model, we would expect 4.5 ± 1.5 cases of absent left hemisphere. The sample size is small, but nevertheless, there then is a <5% probability that 8 of 9 reported cases of hemihydranencephaly involve absent left hemispheres. This possibly may represent a difficulty in development, although not an impossible one,7 when lacking the right hemisphere or a relative proclivity for lesion to the left hemisphere during development. It will be interesting to see if this trend of left hemihydranencephaly is maintained in future cases.
REFERENCES
- Ulmer S, Moeller F, Brockmann MA, Kuhtz-Buschbeck JP, Stephani U, Jansen O. Living a normal life with the nondominant hemisphere: magnetic resonance imaging findings and clinical outcome for a patient with left-hemispheric hydranencephaly.
Pediatrics. 2005;116
:242
–245
[Abstract/Free Full Text] - Muir CS. Hydranencephaly and allied disorders: a study of cerebral defect in Chinese children. Arch Dis Child. 1959;34 :231 –246
- Warkany I. Congenital Malformations: Notes and Comments. Chicago, IL: Year Book Medical; 1971
- Moser RP, Seljeskog EL. Unilateral hydranencephaly: case report. Neurosurgery. 1981;9 :703 –705[Medline]
- Suzuki M, Seki H, Yoshimoto T. Unilateral hydrocephalus combined with occlusion of the ipsilateral internal carotid artery. Surg Neurol. 1985;24 :27 –30[CrossRef][Medline]
- Ohtsuka H. A rare patient with a false median cleft lip associated with multiple congenital anomalies. Ann Plast Surg. 1986;17 :155 –160[CrossRef][Web of Science][Medline]
- van Doornik MC, Heenekam RC. Hemi-hydranencephaly with favourable outcome. Dev Med Child Neurol. 1992;34 :454 –458[Medline]
- Greco F, Finocchiaro M, Pavone P, Trifiletti RR, Parano E. Hemihydranencephaly: case report and literature review.
J Child Neurol. 2001;16
:218
–221
[Abstract/Free Full Text]
PEDIATRICS (ISSN 1098-4275). ©2005 by the American Academy of Pediatrics
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Pediatrics 2005 116: 1604.[Extract] [Full Text]
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