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A 12-year-old male, who had a history of trauma in his right eye at 2 years of age, presented to the clinic with diminution of vision and whitish discoloration in his right eye. Detailed history and clinical examination confirmed findings of congenital corneal anesthesia due to hereditary sensory autonomic neuropathy type 4, a rare genetic disorder. [ABSTRACT FROM AUTHOR]

A 12-year-old male, who had a history of trauma in his right eye at 2 years of age, presented to the clinic with diminution of vision and whitish discoloration in his right eye. Detailed history and clinical examination confirmed findings of congenital corneal anesthesia due to hereditary sensory autonomic neuropathy type 4, a rare genetic disorder.

Congenital corneal anesthesia (CCA) is an extremely rare condition where the cornea is affected in isolation or as a part of congenital syndrome, or can be associated with systemic anomalies. This case series of 12 eyes provides an overview of various clinical presentations and their final treatment outcomes. The average age of presentation was 3.2 years with a female preponderance (75%). Fifty percent of the patients had bilateral involvement and 50% had corneal ulcers at presentation. Two eyes required therapeutic keratoplasty for corneal perforation. All patients had isolated CCA except for one who had an associated hereditary and sensory autonomic neuropathy.

Congenital corneal anesthesia (CCA) is an extremely rare condition where the cornea is affected in isolation or as a part of congenital syndrome, or can be associated with systemic anomalies. This case series of 12 eyes provides an overview of various clinical presentations and their final treatment outcomes. The average age of presentation was 3.2 years with a female preponderance (75%). Fifty percent of the patients had bilateral involvement and 50% had corneal ulcers at presentation. Two eyes required therapeutic keratoplasty for corneal perforation. All patients had isolated CCA except for one who had an associated hereditary and sensory autonomic neuropathy. [ABSTRACT FROM AUTHOR]

Neurotrophic keratitis is a rare degenerative disease of the cornea that can lead to corneal ulceration, scarring, and significant visual impairment. It most commonly occurs in adults and is rarely diagnosed in children. Congenital corneal anesthesia is an extremely rare condition that requires appropriate ophthalmologists' attention in making diagnosis and treatment decisions. This condition usually presents in infancy or early childhood and is characterized by rare blinking rate, decreased tearing or a corneal ulcer that is unresponsive to treatment. In this case report, we describe a patient with multiple systemic and neurological disorders who presented to the ophthalmology department due to corneal erosion unresponsive to treatment. Brain magnetic resonance imaging confirmed bilateral trigeminal hypoplasia and the diagnosis of neurotrophic keratopathy due to bilateral congenital corneal anesthesia was made. The discrepancy between clinical signs and symptoms or treatment non-response in cases of corneal erosions should alert the ophthalmologists to suspect trigeminal dysfunction. MRI is the gold standard to confirm congenital corneal anesthesia and to differentiate from other possible neurotrophic keratitis causes. [ABSTRACT FROM AUTHOR]

Purpose: To report the feasibility and the successful outcomes of a pediatric neurotrophic keratopathy (NK) owing to congenital corneal anesthesia (CCA) treated with allogeneic serum eye drops obtained from the mother as the only therapy. Observations: A 18-month-old girl with generalized pain insensitivity presented with a large epithelial defect in the right eye (RE) and superficial punctate keratopathy (SPK) in the left eye (LE). Corneal sensitivity was completely absent in both eyes (BE). Peripheral serum eye drops obtained from the mother was prescribed and administered every 2 hours in BE. Two weeks after the beginning of treatment, compete healing of the epithelial defect in the RE was obtained, while the severity of the SPK in the LE markedly improved; in parallel, conjunctival hyperemia disappeared in BE. The treatment was continued over the course of 3 months with good tolerability and neither signs of recurrence of the epithelial defect nor other complications were noted. Conclusions and Importance: Allogeneic serum eye drops obtained from the mother are effective in treating pediatric NK owing to CCA. This treatment may represent a readily available and inexpensive option when topical nerve growth factor is unavailable or corneal neurotization is not feasible.

Purpose: Congenital corneal anesthesia (CCA) is a rare clinical entity that poses a diagnostic dilemma, particularly in the pediatric age group with very little literature on this. Accurate initial diagnosis, evaluation, early identification of risk factors, aggressive systemic workup, and appropriate therapy are paramount to prevent visual loss due to long-term complications of corneal anesthesia. The purpose of the study was to estimate and compare the corneal neural architecture using real time, in vivo confocal microscopy (IVCM) in patients with CCA as against a control population. Methods: This was a retrospective nonconsecutive, comparative clinical case series in a tertiary hospital in South India from June 2015 to December 2018. Methods: IVCM was accomplished in cooperative children in whom central cornea was relatively clear. The clearest three to five images from each eye were selected, and the nerves were analyzed for length, thickness, density, dichotomous pattern, and beading. Statistical analysis was done using Origin v7.0 (Origin Lab Corporation, Northampton, MA, USA). Results: In total, 15 eyes of 11 cases and 20 eyes of 10 controls were imaged. Measurements on corneal nerve density showed a significant difference (P = 0.0005), cases having a lower mean (3.85 ± 1.38 mm per mm2) compared to the controls (6.74 ± 1.75 mm per mm2). Measurements on corneal nerve length (P = 0.28), thickness (P = 0.45), and presence of beading (P = 0.97) and dichotomous pattern (P = 0.07) did not reveal a significant difference between cases and controls. Conclusion: There is a strong relationship between the functional loss (absent corneal sensation) and anatomical decrease (reduced subbasal nerve density) of corneal nerves in congenital corneal anaesthesia.

Neurotrophic keratitis is a rare degenerative disease of the cornea that can lead to corneal ulceration, scarring, and significant visual impairment. It most commonly occurs in adults and is rarely diagnosed in children. Congenital corneal anesthesia is an extremely rare condition that requires appropriate ophthalmologists’ attention in making diagnosis and treatment decisions. This condition usually presents in infancy or early childhood and is characterized by rare blinking rate, decreased tearing or a corneal ulcer that is unresponsive to treatment. In this case report, we describe a patient with multiple systemic and neurological disorders who presented to the ophthalmology department due to corneal erosion unresponsive to treatment. Brain magnetic resonance imaging confirmed bilateral trigeminal hypoplasia and the diagnosis of neurotrophic keratopathy due to bilateral congenital corneal anesthesia was made. The discrepancy between clinical signs and symptoms or treatment non-response in cases of corneal erosions should alert the ophthalmologists to suspect trigeminal dysfunction. MRI is the gold standard to confirm congenital corneal anesthesia and to differentiate from other possible neurotrophic keratitis causes.

Purpose: Congenital corneal anesthesia (CCA) is a rare clinical entity that poses a diagnostic dilemma, particularly in the pediatric age group with very little literature on this. Accurate initial diagnosis, evaluation, early identification of risk factors, aggressive systemic workup, and appropriate therapy are paramount to prevent visual loss due to long-term complications of corneal anesthesia. The purpose of the study was to estimate and compare the corneal neural architecture using real time, in vivo confocal microscopy (IVCM) in patients with CCA as against a control population.Methods: This was a retrospective nonconsecutive, comparative clinical case series in a tertiary hospital in South India from June 2015 to December 2018.Methods: IVCM was accomplished in cooperative children in whom central cornea was relatively clear. The clearest three to five images from each eye were selected, and the nerves were analyzed for length, thickness, density, dichotomous pattern, and beading. Statistical analysis was done using Origin v7.0 (Origin Lab Corporation, Northampton, MA, USA).Results: In total, 15 eyes of 11 cases and 20 eyes of 10 controls were imaged. Measurements on corneal nerve density showed a significant difference (P = 0.0005), cases having a lower mean (3.85 ± 1.38 mm per mm2) compared to the controls (6.74 ± 1.75 mm per mm2). Measurements on corneal nerve length (P = 0.28), thickness (P = 0.45), and presence of beading (P = 0.97) and dichotomous pattern (P = 0.07) did not reveal a significant difference between cases and controls.Conclusion: There is a strong relationship between the functional loss (absent corneal sensation) and anatomical decrease (reduced subbasal nerve density) of corneal nerves in congenital corneal anaesthesia. [ABSTRACT FROM AUTHOR]

Abstract: Congenital corneal anesthesia is a rare clinical entity that poses a diagnostic dilemma, particularly in the pediatric age group. The sensory deficit may be confined to the cornea, or extend to other divisions of the trigeminal nerve. The sensory deficit may occur as an isolated abnormality, as part of a complex neurological syndrome, or it may occur in association with multiple somatic abnormalities and congenital insensitivity to pain. This condition usually presents between the ages of 8 to 12 months. Poor vision, photophobia, conjunctival injection, and corneal ulceration in the absence of pain and distress in a child should alert the clinician to the possibility of anesthetic cornea. In the early stages of presentation, punctuate keratopathy is the main feature, which may progress to non-healing persistent corneal epithelial defects. This stage may progress to acute corneal lysis and perforation. In most patients, conservative approaches such as copious lubrication, prevention of self-harm and cautious use of bandage contact lenses are effective in preventing progressive corneal damage. Tarsorrhapy is effective in promoting epithelial healing and permanent lateral tarsorraphy may prevent further development of epithelial defects. Amniotic membrane graft may be considered in order to improve epithelial healing. Corneal grafts carry a poor prognosis. Accurate initial diagnosis, evaluation, and proper management are paramount to prevent visual loss due to long-term complications of corneal anesthesia. This review of the literature outlines the problems and approaches in diagnosis, evaluation, and management of this rare condition [Copyright &y& Elsevier]