OBJECTIVETo discuss the treatment and diagnosis of congenital median perineal cleft in children.

METHODSBetween January 2009 to February 2013, 7 cases were diagnosed as congenital median perineal cleft according to the symptoms. Among them, 4 cases underwent surgery to correct cleft with double triangular perineal flaps. The other 3 cases with minor cleft did not receive surgery management.

RESULTSThere is an median cleft from the perineum to the anus with mucosa on the cleft surface. Primary healing was achieved in all the four patients with satisfactory appearance. The patients were followed up for 1-4 years with almost normal perineal appearance.

CONCLUSIONSCongenital perineal median cleft can be diagnosed according to the symptoms. Double triangular perineal flaps can be effectively correct the cleft to attain normal perineal appearance.

Adolescent ; Anal Canal ; Child ; Humans ; Perineum ; abnormalities ; surgery ; Surgical Flaps ; Treatment Outcome ; Wound Healing

OBJECTIVETo investigate the diagnosis and treatment of hypotelorism.

METHODSFrom Jan. 2000 to Jan. 2014, 6 cases with hypotelorism were retrospectively studied. Among them, 3 cases had craniosynostosis, 2 had holoprosencephaly, and 1 had cleft lip. All the cases were diagnosed and treated by bone graft or spring distraction to correct the hypotelorism.

RESULTS2 cases were treated by none graft and 4 cases were treated by external spring distraction. All the patients completed the treatment successfully with obvious improvement in appearance. No complication happened. 4 cases were followed up for 2 years with an average fronto-orbital axis angle as (50 ± 8) °.

CONCLUSIONSHypotelorism can be successfully corrected by bone graft as fronto-orbital bridge or spring distraction.

Bone Transplantation ; Cleft Lip ; Craniofacial Dysostosis ; diagnosis ; surgery ; Craniosynostoses ; complications ; Humans ; Osteogenesis, Distraction ; Retrospective Studies ; Treatment Outcome

Objective: To explore the effect of distraction osteogenesis in the treatment of syndromic craniosynostosis. Methods: The clinical data of 6 children with syndromic craniosynostosis from January 2014 to September 2018 were retrospectively analyzed. There were 5 males and 1 female, aged from 1 month and 21 days to 6 years and 1 month, with an average age of 30 months. There were 3 Crouzon syndrome, 1 Pfeiffer syndrome, 1 Vogt syndrome (ACS Ⅱ) and 1 Clove leaf skull syndrome. The distraction osteogenesis apparatus was used in this procedure. The distraction osteogenesis was prolonged twice a day, 0.4 mm each time, and the prolongation was stopped when the skull shape was significantly improved. Three-dimensional CT scans of the skull were reviewed after 6 months, suggesting that distraction osteogenesis was good, then the lengthener was removed. Complications were recorded, and extended distances were measured. Cranial indices before and after operation were compared to evaluate the efficacy, safety and feasibility of the operation. Results: The procedure of operation and traction was successful in all 6 children. There were no complications such as cranial spinal fluid (CSF) leakage or intracranial infection occurred. The increased distance was (19.1±3.3) mm, ranging from 15.2 to 25.6 mm. The preoperative cranial index was 89.6 ±7.3, while the postoperative cranial index was 74.2 ±3.6. All patients were followed up from 3 to 20 months, with the average of 14 months. The posterior cranial flatness was improved, and the patients were satisfied with the surgical results. Conclusions Distraction osteogenesis is effective and reliable in treating the premature fusion of cranial suture, and it produces excellent result with low rate of CSF leak and infection.

Objective: To evaluate the effect of three-dimensional Z-shaped osteotomy with cranium distraction osteogenesis for unilateral lambdoid synostosis. Methods: Imaging the skull as a cuboid, we designed the affected lambdoid suture as the middle line of letter Z, the osteotomy line paralleling to the sagittal suture or extending downward posterior cranial fossa as the two horizontal lines of Z to obtain the three-dimensional Z-shaped osteotomy lines. Two or three distraction devices were installed after removing the cranial bone at the premature fused suture with a width of 2.0-2.5 cm. Since the 5th day after operation, distraction was performed at the rate of 0.6 mm each day, twice a day, until the distraction distance reached 2.0-4.5 cm. Finally, we removed the distractors after fixation for three months. Results: Eleven cases of unilateral lambdoid synostosis were treated successfully. No infection or bleeding happened except for one case with distractor entrance scalp infection. No complications, including the fixed screws displacement, penetrating the cranium and the dura mater or distraction devices retracting, occurred. During a mean follow-up period of 24 months (5 to 36 months), all patients were satisfied with the cosmetic and functional result. Conclusions Three-dimensional Z-shaped osteotomy with cranium distraction osteogenesis can not only extend upward to raise the cranial height, but also lengthen backward to singularize the occipital carina, forming a well-pleasing occipitalia. Z shape stabilizes the whole plate, making the separated cranial bones not easy to retract. Therefore, using this procedure is effective and mini-invasive, and especially suitable for young infant.

Objective: To investigate the treatment of nasal deformity in patients with unicoronal craniosynostosis. Methods: In patients over 6 months old, the nasal bones were wedge-removed without fixation. The management of all patients with unicoronal craniosynostosis was distraction osteogenesis of pedicled unilateral frontal bone flap. Results: Postoperative extended distance of the frontal bone was 28—41 mm (mean, 35.4 mm). After extension, three-dimensional reconstruction of cranial CT was carried out, which showed that CVAI was 0.8—1.2 (mean, 0.98), tending to normal. After discharge, dynamic cranial braces were put on for 1 year. Postoperatively, the children were followed up for 8—36 months (mean, 28 months). The shape and nasal deformity of all children were improved compared with those before surgery. Conclusions Nasal wedge resection should be used to correct nasal deformity in children over 6 months with unicoronal craniosynostosis.

We treated a neonate who was diagnosed as rapidly involuting congenital hemangioma (RICH). With a review on the clinical manifestation, differential diagnosis, and treatment of RICH that associated with thrombocytopenia(TP)and coagulopathy(CP)and received platelet transfusions and embolization therapy including absolute ethanol and polidocanol in our hospital in March of 2015. The platelets and coagulation function soon returned to normal, the tumor involuted significantly. Surgical excision was proceeded subsequently. The platelets returned to normal level after a one-time platelet transfusion, meanwhile, multiple reexaminations of blood coagulation function were normal. Postoperatively, incision wound healed well. There was no recurrence, and the functional recovery of upper limb was satisfactory. RICH is a rare type of vascular tumor which may present with TP and CP similar to KHE-KMP in the neonatal period. More attention should be addressed to identify these two diseases, because the treatment and prognosis of which have significant differences. Consumption of coagulation factors with milder platelet decrease may also be a complication of slow flow venous or venolymphatic malformations.

Objective: To explore the feasibility of mandibular distraction for the treatment of airway obstruction in neonates with Braddock-Carey syndrome. Methods: From August 2007 to November 2017, 6 neonates with Braddock-Carey syndrome were treated in our center. All patients experienced the combined thrombopoietic treatment before operation. Bilateral oblique mandibular osteotomy was performed by extraoral approaches and the distraction was initiated on postoperative day 3 at a rate of 1.2 mm/d. It usually took around 2 weeks to get required length of distraction. Distractor was removed after 3-months fixation at second stage. Results: The distraction distance of 6 cases was from 12 to 20 mm. The CT scan showed a widening gap of the airway by 6-10 mm, and the average width was around 7 mm. 5 patients had no bleeding and infection after the operation, while one patient experienced bleeding for 4 times treated by regional compression. All neonates felt painful during distraction, and got relieved after reduction of the frequency and pace. With mandibular advancement, all patients′ face type improved, while paroxysmal bruising, inspiratory dyspnea and difficulty in breastfeeding disappeared. After 6 months of follow-up, all patients′ weight and face type were basically the same as normal children. Conclusions With the treatment of thrombocytopenia before operation, complete hematischesis during the operation, and postoperative treatment of thrombocytopenia, mandibular distraction osteogenesis was a feasible and safe management of upper airway obstruction in neonates with Braddock-Carey syndrome.

Objective: To assess the therapeutic effect of management for lymphatic malformation(LM)in infants. Methods: This retrospective study recruited clinical data of 996 patients with LM from June 2004 to July 2015 in our center. All patients were diagnosed as LM after ultrasound, CT or MR scan. All patients were divided into Group 1 (427 patients, treated by endoscopic LM partial resection combined with cautery and postoperative intratumoral negative pressure and absolute ethyl alcohol), Group 2(239 patients, treated by combined pinyangmycin and dexamethasone injection), Group 3 (330 patients, treated by surgical resection only). The clinical effects were observed in three groups, and therapeutic effect differences in gender, age, maximum diameter, location, range, histological typing, lymph property and treatments were analysed in cervicofacial LM. Results: Group 1: 333 patients were cured (78.0%), Group 2: 165 patients were cured(69.0%), Group 3: 238 patients were cured (72.1% ). The difference in cure rate between Group 1 and Group 2 or between Group 1 and Group 3 was significant(P<0.05). The number of patients with ≥2 treatments in Group 3 was significantly less than that in other two groups. The cure rate of LM in cervicofacial area was significantly lower than that in other parts of body (P<0.05). In cervicofacial LM patients, the therapeutic effect differences in maximum diameter, range, histological typing, lymph property were statistically significant (P<0.05). At the multivariable logistic regression analysis, LM range as well as histological typing were independent factors influencing the therapeutic effect (P<0.05). Conclusions Both treatment of endoscopic LM partial resection combined with cautery and postoperative intratumoral negative pressure and absolute ethyl alcohol, and surgical resection have good therapeutic effect on LM in infants. LM range as well as histological typing are important factors independently influencing the therapeutic effect of cervicofacial LM in infants.

Objective To investigate the clinical effect of radiofrequency ablation (RFA) combined with lauromacrogol for the treatment of parotid gland hemangioma in infants.Methods From Sep.2005 to Dec.2014,a total of 117 patients with parotid gland hemangioma were divided into observation group (n =32),control group 1 (n =30),control group 2 (n =28) and control group 3 (n =27).The observation group was treated by RFA combined with lauromacrogol,while the control group 1 was treated by RFA only,the control group 2 was treated by pingyangmycin only,and the control group 3 received additional pingyangmycin based on RFA.All the patients were followed up for 6 to 24 months,and the clinical effects were observed in four groups.Results Control group 1:16 patients were cured (53.3％),and the number of patients with ≥2 treatments was 9.There was 1 case with skin ulceration,1 with abdominal discomfort,1 with liver function damage and 1 with high fever.Control group 2:17 patients were cured(60.7％),and number of patients with ≥2 treatments was 12.There were 3 cases with skin ulceration,9 with abdominal discomfort,6 with liver function damage and 5 with high fever.Control group 3:21 patients were cured(77.8％),and number of patients with ≥2 treatments was 2.There were 2 cases with skin ulceration,6 with abdominal discomfort,4 with liver function damage and 4 with high fever.However,in observation group,25 patients were cured(78.1％) and number of patients with ≥ 2 treatments was 4,which were significantly different from that in control group 1 or control group 2 (P ＜0.05).And the difference in postoperative adverse reactions between observation group and control group 2 or control group 3 was also of significance (2 with skin ulceration,1 with abdominal discomfort,1 with liver function damage and 6 with high fever,P ＜ 0.05).Conclusions Combined RFA and lauromacrogol has a good therapeutic effect on parotid gland hemangioma with relatively low adverse effect,so it deserves to be suggested and applied in clinical practice.

Objective:To investigate clinical effects of the three-dimensional(3D) digital technique-assisted distraction osteogenesis in newborns with Pierre Robin sequence(PRS).Methods:The data of neonatal Pierre Robin sequence treated with 3D digital technique-assisted distraction osteogenesis in Children’s Hospital Affiliated to Nanjing Medical University from April 2017 to April 2020 was retrospectively analyzed. 3D digital technique was used to assist the pre-operative design and osteotomy navigation template was made with 3D printing technique to guide in the mandibular osteotomy. CT scans were obtained 3 months postoperatively to follow up the growth of mandible. The rates of recurrence, titanium nail falling, infection, secondary operation and nerve injury were statistically analyzed. The counting data were analyzed using chi-square test. The independent influencing factors of postoperative complications were analyzed by multiple logistic regression, and the difference was statistically significant when P<0.05. Results:Three hundred and forty-five patients were included in the 3D digital technique group consisting of 215 males and 130 females with the ages ranged 3-28 days and the weights 1.8-3.2 kg. Surgical results were satisfied and very close to the preoperative design. Craniofacial CT results showed normal growth of mandible 3 months postoperatively. Three hundred and seventy-seven patients were included in the controlled group consisting of 230 males and 147 females with the ages ranged 6-28 days and the weights 1.6-3.6 kg. The recurrence rate, extender titanium nail off rate, reoperation rate and risk of nerve damage in the 3D digital technique group were 0.29%(1/345), 0.29%(1/345), 0.58% (2/345)and 0.29%(1/345), respectively, while in the control group, the numbers were 1.86%(7/377), 2.92%(11/377), 2.65%(10/377), 2.92% (11/377), respectively, which were significant lower than those in the 3D group. The infection rates were 7.83%(27/345) in the 3D digital technique group and 7.69%(29/377) in the control group, and there was no significant difference between the two groups. The results of multi-logistic regression analysis suggested that the 3D digital technique group could be an independent factor to reduce postoperative complications ( P=0.001). Conclusions:In the newborns with Pierre Robin sequence, 3D digital technique-assisted distraction osteogenesiscan significantly improve the accuracy and success rate of the operation and reduce the complication rate as well.