Anal canal duplication in infants and children--a series of 6 cases.

The authors present a series of six anal canal duplications (ACD), duplications of the alimentary tract located along the posterior side of the anal canal, with a perineal opening just behind the anus. Five asymptomatic duplications were diagnosed before the age of one year, by simple perineal inspection. A twelve-year-old girl presented with perineal and anal pains and diarrhoea. Fistulography revealed a tubular structure in five cases and a cystic structure in one case, behind the normal anal canal, in one case communicating with it. A presacral sacrococcygeal teratoma was found in two children and in one case it was visualised by preoperative US in an infant with a lumbosacral myelomeningocele. Surgical excision was performed by a perineal approach in 5 cases, by a combined sacral and perineal approach in the last case, because of the associated teratoma. Non-invasive preoperative investigations, consisting of a pelvic X-ray, US examination, barium enema and fistulography, are sufficient in most cases; MRI is reserved for special indications. Surgical treatment restores a normal perineal aspect, without sequelae, and avoids complications like those described in other types of digestive duplications: infection, ulceration, bleeding, malignant changes during later adult life. Associated anomalies are frequently described in the literature, especially presacral tumours (16%) and anorectal malformations (21%); they can influence the management, the surgical approach and the functional prognosis.     

Anal canal duplication: a retrospective analysis of 12 cases from two European pediatric surgical departments

Anal canal duplication (ACD) represents an extremely rare intestinal congenital anomaly of unknown origin. Usually evidenced within 2 years of age, nearly 45% of reported cases present associated malformations such as presacral mass, anorectal malformation (ARM) and genitourinary anomalies. The confirmative diagnosis is istopathological, with evidence of an anal mucosal lining (squamous ± transitional epithelium), surrounded from a smooth muscle coat and anal glands. We review a conjoined experience from two European pediatric surgical departments. From 1970 to 2005, 12 patients were observed, seven in Pescara, Italy (1997–2005), five in Barcelona, Spain (1970–2004)—mean age at diagnosis 17.8 months, range 0–60; M:F = 1:11. Clinical presentation, diagnostic-surgical approach, and complications were reviewed. According to clinical presentation, patients could be divided in three age groups: asymptomatic (mean age 4.8 months, six patients—one with an associated complex genitourinary malformation, one with a presacral mature teratoma, one with ACD evidenced hysthologically on a retroanal mass removed during the correction of an ARM), mildly symptomatic—constipation, mucous discharge (mean age 29.2 months, four patients—one with associated presacral ependymoma and intestinal neuronal dysplasia type B, one with presacral mass) and complicated—perineal abscess, recurrent fistula (mean age 34 months, two patients). In 11 cases a perianal orifice was evident (ten posteriorly located). The pelvic-MRI was the preferred diagnostic tool in Pescara (5/7, with presacral mass in two patients), fistulography in Barcelona (5/5), where one presacral mass was discovered intraoperatively. Eleven patients underwent surgical removal of the ACD (five perineal approach, five posterior sagittal approach, and one PSARP). Histopathological findings confirmed the diagnosis in operated cases (11). The parents of the male patient denied the consent to surgical treatment. The only major post-operative complication was a sphincteric insufficiency (one case), surgically treated. When facing a perianal orifice, attention should be paid to ACD, particularly in female patients with coexistent genitourinary or intestinal malformations. Pelvic US and MRI are the gold standard to evidence the not rarely associated presacral mass. Surgical early removal (mucosectomy or perineal/posterior sagittal approach, depending on length of ACD and associated presacral mass) is warranted, also in asymptomatic patients, because of the risk of inflammatory complications and cancer (the latter reported in literature in adults).     

Familial triad of anorectal,sacrococcygeal, and presacral anomalies that includes sacrococcygeal teratomas

We present two Australian families with the triad of anorectal, sacrococcygeal, and presacral anomalies. In one family four members had sacral deformities, three of which were associated with presacral masses and anal stenosis. The presacral masses were: one meningocoele combined with a teratoma; one teratoma; and the third remains undiagnosed and unresected at the patient's request. In the second family, one child had an infected presacral teratoma with a small sacral defect and the father had a similar sacral defect with computed tomographic evidence of a presacral meningocoele and teratoma. The presence of anal stenosis and constipation with a sacral defect should raise the possibility of a presacral mass. The mass may take the form of a meningocoele, teratoma, enteric cyst, or any combination of these. Once detected, other family members should be investigated.     

Sacrococcygeal teratoma in Northeastern Nigeria: 18-years experience

Although sacrococcygeal teratomas are rare, there have been several reports from various parts of Nigeria, outside the Northeast region. This report reviews the experience with this tumour from the northeastern part of Nigeria. A retrospective study of 21 children with histologically confirmed sacrococcygeal teratoma managed in 18 years. Over the 18-year-period, 21 histologically confirmed cases of sacrococcygeal teratoma were seen—80% were girls. Thirteen (60.9%) presented during the neonatal period. Eighteen patients presented with benign sacrococcygeal teratoma while three patients and a recurrence from an initial benign tumour had malignant sacrococcygeal teratoma. All malignant tumours had a significant presacral component. There were associated congenital malformations in 3 (14.3%) of the patients. All the benign tumours were resectable. Using the American Academy of Pediatrics (Altman’s) classification (Altman et al., J Pediatr Surg, 9:389–398, 1974), 13 patients were type I, 5 patients type II, 3 patients type Ill and none was for type IV. The commonest post-operative complication was wound infection in six patients. Others were wound dehiscence in three and post-operative diarrhoea in two patients. Three deaths were recorded in this series. The size of mass greater than 5 cm did not correlate with the incidence of post-operative complications (P=0.367). The post-operative follow-up was very poor; but there was a recurrence in a girl, two-years after the initial surgery. Our study revealed that most of our patients with sacrococcygeal teratoma present early to hospital with benign lesions and the immediate post-operative results were excellent. However, the follow-up was poor as such long-term post-operative results are difficult to study. It is to be recommended that sacrococcygeal teratoma presenting early to hospital should have prompt surgical intervention.     

Long-term outcomes of surgery for malignant sacrococcygeal teratoma: 20-year experience of a regional UK centre

Background  The timing of surgery for malignant sacrococcygeal teratoma is controversial. The long-term outcomes and complications of surgery for this rare tumour are presented. Methods  All cases of malignant sacrococcygeal teratoma in the 20-year period 1987–2006 were identified and the case notes retrieved. The age at diagnosis, investigations, presentation, type of surgery, early complications, recurrence rates, long-term complications and outcomes were recorded. Results  Twelve patients (three males, nine females) were identified. Mean age at presentation was 20.8 months (range: 12–39 months). All had the Carboplatin–Etoposide–Bleomycin chemotherapeutic protocol. The average time of follow-up was 10.6 years (range: 1–17 years). Ten patients had excision of their tumours following chemotherapy, whilst two patients had excision prior to chemotherapy. Two patients had recurrence of their tumours. There was one death (8%), which was due to disseminated metastasis. The other 11 children were all well at the last follow-up. Conclusion  Surgery for malignant sacrococcygeal teratoma is safe and has a low complication rate. The long-term outcomes are favourable with minimal side effects.     

Presacral teratoma presenting with congenital urinary ascites

Congenital teratomas occur most frequently in the sacrococcygeal region. Most grown into a large perineo-sacral swelling that is conspicuous externally. Infrequently, the neoplasm is contained almost entirely within the pelvis in the presacral space. Congenital urinary ascites is observed in patients with obstructive uropathy; posterior urethral valves in a newborn is one of the most prominent causes of urinary ascites. We report a case of presacral teratoma leading to rupture of the urinary bladder due to outflow obstruction and causing urinary ascites. The ascites was drained, the bladder was repaired, and the teratoma was successfully excised. A review of the literature did not reveal any similar case. Accepted: 12 November 1996     

Normal anorectal musculatures and changes in anorectal malformation

We investigated the anorectal musclulature in normal children and anorectal malformations (ARM) to evaluate its role in bowel control mechanism. Pelves of 50 neonates died of ARM-unrelated diseases and 16 patients with anorectal malformations (8 high, 5 intermediate, and 3 low ARMs) were dissected and analyzed. Normal anorectal musculature was divided into three muscular tubes: the internal sphincter tube (IAST), longitudinal muscle tube (LMT) and transverse muscle tube (TMT). The LMT came from the outer longitudinal smooth muscle fiber of the rectum and the striated muscle fiber of the levator ani, and the TMT composed of the puborectalis and the external anal sphincter. However, in ARM, the IAST was absent and the LMT, the center of the sphincter muscle complex, was only from the levator ani and could be divided into the pelvic portion and the perineal portion. The former, from the upper rim of the puborectalis to the bulbar urethral, became narrowed and dislocated anteriorly near to the posterior urethra in high ARM and rectal pouch in intermediate ARM. The latter, below the bulbar urethra to the anal dimple, was fused to a column both in high and intermediate ARM. The columnar perineal LMT run downwards and then split, penetrated the superficial part of EAS and terminated at the deep aspect of the skin, to form the anal dimple, which represents the center of the perineal LMT from the perineal aspect. The length of the LMT was longer in high and intermediate ARM than the normal neonate. The columnar perineal LMT and narrowed pelvic LMT could be possibly identified by laparoscopic and perineal approaches retrospectively and widened to allow the passage of the rectum through. The anorectal musculature in ARM is composed of agenesic LMT and TMT and the narrowed LMT gives anatomical evidence of the center, where the neorectum should pull through.     

Sacrococcygeal tumors in infancy and childhood; a retrospective histopathological review of 85 cases

This study retrospectively examines the spectrum of sacrococcygeal tumors reported in a tertiary paediatric pathology department during a 15-year period. There were 85 sacrococcygeal tumors identified in total, including 79 (93%) sacrococcygeal germ cell tumors, of which 62 (78%) were benign, whereas 17 (22%) contained malignant yolk sac tumor elements. The median age at examination in cases with malignant elements present was significantly greater than in those with benign sacrococcygeal teratoma only (median 2 years, range birth--3 years versus median 1 week, range birth--10 years, respectively; p < .01). Of the 85 cases of total sacrococcygeal lesions 6 (7%) represented pathologies other than sacrococcygeal teratoma, including one case each of neuroblastoma, ganglioneuroma, myxopapillary ependymoma, primitive neuroectodermal tumor, lipomatous tumor, and unclassifiable inflammatory tumor. Of these 6 cases 3 were malignant (50%) compared with 17 of the 79 cases of sacrococcygeal germ cell tumors (22%; Z =1.59, p = .08). The median age in the group of non-germ-cell sacrococcygeal masses was 3 years (range 5 months to 13 years).     

Post-operative strictures in anorectal malformation: trends over 15 years

Aim

For decades, paediatric surgeons have employed the standard posterior sagittal anorectoplasty (PSARP) approach to deal with patients with anorectal malformations (ARM). In recent years, we noted an apparent increase in the incidence of anal stricture after surgical repair of ARM following the introduction of laparoscopic pull-through and techniques aiming to preserve the internal sphincter—the internal sphincter sparing approach (ISSA). We decided to analyse our data to find out if these new trends had added to the problem of post-operative strictures.

Methods

All patients with ARM at our institution from January 2000 to December 2015 were identified. A retrospective case note review was carried out. Data collected included patient demographics, type of ARM, operative details, and post-operative outcomes.

Results

114 patients were identified. Ten patients were excluded. Of the remaining 104 children, 48 (46%) were female. Median age was 8.3 (range 1.2–16.8) years. Types of ARM were as follows: perineal fistula (15 patients), anterior stenotic anus (12), imperforate anus without fistula (10), vestibular fistula (32), rectourethral (bulbar) fistula (11), rectourethral (prostatic) fistula (14), rectovesical fistula (7), and cloaca (3). Twenty-seven patients with a perineal fistula or anterior stenotic anus underwent perineal procedures that were variably described by the different operating surgeons. The majority (15 patients) had an anoplasty, 5 had anal transposition, 5 had limited PSARP, and 2 patients had ISSA. Two patients with a cloacal anomaly underwent open cloacal reconstruction. Of the remaining 75 patients, 45 had a PSARP approach, 6 had a laparoscopic-assisted pull-through, and 18 had ISSA. Four girls with vestibular fistula had anal transposition and two boys with imperforate anus without fistula had anoplasty. 15 (14%) children developed anal stricture. Stricture incidence differed according to operation type. PSARP was the most commonly performed procedure, with only 6% developing a stricture. In contrast, 30% of ISSA patients and 50% of children who had laparoscopic pull-through developed a stricture. Strictures also occurred in 11 and 12% of children having anal transposition and anoplasty, respectively.

Conclusion

The laparoscopic-assisted pull-through involves tunnelling the sphincter muscle complex. We found that often the tunnels were not wide enough, resulting in narrowing not just at the ano-cutaneous junction but also at the deeper level. 50% developed strictures. We have modified our technique by ensuring that the tunnels are generous enough to allow the rectum to be pulled through without any resistance. ISSA unfortunately resulted in 30% of our patients developing strictures. This approach, started in 2004, was, therefore, abandoned in 2013. The standard Pena’s PSARP, with or without a laparotomy, has stood the test of time. Any modification of this approach must be carefully thought through and audited meticulously. Strictures can cause significant morbidity, which may need several revisions, and the resulting redo anoplasties run the risk of sphincter damage, ironically which the newer modifications of ISSA were trying to conserve.

     

The outcome of solid tumours occurring in the neonatal period

Sixty-six solid neoplasms occurring in neonates treated at the Red Cross Childrens Hospital over a 34-year period (1957–1991) were reviewed and recalled for long-term follow-up (mean 10.4 years). Associated congenital abnormalities were rare, but chromosomal abnormalities were detected in 3 patients, one of which was familial. Teratomatous germ-cell tumours predominated, followed by neuroblastomas and soft-tissue tumours; 23 had malignant morphologic appearances and 43 were morphologically non-malignant. Seventy-nine per cent presented within the 1st week of life, 41% of these within the first 24 h. Although most sacrococcygeal teratomatous germ-cell tumours were benign, malignancy was present in 2 patients (1 of these presented during the 1st week of life). A further 11 sacrococcygeal teratomas were found on light microscopy to include immature elements and had unpredictable clinical behaviour; 2 of these later metastasized despite adequate surgical clearance.All 4 patients with mesoblastic nephromas and 1 with a neonatal Wilm's tumour survived. In addition, 6 of the 10 patients with a neuroblastoma survived long-term. One of 3 patients with a rhabdomyosarcoma survived as well as 1 of 2 with a hepatoblastoma. Congenital fibrosarcomas, although morphologically aggressive, had an excellent outcome. Surgical excision was performed in all cases, and overall patient survival on long-term follow-up was 66% (44 patients). These survivors included 10 (41%) of those with malignant tumours and 24 (84%) with potentially malignant tumours. Metastatic spread or secondary tumours were shown to occur at variable stages, and early, frequent, and regular follow-up is recommended. Complications of extensive surgery resulted in 3 temporary and 2 long-term problems. Other adverse effects of therapy were observed in survivors of chemo- or radiation therapy, where poor growth was noted in 9 (82%), behavioural disturbances in 3 (27%), and intellectual impairment in 4 (36%).     

腹腔镜辅助下小儿骶尾部畸胎瘤切除术15例

目的探讨腹腔镜辅助切除小儿Ⅲ型骶尾部畸胎瘤的可行性、安全性及操作要点。方法回顾分析腹腔镜辅助手术治疗15例小儿Ⅲ型骶尾部畸胎瘤的临床资料。先经腹腔镜结扎肿瘤供应血管并游离瘤体的腹腔部分,后经骶尾部完整切除肿瘤。结果 12例顺利完成手术,3例中转开腹。术后均顺利恢复,经半年以上的随访未发现复发病例。结论腹腔镜辅助小儿骶尾部畸胎瘤切除术是有效、安全可行且损伤小的手术方法。     

Sacrococcygeal teratoma

Eight cases of sacrococcygeal teratoma are described. Seven patients were diagnosed at birth, and one presented at 3 1/2 months of age with urinary retention. Two patients had associated congenital anomalies (arachnoid cyst, external angular dermoid and bifid sacrum), while one had a family history of sacrococcygeal teratoma. All had the tumour removed together with the coccyx as soon as the diagnosis was made. None had recurrence of the tumour.     

Sacrococcygeal teratoma

Abstract Eight cases of sacrococcygeal teratoma are described. Seven patients were diagnosed at birth, and one presented at 3 1/2 months of age with urinary retention. Two patients had associated congenital anomalies (arachnoid cyst, external angular dermoid and bifid sacrum), while one had a family history of sacrococcygeal teratoma. All had the tumour removed together with the coccyx as soon as the diagnosis was made. None had recurrence of the tumour.     

PERIANAL EPENDYMOMA PRESENTING IN THE NEONATAL PERIOD

Extraspinal ependymomas are a rare type of glioma that may arise in the sacrococcygeal region, presenting as a pelvic mass in an infant or child. Ependymoma presenting in the newborn period has not been described previously. Herein we describe a case of a newborn boy who presented with a perianal ependymoma, which was subsequently found to have presacral extension. The major diagnostic challenge this case presented was to rule out the alternative diagnoses of sacrococcygeal teratoma or a developmental malformation heterotopia.     

Sacrococcygeal teratoma: an analysis of 37 cases

Thirty seven cases of sacrococcygeal teratoma were treated in Children Hospital, B.H.U. from 1974 to 1982. Thirty-one of these were benign and 6 were malignant. In thirty two cases a swelling was present since birth and in the remaining the swelling appeared later and these all were malignant. Most of them (70%) were below one year on admission. Usual presentation was a swelling on the sacrococcygeal region (94·6%) with an intrapelvic extension. Immature neural elements were present only in malignant cases. Benign treatomas were treated by early total surgical excision; recurrence was noted in one case only. Malignant cases however showed poor results following surgical excision and post-operative radiotherapy and chemotherapy.     

Head and neck teratomas in children

A retrospective review of seven patients with teratomas of the head and neck treated at out center over the past 5 years, which represented 2% of all teratomas (sacrococcygeal, ovarian, and retroperitoneal) seen over the past 20 years, was performed. After investigation to exclude associated anomalies, all but one of the children underwent surgery for removal of the tumour. All excised tumours were subjected to histopathological examination to confirm their teratomatous nature. The patients were followed up at regular intervals for up to 4 years. The patients' ages ranged from newborn to 2.5 years. There were three cervical, two oral, and two skull teratomas. The youngest patient had a cervical teratoma with respiratory compromise, requiring tracheal intubation. All but one patient (skull teratoma) had excision of the tumours with a satisfactory outcome. Histopathological examination of the excised tumours showed mature tissue from the three germinal layers in all specimens. Specific components included glandular epithelium, keratinising epithelium, and muscle fibres. Follow-up did not show any recurrence in the operated children. The three with cervical teratomas had normal levels of thyroid hormone post-operatively. Head and neck teratomas in children are mostly benign lesions amenable to curative excision. In cervical teratomas airway management takes priority. Accepted: 8 November 1999     

Germ cell tumors in children: gonadal and extragonadal.

Sixty-three pediatric patients with germ cell tumors are presented with details of symptoms, histological findings, staging, serological markers, treatment, and response to therapy. The primary sites were: ovarian 32, testicular 17, presacral 7, mediastinal 3, intraabdominal 2, vaginal 1, and right inguinal canal 1. These patients were treated with T2 (sequential use of dactinomycin, doxorubicin, vincristine, and cyclophosphamide, with or without radiation), T6 (combination chemotherapy with cyclophosphamide, bleomycin, dactinomycin, doxorubicin, methotrexate, vincristine), or VAB treatment protocols (velban, dactinomycin, bleomycin, cisplatin). The cure rate for stage I ovarian and testicular germ cell tumors was 100%; for stage III, all primary sites, 82% and for stage IV, all primary sites, 75%. Histology was prognostic in ovarian tumors of the immature malignant teratoma type; the neural type immature teratoma, grades II and III, had the worst prognosis. Initial debulking surgery in combination with chemotherapy and radiation plays an important role in germ cell tumors. Stages II, III, and IV germ cell tumors require aggressive treatment with surgery, radiation, and chemotherapy. For stage I patients, with primary ovarian malignant tumor, cure with surgery alone can be achieved in 50% of the cases and in testicular tumors in about 70% of the patients. For those with stage I and elevated serological markers, it is feasible to follow these markers and give no treatment until there is evidence of persistent elevation or a rise in titers after an initial fall. In those without elevated serological markers, one should take into consideration the size of the tumor and the histological type before taking the "wait and see" approach. These stage I tumors are highly curable when they first present but, if allowed to recur, chemotherapy may not offer the patient such a favorable response and cure rate.     

经肛门治疗Currarino综合征

目的 探讨Currarino综合征(Currarino syndrome,CS)的诊断和经肛门手术治疗的经验.方法 2013年1月至2015年5月,我院共收治11例CS患儿,男2例,女9例,平均年龄11个月.其中7例肛门位置正常,但肛门直肠严重狭窄;1例患儿反复出现肛周感染及肛瘘;3例患儿可见肛门直肠畸形.其中5例患儿术前已行结肠或回肠造瘘术.术前全部患儿行肛门指诊、骶尾部正侧位片、下消化道造影及骶部核磁共振检查.术中患儿经过肛门或瘘口游离直肠,肠管拖出后,向上牵开肠管.将切口后方向左右牵开,可暴露并切除骶前肿物.术后全部患儿给予抗感染治疗,术前已有造瘘患儿术后第1天恢复饮食,无造瘘患儿术后2d恢复饮水,术后4d恢复低渣进食,无并发症患儿术后7d出院,14d扩肛.术后每3个月复查.已有造瘘患儿术后3个月行造瘘还纳术.结果 肛门位置正常患儿全部有直肠肛门严重狭窄,小指不能进入.全部患儿影像学检查可见骶骨畸形和骶前肿物.11例患儿全部顺利完成手术,术后8例恢复良好,1例患儿出现骶前及中枢神经系统感染,1例高位肛瘘,1例出现术后骶前出血.全部患儿目前恢复良好.结论 对于年龄小于3岁,肿物最大径小于5 cm的CS患儿,经肛门一次完成肛门成型和骶前肿物切除是可行的.     

Angiotensin-Induced Hypertension Chemotherapy in Children with Advanced Solid Tumors

Angiotensin-induced hypertension chemotherapy (IHC) was investigated in six children with the following advanced malignancies: hepatocellular carcinoma, extraskeletal Ewing's sarcoma, sacrococcygeal malignant teratoma, small round cell tumor of the chest wall, hepatoblastoma and osteogenic sarcoma. Partial response was achieved in three of these patients, two showed no change, and in one IHC was used as adjuvant chemotherapy. The side effects of IHC were minimal and tolerable. Angiotensin-IHC may provide a new approach to pediatric cancer chemotherapy.     

Malignant childhood teratomas in the Nigerian savannah

At the Ahmadu Bello University Hospital in Zaria within the Guinea Savannah of Nigeria, 54 childhood teratomas were seen between 1972 and 1983. Four of the 28 sacrococcygeal, three of the 13 ovarian and the only lumbar teratoma were malignant. Surgical excision was the mainstay of management and adjuvant therapy with vincristine, actinomycin D and cyclophosphamide (VAC) was planned but was not always executed because of irregular drug supply. Two of the children died in the hospital and three were discharged home for terminal care; two with malignant ovarian teratomas survived at least for 15 and 18 months respectively, but with evidence of distant spread. It is concluded that surgery alone is unlikely to cure these malignant tumours but that improvement in the prognosis of these lesions in Nigeria must await the availability of combined therapy with multiple cytotoxic drugs and irradiation.