Asymptomatic Interhypothalamic Adhesions in Children

Abstract

SUMMARY:With the use of high-resolution MR imaging techniques, we have increasingly observed anomalies of the hypothalamus characterized by a band of tissue spanning the third ventricle between the hypothalami, often without associated clinical sequelae. Historically, hypothalamic anomalies are highly associated with symptoms referable to a hypothalamic hamartoma, midline congenital disorder, hypothalamic-pituitary dysfunction, or seizures, with very few asymptomatic patients reported. The interhypothalamic tissue described in our cohort was observed incidentally through the routine acquisition of high-resolution T1WI. No referable symptoms were identified in most of the study group. In the appropriate patient population in which associated symptoms are absent, the described hypothalamic anomalies may be incidental and should not be misdiagnosed as hypothalamic hamartomas. ABBREVIATIONS:GMH ϭ gray matter heterotopia; IHA ϭ interhypothalamic adhesion

Asymptomatic Interhypothalamic Adhesions in Children

“…1 We agree with the main message of the article: Referable hypothalamicpituitary axis symptoms are rare in patients with interhypothalamic adhesions. However, because symptoms can be present on occasion, it is prudent to exclude endocrinopathy on clinical grounds.…”

“…[2][3][4] Indeed, review of Fig 1 demonstrates sub-tle midline anomalies not mentioned in the article, including hypogenesis or volume loss of the splenium (Figs 1A and E) and a partially fenestrated, persistent cavum septum pellucidum ( Fig 1D). 1 In normal brains, the callosal splenium is typically equal to or larger in caliber than the genu. While a cavum septum pellucidum is a normal variation, it is uncommon in the general population beyond the neonatal period.…”

High prevalence of syndromic disorders in patients with non-isolated central precocious puberty

“…They are less frequently described and are rarely associated with midline developmental abnormalities, as shown by the very small number of cases reported to date. The underlying etiology of IHA remains unclear, but it has been suggested that IHA results from incomplete hypothalamus cleavage, failed apoptosis or abnormal neuronal migration (28,29). Endocrine dysfunction with anterior pituitary deficiency has been reported in only three patients with IHA (29).…”

“…The underlying etiology of IHA remains unclear, but it has been suggested that IHA results from incomplete hypothalamus cleavage, failed apoptosis or abnormal neuronal migration (28,29). Endocrine dysfunction with anterior pituitary deficiency has been reported in only three patients with IHA (29). An association of developmental defects of the hypothalamic-pituitary area, such as septo-optic dysplasia and/or ectopic posterior pituitary, with CPP has been reported in a few cases (30,31,32), but no similar case of CPP and IHA has ever been described, to our knowledge.…”

Hypothalamic Adhesions: Asymptomatic, Incidental, or Not?

“…2 The authors proposed that it may be a form fruste of holoprosencephaly, given the prevalence of midline abnormalities. Ahmed et al 3 recently reported in the AJNR that most patients with this finding are asymptomatic and that these are often incidental findings. So what is the practitioner to do with these seemingly conflicting results?…”