Mutilating eye surgery involves depriving the eyeball of its contents, or completely removing it from the orbit with or without its appendages. Ophthalmologic mutilating surgery is determined by evisceration, enucleation and exenteration [1,2,3]. Enucleation involves separating all extraocular muscles and resection from the optic nerve [4]. Evisceration involves removing eye contents, while the scleral shell remains connected to the extraocular muscles [5,6]. The exenteration involves the total withdrawal of the globe from all the soft tissues of the orbit [7]. 

Eye conditions, which require the indication of mutilating surgery, are in a great number and very different [8,9] in both adult and children.

Mutilating surgery always generates a psychological shock, because losing an organ as precious as the eye is not easy to manage especially when it is a child. It is an irreversible procedure. In addition to the clinical indication, the doctor must take into account the visual potential of the eye, the potential for complications and the patient's psychological state. So, decision-making for its implementation strongly involves the patient and his legal representatives informed consent. 

Our study aimed at describing epidemiological characteristics of mutilating surgery in 0-16-year old children in the pediatric ophthalmology department of IOTA.

This was a retrospective descriptive study on the case history of children aged 0 to 16 years who underwent mutilating surgery in the pediatric ophthalmology department of IOTA (the only available pediatric ophthalmology center in the country) between January 1, 2014 and December 31, 2018 (5 years). Data included age, gender, diagnosis, operated eye, type of anesthesia, type of surgery, and prosthesis fitting. The agreement from IOTA management was obtained before the study was carried out. Data analysis was performed with EPI INFO TM 6.04 software. 

During the period, 106 eyes of 105 children were operated through mutilating surgeries out of a total of 1405 procedures, with a frequency of 7.55%. Mutilating surgery involved the two eyes  in  only one case (bilateral retinoblastoma). The right eye was affected 58.49% (n = 62). The average age was 4.02 years with extremes of 1 and 16. Male children were the most frequent with 53.33% (n = 56). Sex-ratio was 1.14. Indications for mutilating surgery were dominated by oculo-orbital tumors (including retinoblastoma) at 60.38% (n = 65). (Table 1). Retinoblastoma alone accounted for 54.72%. General anesthesia was performed in 96.22% (n = 102) of the cases. Enucleation was the commonest with 64.15% (n = 68). It was bilateral in one case in a child with bilateral retinoblastoma with an increased risk of metastasis. The exenteration was performed in 2 cases. Evisceration accounted for 33.96% (n = 41). Eye trauma was the main etiology of these evisceration with 58.33% (n = 21). (Table 2) There were no intraoperative complications. An ocular prosthesis was fitted in 75.47% of the cases.

The frequency of mutilating surgery in our study was 7.5%. This rate is higher than that of other studies but carried out in a non-age specific population. Indeed, Pandey, P. R. [10] in Nepal, Eballé et al., [11] in Cameroon, reported frequencies 1.4% and 1.6% respectively. Mutilating surgery is relatively common in children, especially in our study, which took place in a pediatric ophthalmology department. Other studies in the general population in China [12], in Cameroon [11] found predominance in the age group 0 to 10 years. Our average age was 4.02 years showing the prevalence of malignant tumors in this case retinoblastoma in our countries where treatment is late stages, leaving only less chance of conservative treatment. As in our study, Vonor, K. et al., [13] in Togo and Ibanga A [14] reported a high male frequency which was related to the frequent exposure of boys to trauma, but in our study the tumor causes predominated. The causes of surgery in general may vary per country. In fact, according to some authors, tumors and pain in the blind eye are the most common indications for mutilating surgery on the globe in developed countries, while infectious causes and trauma are most common in developing countries [11,15,16].

In children, as in our study, most series have shown that the eye tumors first represented by retinoblastoma are the most common causes of mutilating surgery [17,18]. 

Retinoblastoma is a malignant tumor developed linking to retinal cells and specific to children. Early diagnosis is required for the conservation of the eyeball. A study in Bamako showed that almost two thirds of children with retinoblastoma reached to the diagnosis at the extraocular stage [19]. This situation explains the high frequency of retinoblastoma among eye tumors. So, an adapted diagnostic and management program for retinoblastoma still remains a challenge in our countries. General anesthesia is best suited for surgery in little children. It allows easy surgical procedures without any resistance from the child. In addition, many parents are reluctant to local anesthesia because it does not provide perfect analgesia. Despite these findings, peribulbar anesthesia was performed in 3.78% of the cases; all cases of evisceration but after informed consent of the child’s parents. Enucleation was the most performed surgical procedure with 64.15%; according to some authors, evisceration is the commonest mutilating ocular intervention [20]. This is true in contexts where the diagnosis of ocular pathologies is made early. Enucleation is mainly carried out for advanced malignant tumors in the absence of any other therapeutic means [21] as in our developing countries where a lot of effort remains to be provided in the prevention and early management of ocular pathologies of the child. The population must be made aware of the need to seek the help of a health center as soon as possible in the event of any morphological or functional, congenital or acquired abnormality observed in a child. The exenteration is indicated in malignant tumors with a very high invasion potential at least to preserve the vital prognosis. As in most studies, it is less practiced compared to evisceration and enucleation. Evisceration accounted for 33.96% in our series. Trauma (58.33%) and eye infection (41.67%) were the main etiologies (%). Poor management of infections in children and especially the late admission at health facilities would largely explain this result. Childhood is an often difficult period; children play violent games thus exposing themselves to physical attacks which unfortunately can affect the eye. Trauma is a significant cause of mutilating surgery; 24.53% in our study. Trauma was more frequently the cause of mutilating surgery in young subjects in several studies [22,23,24].

Table 1: Distribution according to the etiology of mutilating surgery

Table 2: Distribution of etiologies according to the type of mutilating surgery

Rehabilitation after mutilating surgery is a great challenge, especially in a particular population of children. Parents helpless facing the functional and anatomical loss of their child's eye are most often ready at any cost to improve their physical appearance. Over two-thirds of our children (75.47%) have benefited from prosthesis. IOTA has a prosthesis department managed by an ocularist thus facilitating access to this equipment often considered as a luxury one.   

Mutilating surgery occupies a relatively frequent place in pediatric ophthalmological surgery. Being an irreversible act, its implementation must follow a well informed decision strongly involving the legal representative of the child if possible the child himself. Early diagnosis and appropriate management of childhood eye tumors are the best means of prevention.

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