Outcomes for Prenatally Diagnosed Right Congenital Diaphragmatic Hernia.

BACKGROUND: Right congenital diaphragmatic hernia (CDH) occurs less frequently than left CDH. Therefore, prognostic indicators for right CDH are not as well studied as for left CDH. METHODS: A retrospective review from a single, tertiary referral center (from 1994 until July 2013) of patients with unilateral right CDH was conducted. Prenatal characteristics were evaluated and correlated with survival to discharge and need for extracorporeal membranous oxygen (ECMO). RESULTS: In total, 34 patients were identified. There were 12 postnatal deaths and 2 fetal demises (6%), representing an overall mortality of 41%. Six patients required ECMO. Nine patients underwent fetal intervention and were analyzed separately. For patients not undergoing fetal intervention, the survival rate was 52% and a higher mean (±SD) lung-to-head ratio (LHR) was associated with survival (1.1 ± 0.4 vs. 0.8 ± 0.2, p = 0.03). There were no deaths or need for ECMO in any patient with an LHR ≥1.0. Of the 9 patients who underwent fetal intervention, survival was 78% and only 1 patient required ECMO. Fetal intervention was primarily tracheal occlusion (n = 8). CONCLUSIONS: An LHR <1.0 is associated with worse survival for right CDH and may also reflect the need for ECMO.

Congenital H-type tracheoesophageal fistula: A multicenter review of outcomes in a rare disease.

OBJECTIVE: To perform a multicenter review of outcomes in patients with H-type tracheoesophageal fistula (TEF) in order to better understand the incidence and causes of post-operative complications. BACKGROUND: H-type TEF without esophageal atresia (EA) is a rare anomaly with a fundamentally different management algorithm than the more common types of EA/TEF. Outcomes after surgical treatment of H-type TEF are largely unknown, but many authoritative textbooks describe a high incidence of respiratory complications. METHODS: A multicenter retrospective review of all H-type TEF patients treated at 14 tertiary children's hospital from 2002-2012 was performed. Data were systematically collected concerning associated anomalies, operative techniques, hospital course, and short and long-term outcomes. Descriptive analyses were performed. RESULTS: We identified 102 patients (median 9.5 per center, range 1-16) with H-type TEF. The overall survival was 97%. Most patients were repaired via the cervical approach (96%). The in-hospital complication rate, excluding vocal cord issues, was 16%; this included an 8% post-operative leak rate. Twenty-two percent failed initial extubation after repair. A total of 22% of the entire group had vocal cord abnormalities (paralysis or paresis) on laryngoscopy that were likely because of recurrent laryngeal nerve injury. Nine percent required a tracheostomy. Only 3% had a recurrent fistula, all of which were treated with reoperation. CONCLUSIONS: There is a high rate of recurrent laryngeal nerve injury after H-type TEF repair. This underscores the need for meticulous surgical technique at the initial repair and suggests that early vocal cord evaluation should be performed for any post-operative respiratory difficulty. Routine evaluation of vocal cord function after H-type TEF repair should be considered. THE LEVEL OF EVIDENCE RATING: Level IV.

Magnetic Mini-Mover Procedure for pectus excavatum IV: FDA sponsored multicenter trial.

PURPOSE: The Magnetic Mini-Mover Procedure (3MP) is a minimally invasive treatment for prepubertal patients with pectus excavatum. This multicenter trial sought to supplement safety and efficacy data from an earlier pilot trial. METHODS: Fifteen patients with pectus excavatum had a titanium-enclosed magnet implanted on the sternum. Externally, patients wore a custom-fitted magnetic brace. Patients were monitored closely for safety. Efficacy was determined by the Haller Index (HI) and satisfaction surveys. After 2 years, the implant was removed. RESULTS: Mean patient age was 12 years (range 8-14), and mean pretreatment HI was 4.7 (range 3.6-7.4). The device was successfully implanted in all patients. Mean treatment duration was 25 months (range 18-33). Posttreatment chest imaging in 13 patients indicated that HI decreased in 5, remained stable in 2, and increased in 6. Seven out of 15 patients had breakage of the implant's titanium cables because of fatigue fracture. Eight out of 13 patients were satisfied with their chest after treatment. CONCLUSION: The 3MP is a safe, minimally invasive, outpatient treatment for prepubertal patients with pectus excavatum. However, the magnetic implant design led to frequent device breakage, confounding analysis. The HI indicated mixed efficacy, although surveys indicated most patients perceived a benefit. STUDY TYPE/LEVEL OF EVIDENCE: Case series, treatment study. Level IV.

Inflammatory myofibroblastic tumors in children.

BACKGROUND: Inflammatory myofibroblastic tumor (IMFT) is an uncommon neoplasm in children. METHODS: Retrospective review from 1993 to 2014 of patients ≤18years of age with a histopathologic diagnosis of IMFT treated at two tertiary centers. RESULTS: Thirty-two patients were diagnosed with IMFT. Mean (±SD) age was 9.3±5.7years at diagnosis. Tumor location was variable: abdomen/pelvis (28%), head/neck region (22%), intrathoracic (22%), genitourinary (9%), bowel (6%) liver (6%), and musculoskeletal (6%). Median follow-up was 2.6±4.6years, with 3 recurrences and 2 deaths, which occurred only after recurrence. Positive microscopic margin after resection was associated with recurrence, compared to those that had a negative margin (40% vs. 0%, p=0.04). Recurrence was associated with increased mortality (67% vs 0%, p=0.01). Time from first symptoms to resection was shorter in those with recurrence (25.8±22 vs. 179±275days, p=0.01) and in nonsurvivors (44.0±8.0 vs. 194.3±53.4days, p=0.02). Adjuvant chemotherapy, not including steroid monotherapy, either given before or after resection, was administered more often to nonsurvivors (100% vs 4%, p=0.009), and use of corticosteroids was also higher in the nonsurvivors (100% vs. 15%, p=0.04). CONCLUSIONS: IMFT is a rare pediatric neoplasm with variable locations. Complete excision is critical for cure. Proposed guidelines for diagnosis, treatment and surveillance of theses tumors in children are reported.

Challenges and climate of business environment and resources to support pediatric device development.

The incidence of pediatric disease conditions pales in comparison to adult disease. Consequently, many pediatric disorders are considered orphan diseases. Resources for the development of devices targeting orphan diseases are scarce and this poses a unique challenge to the development of pediatric devices. This article outlines these challenges and offers solutions.

Granular cell tumors and congenital granular cell epulis in children: Similar entities.

BACKGROUND/PURPOSE: Granular cell tumor (GCT) is an unusual lesion thought to originate from Schwann cells. Congenital granular cell epulis (CGCE) is a rare fibroma-like lesion arising from the alveolar ridge in newborns. These entities have been described as distinct entities. METHODS: A retrospective review was performed of children with a histopathologic diagnosis of GCT or CGCE from 1991-2014. Data were recorded and analyzed. All values reported as the mean±standard deviation. RESULTS: GCT or CGCE was identified in 41 patients with a mean age of 7.8±6.1years. Thirty-one patients had GCT, and 10 had CGCE. All patients underwent excisional biopsy, except 1 who underwent incisional biopsy only. Fifteen patients had positive margins after excision (12 GCT and 3 CGCE). Only 1 had a local recurrence, and this same patient had multifocal GCT. Only 1 patient had an invasive lesion without recurrence after wide local excision. All patients survived, with a median follow-up of 42.5months (0.2-204.2months). CONCLUSION: In children, both GCT and CGCE exhibit benign behavior, and complete excision does not appear to be mandatory, as recurrence or invasive disease is rare. When invasive features are present, wide local excision should be undertaken.

Compensatory hypertrophy of the remaining kidney in medically complex living kidney donors over the long term.

BACKGROUND: The criteria for living kidney donation are changing, resulting in increased numbers of individuals with risk factors being accepted as donors. The long-term function and volume changes in the remaining kidney of these medically complex donors remain largely unknown. METHODS: Living kidney donors with three separate risk factors (older age, obesity, or hypertension) were reevaluated 5 years after donation. The function and volume of the remaining kidney were assessed and compared to those of standard donors. RESULTS: The body size correlated significantly with the kidney size and glomerular filtration rate (GFR) at the time of donation. Five years after donation, the remaining kidney size increased by a mean of 29.3%, and the GFR by 35.6%. The increase in GFR was uniform. In univariate analysis, neither the changes in the size nor the changes in the 1GFR were found to be associated with the risk factors. CONCLUSION: Medically complex living donors demonstrate similar compensatory increase in function and volume of the remaining kidney compared to standard donors, 5 years after donation.

Laparoscopy for small bowel obstruction in children--an update.

INTRODUCTION: We evaluated the current role of minimally invasive surgery (MIS) in children with small bowel obstruction (SBO) at our institution. SUBJECTS AND METHODS: A retrospective review of patients undergoing MIS for acute SBO was performed from 2008 to 2013. The study population was compared with a historical control including patients from 2001 to 2008. RESULTS: There were 71 patients who met inclusion criteria; 35 were male, and 36 were female. Sixty-two children underwent laparoscopy for their first episode of SBO, and 12 underwent laparoscopy for recurrent SBO, accounting for 74 episodes of SBO managed with MIS. The most common etiology of SBO was adhesions (n=40). Laparoscopy and laparoscopic-assisted procedures were associated with shorter nasogastric tube decompression (1.4±2 days [P<.001] and 1.5±2.7 days [P=.002], respectively) and time to regular diet (3.9±4 days [P=.002] and 4.6±2.8 days [P=.024], respectively) compared with those converted to laparotomy (5.1±4.9 days of nasogastric tube decompressions and 8±4.7 days to regular diet). There was no difference in postoperative morbidity comparing laparoscopy (11%), laparoscopic-assisted (5%), and laparoscopic converted to open procedures (18%) (P=.48). CONCLUSIONS: Laparoscopy continues to be a safe diagnostic and therapeutic tool in the management of pediatric SBO.

Amniotic band syndrome: a single-institutional experience.

INTRODUCTION: Amniotic band syndrome (ABS) is uncommon. We review our single-institutional experience to define its natural history and outcomes. MATERIALS AND METHODS: We conducted a retrospective analysis from a single, tertiary referral center of patients evaluated for and confirmed to have ABS from 1997 to 2012. RESULTS: Twenty-eight patients had confirmed ABS. The mean ± SD maternal age was 27.9 ± 5.9 years, and the mean gestational age at diagnosis was 20.7 ± 3.8 months. Oligohydramnios was reported in 4 patients. Eleven patients had membrane disruption, of whom 4 had undergone a prior percutaneous intervention. Extremities were the most common site affected (n = 20), followed by the umbilical cord (n = 7), abdomen (n = 5), limb-body wall complex (n = 5), head (n = 1), and chest (n = 1). Nine patients were felt to be candidates for fetal intervention; 5 underwent fetoscopic amniotic band lysis with 4 survivors. Overall survival, excluding 3 terminations, was 74%. There were 5 fetal demises and one neonatal death. Cord involvement was higher in nonsurvivors (67%) compared to survivors (19%, p = 0.05). DISCUSSION: ABS most commonly involves the extremities. Membrane disruption is not always present. Fetoscopic lysis is appropriate for select patients, and special consideration should be given for cord involvement, which is associated with a worse outcome.

Multi-institutional experience with penetrating pancreatic injuries in children.

PURPOSE: Penetrating pancreatic injuries in children are uncommon and are not well described in the literature. We report a multi-institutional experience with penetrating pancreatic injuries in children. METHODS: A retrospective review of children sustaining penetrating pancreatic injuries was performed at eight pediatric trauma centers. RESULTS: Sixteen patients were identified. Eleven patients were male; (mean ± SE) age was 11.7 ± 1.2 years. The mechanism of injury was gun-shot wound in 14 patients and mean injury-severity score was 18 ± 3. All patients had associated injuries, most frequently small bowel injuries (n = 9). Patients had either grade I (n = 4), grade II (n = 7), or grade III (n = 4) injuries; there was a single grade V injury. All patients underwent exploratory celiotomy. Drainage of the injured pancreas was performed in 11 patients, and 2 patients underwent pancreatorrhaphy in addition to drainage; 3 underwent resection for grade III (n = 2) and grade V (n = 1) injuries. Thirteen patients required other intra-abdominal procedures. All patients required intensive care over a mean 11.0 ± 3.0 days. Mean duration of stay was 30.1 ± 5.6 days. Post-operative morbidity was 62.5% with no mortalities. CONCLUSIONS: Penetrating pancreatic injuries in children are uncommon and most often due to firearms. There is a high association with other injuries particularly hollow viscous perforation.

Review of our experience with neuroblastoma and ganglioneuroblastoma in adults.

BACKGROUND: Neuroblastomas and ganglioneuroblastomas (NB/GNB) are malignant tumors that rarely occur in adults. Their disease progression and appropriate treatment are unclear. METHODS: All adults (age ≥18 years) were evaluated for histologically confirmed NB/GNB within our institution. Data were collected via chart review and direct patient contact. RESULTS: From 1980 to 2009, a total of 15 adult patients with NB/GNB were evaluated: six men (mean age 23 years, range 19-33 years) and nine women (mean 34 years, range 20-66 years). Their overall average age at diagnosis was 30 years. Tumor-related symptoms occurred in ten patients: Pain (abdominal 3, back 2, pelvic 1, groin 1) was more common than a mass (abdominal 2) or dysmenorrhea (1). Five patients had tumors found incidentally by computed tomography (4) or chest radiography (1). Primary tumor origins were in the pelvis (4), mediastinum (3), abdomen (2), adrenal gland (2), retroperitoneum (2), and mixed locations (2). Altogether, 12 patients underwent surgical resection (biopsy in 3; resections of R0 in 5, R1 in 3, R2 in 4). Ten underwent chemotherapy. Histology showed four GNBs and 11 NBs. Five patients with stage I disease survived a mean of 21 years (range 10-40 years). Two are alive today. Three stage III patients died at 2, 6, and 9 years after diagnosis (mean 5.7 years). Six of seven patients (one was lost to follow-up) with initial stage IV neuroblastoma died within 5 years (mean 2.7 years). NB and GNB patients had similar survivals. CONCLUSIONS: Adult-onset NB/GNB is rare. Symptoms appear to occur later when incurable stage IV disease is detected. Complete surgical resection can lead to long-term, disease-free survival in stage I patients.

Youth ice hockey injuries over 16 years at a pediatric trauma center.

BACKGROUND: Youth ice hockey is an exciting sport with growing participation in the United States. Updated assessment of injury patterns is needed to determine risk factors for severe injury and develop preventive efforts. The purpose of this study was to evaluate our experience as a level 1 pediatric trauma center in Minnesota treating injured youth ice hockey players. METHODS: Children #18 years old who presented to our institution from July 1997 to July 2013 with an injury sustained while participating in ice hockey were identified. Patient demographic information, injury characteristics, and outcomes including use of computed tomography, hospital admission, and procedures were obtained. Age and gender-specific patterns were determined for injuries and outcomes. RESULTS: Over 16 years, 168 injuries in 155 children occurred, including 26 (15.5%) injuries in girls. Extremity injuries were most common, followed by traumatic brain injury. Injuries to the spine, face, and trunk were less common. Traumatic brain injury and injuries to the spine were most common in younger children (#14 years old) and girls, whereas injuries to the face were most common in older players ($15 years old). Most injuries resulted from intentional contact. Admission to the hospital was needed in 65 patients, including 14 (8.3%) who needed intensive care. A major procedure was needed by 23.2% of patients because of their injuries. CONCLUSIONS: Youth ice hockey trauma can be severe, necessitating a thorough evaluation of injured children. Injury patterns are influenced by age and gender, providing an opportunity for targeted preventive efforts.

Fetoscopic approach to amniotic band syndrome.

BACKGROUND: Amniotic band syndrome (ABS) is an uncommon complication of pregnancy that can result in fetal demise. METHODS: We present our experience with fetoscopic amniotic band release. RESULTS: Five patients underwent fetoscopic amniotic band release for preoperatively diagnosed ABS involving at least one extremity. Four of five patients were found to have involvement of the umbilical cord at the time of fetoscopy. One of these four did not have the band released and underwent fetal demise at 24 weeks. All four survivors had good functional outcomes of affected limbs. Two patients developed membrane separation and had preterm deliveries at 32 weeks gestation whereas the other two carried to term. No maternal complications were noted. CONCLUSIONS: Fetoscopic amniotic band release is safe. Umbilical cord involvement is difficult to assess preoperatively, but when it is present should be treated to reduce the risk of fetal demise.

Operative vs nonoperative management for blunt pancreatic transection in children: multi-institutional outcomes.

BACKGROUND: The management of traumatic pancreatic transection remains controversial. STUDY DESIGN: A multi-institutional review from 1995 to 2012 was conducted comparing operative with nonoperative management for grades II and III blunt pancreatic injuries in patients younger than 18 years. RESULTS: Fourteen pediatric trauma centers participated, yielding 167 patients; 57 underwent distal pancreatectomy and 95 were managed nonoperatively. Fifteen patients treated with operative drain placement only were studied separately. Patients undergoing resection had a shorter time to goal oral feeds (7.8 ± 0.7 days vs 15.1 ± 2.5 days; p = 0.007) and a lower rate of pseudocyst formation (0% vs 18%; p = 0.001). Pseudocyst formation resulted in a greater need for endoscopic and interventional radiologic procedures (26% vs 2%; p = 0.002) in the nonoperative group, as well as a longer time to complete resolution (38.6 ± 6.4 days vs 22.6 ± 5.0 days; p = 0.05) compared with resection. When looking at those patients with clear evidence of main duct injury at presentation, those undergoing resection also had fewer complications (33% vs 61%; p = 0.05) and fewer total days in-hospital (12.6 ± 8.4 days vs 17.5 ± 9.7 days; p = 0.04) compared with nonoperative management. CONCLUSIONS: In children with blunt pancreatic injury, distal pancreatectomy is superior to nonoperative management with more rapid resumption of diet, fewer repeat interventions, and a shorter period to complete resolution. When the main duct is involved, the benefits to operative resection also include lower morbidity and fewer days of hospitalization. Therefore, assessing the status of the pancreatic duct is paramount in determining management.

Protocol versus ad libitum feeds after laparoscopic pyloromyotomy: a prospective randomized trial.

BACKGROUND: We conducted a prospective, randomized trial comparing protocol to ad libitum (ad lib) feeding after laparoscopic pyloromyotomy. METHODS: Infants undergoing laparoscopic pyloromyotomy were randomized to protocol versus ad lib feeding strategies. The protocol started with Pedialyte® two hours post-operative. This was repeated by another round of Pedialyte®, then two rounds of half-strength formula or breast milk, followed by two rounds of full strength formula or breast milk, followed by the home feeding regimen, at which time the patient was discharged if feeding well. The ad lib group was allowed formula or breast milk two hours after the operation and considered for discharge after tolerating three consecutive feeds. The primary outcome variable was the length of postoperative hospitalization. RESULTS: One hundred fifty infants were enrolled between January 2010 and December 2011. There were no differences in patient characteristics at presentation. While the ad lib group reached goal feeds sooner than the protocol group, this did not translate into a difference in duration of postoperative hospitalization. There were more patients with emesis in the ad lib group after goal feeding was reached, but no difference in readmissions. CONCLUSION: Ad lib feeding allows patients to reach goal feeds more rapidly than protocol feeding following laparoscopic pyloromyotomy. However, this goal is usually reached beyond discharge hours, resulting in a similar duration of hospitalization.

Accuracy of American College of Surgeons National Surgical Quality Improvement Program Pediatric for laparoscopic appendectomy at a single institution.

BACKGROUND: In October 2008, the American College of Surgeons revealed the National Surgical Quality Improvement Program (NSQIP) Pediatric in an effort to improve quality of surgical care in children. A 5% disagreement rate of data reported between institutions is accepted. The two goals of this study were to (1) determine if the random sampling performed with NSQIP data collection was representative of the population, and (2) verify that data captured in NSQIP was accurate. METHODS: For children undergoing laparoscopic appendectomy from April 2010-April 2011, demographic data, length of stay (LOS), and rates of surgical site infection (SSI) and postoperative abscess recorded in NSQIP (group 1) were compared with data from chart review (group 2). Secondarily, all NSQIP data were examined for accuracy by comparing relevant data points to existing databases. All disagreements were further examined with review of the medical chart. Unpaired t-test and χ(2) with Fisher's exact test were used in the statistical analysis. RESULTS: NSQIP Pediatric captured data from 126 children (group 1); group 2 had 525 children. There were no significant differences in age, body mass index, gender, race or LOS between the two groups. Rate of SSI was 1.6% in group 1 and 1.7% in group 2 (P = 0.92). Abscess rate was 1.6% in group 1 and 3.4% in group 2 (P = 0.28). There were six errors in the NSQIP database. One child was listed as having two SSI. One child with postoperative abscess was missed and another was not counted as they were not categorized correctly. Recorded LOS was incorrect for two children and the other had incorrect age. CONCLUSIONS: NSQIP Pediatric captured a representative sample of patients undergoing laparoscopic appendectomy. Errors were found in the reporting of outcomes for SSI and postoperative abscess in children undergoing laparoscopic appendectomy. Given the low incidence of these outcomes, there is little effect on percentages of complications reported.

Clinical outcomes following bowel resection versus reduction of intussusception.

BACKGROUND: Intussusception is most commonly managed with air-contrast reduction. However, when this fails, emergent operation with resection or manual reduction is indicated. It is not known if there are advantages to resection compared with manual reduction. METHODS: A retrospective review of all patients receiving operative care for intussusception from February 2000 to December 2011. Patients undergoing intestinal resection were compared with those treated with manual reduction alone. RESULTS: Of 111 patients, 49 underwent resection and 62 underwent manual reduction. Mean (±SD) time to oral intake favored manual reduction (2.1 ± 1.2 versus 2.6 ± 1.2 d, respectively, P=0.05). Manual reduction was associated with a greater need for repeat imaging (47% versus 18%, P=0.002) and the only recurrences were with manual reduction (8% versus 0%, P=0.1). Mean duration of stay was no different (P=0.36), nor was the need for reoperation (P=0.9). CONCLUSIONS: Patients undergoing manual reduction have an increased number of radiographic imaging procedures. The surgeon should have a low threshold for resection for intussusceptions requiring operative management.

Single incision versus standard 4-port laparoscopic cholecystectomy: a prospective randomized trial.

BACKGROUND: Laparoscopy through a single umbilical incision is an emerging technique supported by case series, but prospective comparative data are lacking. Therefore, we conducted a prospective, randomized trial comparing single site umbilical laparoscopic cholecystectomy to 4-port laparoscopic cholecystectomy. METHODS: After IRB approval, patients were randomized to laparoscopic cholecystectomy via a single umbilical incision or standard 4-port access. The primary outcome variable was operative time. Utilizing a power of 0.8 and an alpha of 0.05, 30 patients were calculated for each arm. Patients with complicated disease or weight over 100 kg were excluded. Post-operative management was controlled. Surgeons subjectively scored degree of technical difficulty from 1=easy to 5=difficult. RESULTS: From 8/2009 through 7/2011, 60 patients were enrolled. There were no differences in patient characteristics. Operative time and degree of difficulty were greater with the single site approach. There were more doses of analgesics used and greater hospital charges in the single site group that trended toward significance. CONCLUSION: Single site laparoscopic cholecystectomy produces longer operative times with a greater degree of difficulty as assessed by the surgeon. There was a trend toward more doses of post-operative analgesics and greater hospital charges with the single site approach.

Costal cartilage excision for the treatment of pediatric slipping rib syndrome.

BACKGROUND: Costal cartilage excision is an effective treatment of slipping rib syndrome (SRS), although the diagnosis of SRS may be elusive. We review our experience with SRS in the pediatric patient. METHODS: This is a retrospective review from 2000 to 2011 of patients presenting with symptoms of SRS before 18 years of age. RESULTS: Seven patients were identified who were diagnosed with SRS and underwent costal cartilage excision. All patients presented with unilateral chest pain that was exacerbated by activity. Five patients reported a popping or clicking sensation with activity that was associated with pain. On physical examination, all patients had reproducible pain with palpation over the affected cartilage. Four patients had a mobile or popping rib with palpation, and 4 also had chest wall asymmetry. Five patients underwent imaging, and 4 patients were referred to specialists. It was a median of 2 years (0-5 years) from onset of symptoms to resection. At resection, all cartilages were grossly abnormal. There were no postoperative complications. Follow-up was complete for all patients over a median 0.9 years (0.2-2.0 years). One patient had recurrence of pain in a different location; another had persistent pain, which was less severe. CONCLUSIONS: Slipping rib syndrome presents with costal cartilage pain that is reproducible on physical examination and commonly associated with a mobile rib. Excision of the affected cartilage(s) is an effective treatment and should be considered early to avoid unnecessary diagnostic tests and evaluation, which delay definitive therapy.

Pilonidal Disease Mimicking Fistula-in-Ano in a 15-Year-Old Female.

Pilonidal disease typically presents with an abscess or intermittent pain and drainage in the sacrococcygeal region during the pubertal years. Further examination typically reveals pits in the midline of the sacrococcyx area due to entrapment of hair with recurrent entrapment, infection, and drainage. The following paper describes an unusual presentation of a pilonidal cyst with fissure and perianal drainage.