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33 Prophylactic Procedures inPediatric Surgery
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393
after prophylactic resections [28, 29]. In children, it has been found that MD-related complications are higher in children younger than 8years of age than older children [30].
In conclusion, it has been recommended that leaving an incidental MD in situ is mandatory, which is identied on imaging studies. Asymptomatic MD found during abdominal exploration in early ages of life, one should resect the MD.In young adults <50years of age, espe­cially men, MD’s longer than 2cm, and with an associated anomaly on palpation, resection must be carried out. In elderly patients, no resection is recommended in the appearance of a normal­appearing MD.
33.4 Prophylactic Surgery
forIntestinal Malrotations
Intestinal malrotation is a rare congenital anom­aly of intestinal position with an incidence of 3.9 per 10,000 live births according to the report of the Centers for Disease Control, where other reports an incidence of 0.2–1% in the pediatric population [31, 32]. Malrotation is a result of an error in intestinal rotation and xation of the intestinal mesentery. The duodenojejunal junc­tion lies at the right side of the midline close to the ileocecal valve causing a relatively narrow mesenteric stalk. This anatomical decit may cause midgut volvulus, followed by ischemic bowel, possible short gut syndrome, and death.
Most of the patients are symptomatic under the age of 1, where 50% of patients are in the newborn period [33]. The sudden onset of the symptoms after volvulus is typical at this age with bilious vomiting, abdominal distension, abdominal tenderness, peritonitis indicating per­foration, and rectal bleeding indicating bowel ischemia at later phase. In later childhood, the symptoms become more atypical like cyclic vomiting (often non-bilious), recurrent abdomi­nal pain, and failure to thrive [34].
The surgical procedure for malrotation was described by Ladd in 1936 as detorsion of the volvulus, division of the Ladd’s bands, widening of the mesenteric root, and positioning the small bowels to the right and the large bowels to the left
quadrants of the abdomen [35]. Some authors added prophylactic appendectomy to this original procedure to avoid misdiagnosis of a left lower quadrant appendicitis, where others have dis­couraged this maneuver to avoid associated com­plications [36]. Although the laparoscopic repair of volvulus in a neonate was described by van der Zee etal. in 1995, this approach is still not per­formed routinely in infants and children as the rst choice [37, 38].
The treatment of malrotation in symptomatic children has been well established, but the treat­ment of malrotation in an asymptomatic child or malrotation diagnosed incidentally remains con­troversial regarding the need and the timing of the operation. Some clinical conditions may be associated with malrotation or nonrotation like congenital diaphragmatic hernia (CDH), ompha­locele, gastroschisis, congenital heart disease (CHD), and heterotaxy syndrome (HS), for which prophylactic Ladd’s procedure remains a matter of debate [39].
Surgical correction of malrotation with or without symptoms is warranted for infants because of the high risk of volvulus at this period [40]. It is also not clear how much the risk of vol­vulus decreases within years of age because there are also reported patients’ malrotation with vol­vulus in age 70s [34]. In the report of Prasil etal. (2000), they have the charts of patients operated on for malrotation in means of age (<2 or >2years old). They have found that 17.2% of patients older than 2 years have volvulus and recom­mended surgical attention in all patients regard­less of age [41]. Malek etal. (2006) designed a model of the probability of a Ladd’s procedure and reported that most patients with malrotation will undergo this operation in childhood. They recommended careful observation of adults with asymptomatic malrotation for unusual or unexplained abdominal discomforts associated with a partial or total volvulus [42]. In a recent review of American Pediatric Surgical Association on asymptomatic malrotation, they have stated that upper gastrointestinal studies remain the best imaging modality for malrota­tion, but even ultrasound cannot be used to rule our malrotation or volvulus, and the narrow­based mesentery cannot be determined with
394
G. Köylüoğlu and M. O. Öztan
imaging studies as a predictor of volvulus in the future. As a Grade C recommendation, they said to operate on asymptomatic patients who are “younger at age” without given a specic age [43].
In conclusion, it is a fact that labeling of any malrotation as “asymptomatic” is not reasonable because many patients have been thought of as asymptomatic-declared abdominal symptoms at carefully taken history. To prevent the cata­strophic results of midgut volvulus, prophylactic surgery for malrotation is recommended in all patients at low risk for postoperative morbidity or mortality.
33.5 Prophylactic Fecal Diversion
Fecal diversion in children is used for several aims; it is mainly used to divert the fecal stream for decompression, for emergency salvage, and before the reconstructive correction of the lower colorectal anomalies.
After the initial management of the patient with a traumatic wound, it is crucial to achieve a proper infection control for the prevention of sep­sis and establish a good wound healing. In patients with severe full-thickness perineal and gluteal burns, open pelvic traumas, or colorectal traumas, fecal contamination may be prevented with a temporary-diverting colostomy [44–46]. It is also benecial for giving the child a favorable long-term functional outcome by reducing the depth of the wound, facilitating wound care, and reducing the debridement frequency in the opera­tion room.
A protective colostomy in anorectal malfor­mation (ARM) is needed to avoid contamination before the denitive operation [47, 48]. An infec­tion and dehiscence is the most unwanted situa­tion after ARM repair because there is a greater risk of damaging the continence mechanism, and secondary procedure is much complex than the primary one [49]. In patients with at perineum, meconium-stained urine, bowel gas above the coccyx, and cloaca receive a diverting colostomy, preferably located at the descending colon [50]. The distal part of the stoma may be created as a
mucous stula to avoid prolapse [47]. After the denitive repair, the colostomy is closed after reaching the appropriate size of the anus.
Another group of patients, who need a fecal diversion, are the patients with inammatory bowel disease and familial adenomatous polypo­sis coli [51]. After initial subtotal or total colec­tomy in patients with ulcerative colitis and familial polyposis, it has been reported that a temporary loop ileostomy prevents anastomotic leak from the created ileal pouch and reduces overall complication rate [52]. In Crohn’s disease (CD) patients, the role of diversion is to allow healing of perianal disease and induce remission in refractory colonic and perianal CD [53, 54]. Although this approach has a high incidence of disease remission, intestinal-continuity restora­tion rates differ between 10 and 39% in various reports [53, 55].
33.6 Prophylactic Incidental
Appendectomy
In a patient without acute appendicitis ndings but undergoing laparotomy for other reasons, the appendectomy is called prophylactic incidental appendectomy (IA) [56]. However, planned appendectomy in absence of appendicitis or another surgical procedure is called elective appendectomy [57]. However, this surgical pro­cedure is a controversial issue because of poten­tial complications. An easy surgical procedure, no additional anesthesia, lower morbidity rate, and exclusion of difculties in diagnosing appen­dicitis in the future are the benets of the IA [58]. Prolonged operation time, increased morbidity, and transformation of the process from clean to clean-contaminated due to colonic ora are undesirable features of IA [58]. Besides, the appendix is increasingly being used in urological and biliary reconstructive surgeries and for colonic irrigation in bowel management [56]. Due to all these advantages and disadvantages, the decision of whether to make IA or not has been considered more (Table33.1) [56].
Considering the developments in the use of appendix for reconstruction in recent years, the
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Table 33.1 Summary of recommendations
Condition Recommended Not recommended Malrotation Yes, to prevent future
Congenital diaphragmatic hernia Yes, to prevent later atypical
Oncologic surgery Yes, neutropenia may hide the
Anorectal malformations and exstrophy
Neurological diseases, ventriculoperitoneal shunt, hydrocephalus, etc.
Cystic brosis Maybe yes in the cystic brosis,
Hirschsprung’s disease and chronic constipation
Incidentally discovered fecalith Yes, it may be, depending on
Biliary atresia and choledochal cysts
misdiagnosis [
appendicitis [
symptoms of peritonitis [
56] Due to high rate of intestinal/bladder
Not [
Not [56] Due to high rate of intestinal/bladder
in order to irrigate meconium
66]
[ Not [56] For antegrade continence enema in bowel
the patient’s easy access to healthcare[56]
67] Due to the possibility of its use in biliary
Not [
59]
62]
64]
It may require for bowel/bladder incontinence and a long-term gastrostomy
60, 61]
[ When a patch is required for repair, due to
increased risk of contamination [63] Appendectomy during Wilms’ tumor
surgery does not change the postoperative complication rate [
incontinence [
incontinence [
Unclear in other circumstances [
management [56]
reconstruction [67]
56]
56]
395
65]
56]
comorbid conditions of the patient should be considered before performing IA. Advances in minimally invasive surgery make the decision even more difcult. The long-life expectancy in children, the additional medical history, and the possibility of using appendix as a tubular channel should be rigorously evaluated during the decision- making process [56].
33.7 Prophylactic Cholecystectomy
Prophylactic cholecystectomy (PC) is dened as the removal of the non-diseased gallbladder when laparotomy is being undertaken for other reasons.
Choledochal cysts cause many complications, such as ductal stricture, stone formation, cholangi­tis, rupture, and secondary biliary cirrhosis. In addition, the risk of choriocarcinoma, pancreas, and gallbladder cancer risk increase 20–30 times compared to the normal population [68]. While the incidence of malignancy in cysts is 0.4% under the age of 18, it reaches 11% in all adults and 38%
over the age of 60. The presence of abnormal pan­creaticobiliary junction (APBJ) in cysts increases the risk of malignancy [
69]. APBJ alone increases
the risk of pancreatic and biliary malignancy, even without cyst or ductal dilatation. Especially gall­bladder cancers are common in APBJ patients without cysts. Prophylactic cholecystectomy is recommended in these patients [70].
The incidence of gallstones has increased in patients with short bowel syndrome (SBS). In one study, gallstones were detected in 4 of 24 patients who underwent ileal resection in the neonatal period [
71]. This rate rises up to 44% in
adult ages [72]. Cholelithiasis causes more com­plications in patients with SBS compared to the general population. Approximately, half of the patients with SBS go to recurrent laparotomies. Prophylactic cholecystectomy is a reasonable procedure to be performed safely and without causing any complications [73]. In general, urgent intervention requirements may be required because patients with SBS undergo multiple operations. In such cases, cholecystectomy may not be recommended. Also, the issue of whether prophylactic cholecystectomy causes intestinal
396
G. Köylüoğlu and M. O. Öztan
dysfunctions and hepatic diseases in patients with SBS has not yet been claried [73].
Splenectomy is recommended for the treat­ment of hereditary spherocytosis (HS) in chil­dren. During the same operation, cholecystectomy should be performed if there are stones in the gallbladder. If there is no stone, prophylactic cho­lecystectomy is not recommended. In a study, stone formation was not observed in the follow­ up of patients without cholelithiasis during sple­nectomy [74].
33.8 Prophylactic Splenectomy
Prophylactic splenectomy can be dened as the removal of the spleen, which is actually disease­free, which exaggerates one or more of its normal functions in order to contribute to the treatment of some special hematological diseases in chil­dren. Splenectomy is indicated in patients with (HS), auto hemolytic anemia, and thalassemia because in any case, the spleen causes excessive hemolysis [75].
HS is the most common cause of hemolytic anemia, although it is rarely seen. It occurs due to a defect in the red blood cell membrane. Prophylactic splenectomy is effective in improv­ing anemia in patients with severe hemolysis. Partial splenectomy may be preferred in children under the age of 6. Compared with total splenec­tomy, partial splenectomy also has a lower risk of sepsis of encapsulated bacteria. If necessary, a total splenectomy can be delayed until after the age of 6 [76].
Acute splenic sequestration crisis observed in sickle cell disease is a serious complication that requires prophylactic splenectomy. In the past, splenectomy was not recommended before 5 years of age because of fear of postsplenec­tomy sepsis. Recently, reports are indicating that splenectomy can be done at an earlier age with appropriate vaccination and prophylactic antibiotics [77].
Also, prophylactic splenectomy is performed since the spleen is responsible for platelet destruction as in idiopathic thrombocytopenic purpura. Splenectomy is usually a suitable option
for a small percentage of chronic ITP patients with severe thrombocytopenia and hemorrhagic symptoms and requiring repeated pharmacologi­cal interventions. Although splenectomy is effec­tive in most patients, rates of splenectomy among children with ITP have decreased signicantly since the early 2000s, especially among children under 5years of age [78]. While the cause of the decline is not clear, it may be associated with an increased availability of effective second-line treatments.
33.9 Prophylactic Surgery inPediatric Surgical Oncology
Prophylactic surgery in children with certain can­cer predisposition syndrome may decrease the incidence of malignancy. It is superior due to low complication rate and high cost-effectiveness compared to conventional screening and routine examinations [79].
33.9.1 Familial Adenomatous Polyposis
Colorectal cancer is seen in 1/471 rate in familial adenomatous polyposis (FAP) patients before 20 years of age [80]. The risk of developing cancer during life is approximately 100%. In general, three prophylactic surgical methods are used: total proctocolectomy with ileal pouch-anal anastomosis (IPAA), total abdominal colectomy with ileorectal anastomosis (IRA), and procto­colectomy with ileostomy [81]. The timing and age of prophylactic colectomy are uncertain because the data are limited in terms of surgical results in children. In classical FAP, the timing of surgery is done between the ages of 15 and 25, depending on age, compliance, presence of dys­plasia/cancer, genotype, and the number of ade­nomas [81]. The most appropriate age should be determined according to the psychological com­pliance of the young patient to aggressive sur­gery. IRA or IPAA options are determined according to the number and/or size of polyps,
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397
postoperative follow-up compliance, and fami­ly’s common sense. Patients undergoing IRA develop 30% rectal cancer until age 60. IPAA is more advantageous in terms of optimal bowel control. As can be seen, discussions on the tim­ing, size (extend) of surgery, and the types of reconstruction continue in pediatric FAP man­agement [79].
33.9.2 Medullary Thyroid Cancer
In children, the thyroid gland is particularly sen­sitive to irradiation and carcinogenesis. Unlike adults, thyroid cancers show regional lymph node and distant organ metastasis at the time of diag­nosis. Despite these characteristic features, thy­roid cancers in childhood have a good prognosis. Medullary thyroid carcinoma (MTC) in children is detected either as a solitary nodule or due to the presence of MTC in one of the family members and typically as part of MEN2A or MEN2B.
Total thyroidectomy performed with central neck dissection in children with RET gene muta­tion is the standard prophylactic surgical approach. Early total thyroidectomy seems to be effective in preventing the development of MTC in the long term [82]. However, due to insuf­cient data, performing prophylactic surgery, especially based on RET gene positivity, espe­cially in the early (under 2 years) period may cause unnecessary thyroidectomies [83]. Compared to adults, thyroidectomy complica­tions are much higher in children, and especially infants. In very young children, it is very difcult to distinguish parathyroid glands from surround­ing tissues during surgery. Although the compli­cation rate of experienced surgeons is quite low, postponing thyroidectomies under the age of 2 should be considered [79]. However, the American thyroid association (ATA) has revised the MTC guidelines on disease management [84]. Today, the decision on the timing of prophy­lactic thyroidectomy is not based solely on DNA analysis. In addition, clinical data and most importantly, basal or stimulated serum calcitonin level is used. The ATA revised guidelines identi­ed the highest-risk, high-risk, and moderate-risk
groups for prophylactic thyroidectomy in chil­dren. In those at the highest-risk group, thyroid­ectomy should be performed in the rst year of life, even in the rst months of life. Prophylactic thyroidectomy should be performed at the age of 5 or earlier considering the serum calcitonin lev­els in the high-risk group. Timing in the medium­risk group should be based on high serum calcitonin levels. It can be extended for several years or even 10years with 6-month or 1-year evaluations [79].
33.10 Conclusion
As can be understood from the abovementioned diseases, when performing a prophylactic surgi­cal procedure, the benet-harm balance, the risks that may develop later in life, the psychological conditions of the patients and their parents, and the age group to be applied should be carefully evaluated.
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Prophylactic Surgery
Данная книга находится в списке для перевода на русский язык сайта https://meduniver.com/
forNeurosurgical Pathologies
NurullahYüceer
34
34.1 Introduction
Neurosurgical pathologies, in which the most prophylactic surgery is performed, are brain tumors, cerebrovascular diseases, craniospinal injuries, congenital and degenerative diseases. As a neuroradiological examination in patients sus­pected of intracranial pressure increase as a result of clinical evaluations, the most commonly used diagnostic methods today are magnetic reso­nance imaging, computed tomography (CT) and angiography with direct radiographs. Timing of prophylactic surgery differs among these pathol­ogies. It is preferred that the timing of prophylac­tic surgery is limited to days in cerebral aneurysms. The timing of prophylactic surgery in brain and spinal tumors can be limited to weeks. The prophylactic surgery timing in congenital and degenerative patients can be within months. Neurological examination is normal in the major­ity of patients scheduled for prophylactic surgery. The results are very good in these patients who underwent prophylactic surgery [1, 2].
N. Yüceer (*) Department of Neurosurgery, School of Medicine, İzmir Katip Çelebi University, İzmir, Turkey e-mail: nurullah.yuceer@ikc.edu.tr
34.2 Increased Intracranial
Pressure andHydrocephaly
Increased intracranial pressure reects changes in the brain, cerebrospinal uid (CSF) and blood volume that make up the intracranial structures. Intracranial pressure is 10–15 mmHg in adults and older children, 3–7mmHg in young children,
1.5–6 mmHg in newborns. In patients with
increased intracranial pressure, headache, nausea and vomiting and bilateral papillary edema are typical. Hydrocephalus is an abnormal, usually progressive accumulation of CSF within the ven­tricular system that distends the ventricles and often raises. The main causes that can lead to hydrocephalus are congenital causes, such as ste­nosis of the aqueduct of Sylvius or atresia of the foramina of Magendie and Luschka, tumors, intraventricular hemorrhages, infections, vascu­lar pathologies, traumas. Prophylactic treatments are applied to the causes of hydrocephalus to pre­vent possible complications [3–5].
In intracranial space-occupying lesions, changes are observed in these intracranial structures. Not only the growth of intracranial space- occupying lesions, but also increases in intracranial blood vol­ume/or cerebrospinal uid lead to increases in intracranial pressure. Intracranial pressure increase usually causes headache, nausea, vomiting and bilateral papillary edema in patients. If patients with increased intracranial pressure are not treated,
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2021 O. N. Dilek et al. (eds.), Prophylactic Surgery, https://doi.org/10.1007/978-3-030-66853-2_34
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N. Yüceer
Fig. 34.1 Top row: Third ventricular colloid cyst causing acute hydrocephalus in a 36-year-old woman. The patient was brought to the emergency room with a loss of con­sciousness. CT scan (a) and T1- and T2-weighted axial MRIs with contrast demonstrate third ventricular colloid cyst causing acute hydrocephalus. The patient was oper­ated on urgently. Her postoperative neurological examina­tion was normal. Control postoperative CT scan was normal (d). Bottom row: Left frontal glioblastoma in a 55-year-old man. The patient presented with the com-
neurological decits, and various herniation syn­dromes, which lead to changes in consciousness, develop [1, 2, 6] (Fig.34.1).
34.3 Brain Tumors
According to the classication made by the World Health Organization, brain tumors are divided into seven subgroups: neuroepithelial tumors, tumors of the cranial-spinal nerves, tumors of the meninges, lymphoma­hematopoietic tumors, stretching-cell tumors, sellar tumors and metastatic tumors (Fig.34.2). Approximately, 40% of brain tumors are glial tumors. While 60% of brain tumors in adults show supratentorial location, in children, the same rate is seen in the posterior fossa.
plaints of headache and speech disorder that had been present for a month. In his examination, dysphasia was detected. CT scan (a), T1- and T2-weighted MRIs (b, c) examinations revealed a tumor in the left frontal that caused edema. Gross total tumor excision was performed. Postoperative CT scan (d) was normal. The patient’s speech improved after the operation. Pathology examina­tion conrmed gliobastoma. Radiotherapy and chemo­therapy were performed
Medulloblastoma is the most common malignant tumor in the posterior fossa that does not have a glial origin in children [7].
It is well known that low-grade glial tumors rise to higher-grade tumors (Fig.34.2). Especially in diffusion and spectroscopic examinations using magnetic resonance imaging, preventive surgical interventions can be recommended to patients considering that there may be an increase in high-grade tumors in patients with low-grade glial tumors [8–10]. The same can be considered in benign tumors, such as meningioma (Fig.34.3) [11]. Hemangioblastomas are life-threatening tumors that tend to bleed and grow [12].
Prophylactic surgeries are performed in patients with acromegaly (Fig. 34.4), Cushing disease and those who are not hormone secretar­ies, and who are at risk of vision loss with chiasm
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